THE  LIBRARY 

OF 

THE  UNIVERSITY 

OF  CALIFORNIA 

LOS  ANGELES 


GIFT 

Mrs.   Frank  A.   Edwards 


WW 


CONTRIBUTORS 

f> 
BURR,    C.   W.,   M.D. 
DERCUM,    F.    X.,    M.D. 
DUANE,    ALEXANDER,    M.D. 
FRAZIER,    C.    H.,    A.B.,    M.D. 
HANSELL,    HOWARD    P.,    A.M.,  M.D. 
HIRSCH,    WILLIAM,    M.D. 
JACKSON,   EDWARD,    A.M.,    M.D. 
LLOYD,    J.    HENDRIE,    A.M.,    M.D. 
MILLS,    CHARLES   K.,   M.D. 
POSEY,    WM.    CAMPBELL,    A.B.,    M.D. 
RISLEY,    S.    D.,    A.M.,    M.D.,    PH.D. 
SACHS,    B.,    M.D. 
SAILER,    JOSEPH,    M.D. 
DE  SCHWEINITZ,    G.    E.,    A.M.,    M.D. 
SOUTER,    WILLIAM    NORWOOD,    M.D. 
SPILLER,    WILLIAM   C,    M.D. 
TAYLOR,    E.    W.,    M.D. 
WALTON,    G.    L.,    M.D. 
WEEKS,    JOHN    E.,    M.D. 
WEISENBURG,    T.    H.,    M.D. 
WOOD,    CASEY    A.,    CM.,    M.D.,    D.C.L. 
WURDEMANN,    H.   V.,    M.D. 


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PREFACE 

Although  it  is  generally  conceded  that  a  knowledge  of  neurology 
is  indispensable  to  the  ophthalmologist  and  that  an  acquaintanceship 
with  ophthalmology  is  of  the  greatest  service  to  the  neurologist,  there 
is  no  book  in  the  English  language  which  covers  the  ground  where 
these  two  specialties  meet.  Several  German  works  and  one  French 
book  have  appeared  M'liich  include  much  that  may  be  found  in  these 
pages,  but  even  the  student  who  is  conversant  with  these  languages  will 
frequently  find  these  books  lacking  in  the  information  he  seeks,  and  he 
will  often  be  compelled  to  search  through  many  other  sources  of  infor- 
mation before  he  obtains  what  he  desires.  It  has  been  the  aim  of  the 
editors,  therefore,  to  present  under  a  single  cover  the  phases  of  ophthal- 
mology and  neurology  which  are  in  any  way  connected  with  each  other, 
hoping  thereby  not  only  to  facilitate  the  labors  of  those  practising  these 
specialties,  but  also  to  present  ophthalmo-neurology  in  such  a  form 
that  the  general  practitioner  may  without  difficulty  become  conversant 
with  its  symptomatology. 

While  it  may  be  impossible  for  a  physician  in  general  practice 
to  be  at  the  same  time  an  ophthalmologist  and  a  neurologist,  it  is  quite 
possible,  and  indeed  essential,  that  he  should  be  aware  of  the  extent, 
as  well  as  of  the  limitation,  of  the  knowledge  of  these  two  specialties 
and  of  the  aid  they  may  offer  him  in  the  recognition  of  disease.  So 
many  general  morbid  processes  are  announced  by  some  minute  change 
in  the  nervous  system,  and  such  unusual  opportunities  of  studying 
alterations  in  the  vascular  system  are  presented  in  the  eye,  that  the 
value  of  a  clear  understanding  of  disease  of  these  structures  cannot  be 
overestimated,  and  the  necessity  for  proper  training  in  the  methods 
which  lead  to  its  recognition,  cannot  be  too  strongly  insisted  upon. 

Realizing  the  extent  of  the  task  before  them,  and  the  increased 
value  which  the  work  would  have  if  it  represented  the  views  of  others 
as  well  as  their  own,  the  editors  have  been  most  fortunate  in  securing 
a  number  of  distinguished  American  ophthalmologists  and  neurologists 
as  collaborators,  so  that  the  volume  consists  of  a  series  of  monographs 
by  authors  peculiarly  well  fitted  by  previous  study  and  investigation 
to  treat  authoritatively  of  the  subjects  assigned  to  them. 


▼i  PREFACE 

In  dividing  the  subjects  into  the  various  chapters,  care  was  taken 
to  avoid  repetition  of  the  same  matter  by  different  authors.  In  the 
chapters  where  overlapping  does  occur,  it  was  done  purposely,  with  the 
idea  of  having  both  the  ophthalmological  and  the  neurological  phases 
fully  presented. 

The  editors  desire  to  express  their  thanks  and  deep  sense  of  appre- 
ciation to  their  contributors  for  the  very  admirable  way  in  which  they 
have  treated  the  subjects  allotted  to  them.  It  has  been  a  source  of 
great  regret  that  the  work  did  not  appear,  as  originally  planned,  some 
six  months  ago,  but,  despite  every  effort  of  the  publishers,  certain  labor 
difficulties  preventetl,  and  it  has  been  impossible  to  issue  it  until  now. 

The  index  has  been  prepared  by  Dr.  Paul  La  Roque  and  the  editors 
would  express  to  him  their  thanks  for  his  thoroughness. 

The  Editoes. 
Philadelphia,  April  10,  1906. 


TABLE  OF  CONTENTS 

By  William  G.  Spiller,  M.D. 

THE     INTRACKANIAL      PORTION      OF     THE     ENCEPHALIC     NERVES, 
ESPECIALLY   THOSE   RELATED  TO    THE   EYE. 

The  olfactory  nerve — The  optic  nerve — The  primary  optic  centres — "  Pupillary 
fibres  "  in  the  visual  system — The  cortical  centres  of  vision — The  oculomotor 
nerve — Trochlear  nerve — Trigeminal  nerve — Abducens  nerve — Facial  nerve 
— Acoustic  nerve — Glossopharyngeal  and  vagus  nerves — Accessorius — Hypo- 
glossal nerve  1  to  19 

CHAPTER  II. 

By  William  Norwood  Souter,  M.D. 

SIGHT:    THE   STRUCTURE    AND   OPTICAL    PROPERTIES  OF   THE   EYE. 

The  structure  of  the  eye — The  eye  as  a  refractory  apparatus — Anomalies  of 
refraction — Determination  of  visual  acuteness  and  of  the  refraction  of 
an    eye   20  to  56 

CHAPTER  III. 

By  Charles  K.  Mills,  M.D. 

THE   PSYCHOLOGY  OF  THE  VISUAL  ACT  AND  THE  FOCAL  DISEASES 
OF  THE  VISUAL   CORTEX. 

Introductory  outline — The  limits  and  sub-divisions  of  the  visual  cortex — His- 
tology of  the  visual  cortex,  especially  with  reference  to  visual  localization — 
The  position  and  the  sub-divisions  of  the  primary  cortical  visual  area — The 
focal  diseases  of  the  visual  cortex — Hemianopias  and  other  defects  in  the 
visual  fields  due  to  cortical  lesion — Cerebral  chromatic  phenomena  and  the 
cortical  centres  for  color  recognition — Amblyopias  due  to  disease  of  the 
visual  cortex  or  of  its  entering  and  connecting  tracts — The  visual  portion 
of  the  cerebral  zone  of  speech  and  visual  aphasias — Mind  blindness — Word 
blindness  and  allied  disorders — Agrammatism — Visual  anomia  or  word 
dumbness    57  to  161 

CHAPTER  IV. 
By  Casey  A.  Wood,  M.D. 
GENERAL  EXAMINATION   OF  THE   EXTERIOR  OF  THE   EYE  AND  THE 
REGION    OF  THE  EYE  ;  PERIMETRY  ;  COLOR-BLINDNESS  ;  PERIPH- 
ERAL   BLINDNESS     AND     SUBJECTIVE      VISUAL     SENSATIONS; 
AMBLYOPIA. 
Field  of  vision — Subjective  visual  sensations — Amblyopia 152  to  177 


viii  CONTENTS 

CHAPTER  V. 

Bt  Alkxakoer  Duank,  M.D. 

THE  EXTUA-OCULAR  MUSCLES. 
Physiology  of  the  eye  muscles  and  eye  movements — Tests — Nomenclature  and 
classiJication  of  the  ocular  deviations — Paralysis  of  the  individual  muscles 
— Spasm-  of  the  individual  ocular  muscles — Convergent  and  divergent 
anomalies — Comitant  heterophoria  and  squint — Derangements  of  the  asso- 
ciated parallel  movements  of  the  eyes — Conjugate  paralysis  and  spasm — 
Tremor  of  the  associated  parallel  movements,  nystagmus  and  pseudo- 
njrstagmus — anomalous  deviations  of  the  eyes — Disjunctive  movements. 
178  to  284 

CHAPTER   VI. 

By  John  E.  Weeks,  M.D. 

THE    INTRA-OCULAR    MUSCLES. 
Iris — Examination     of    the     iris — Light   reflexes — Examination    of     the    light 

reflex — Pathological   variations  in  the  pupil 285  to  319 

CHAPTER   VII. 
By  Edward  Jackson,  A.M.,  M.D. 

PERIPHERAL  AFFECTIONS  OF  THE  FIFTH,  SEVENTH,  AND  CERVICAL 
SYMPATHETIC  NERVES;  OCULAR  LESIONS  CAUSED  BY  THEM: 
TREATMENT. 

The  fifth  nerve — The  seventh  nerve — The  sympathetic  nerve — The  secretion  and 

removal  of  tears — The  lids  and  their  movements 320  to  359 

CHAPTER  VIII. 
By  H.  V.  WCrdemann,  M.D. 

DISEASES  OP  THE   RETINA    AND  OPTIC  NERVE. 
Ophthalmoscopy — Ophthalmic  signs  of  general   and  nervous  diseases — Sensory 
aflTectjons — Va.scular    lesions — Inflammation    of    the    retina — Varieties    of 
retinitis  associated  with  general  and  nervous  affections — Inflammation  of 
the  optic  nerve — Optic  nerve  atrophy 360  to  386 

CHAPTER   IX. 

By  William  G.  Spiller,  M.D. 

TUMORS  AND  OTHER  LESIONS  OF  THE  BRAIN,  ESPECIALLY  THOSE 
IN  RELATION  TO  THE  VISUAL  APPARATUS  AND  OCULAR 
NERVE.S. 

General  symptoms  of  tumor — Ix»8ions  of  the  occipital  lobe — Tumors  of  the 
parietal  lobe — Tumors  of  the  temporal  lobe — Tumors  of  the  motor  area — 
Tumors  of  the  frontal  lobe — Tumors  of  the  optic  thalamus — Tumors  of  the 
corpora  quadrigemina  and  cerebral  peduncles — Lesions  of  the  pineal  gland — 
Tumors  of  the  pituitary  body — Tumors  of  the  optic  nerve — ^Tumors  of  the 
pons  and  medulla — Tumors  of  the  cerel)ellum 387  to  433 


CONTENTS  ix 

CHAPTER  X. 
By  E.  W.  Taylor,  M.D. 

BULBAR  AND  PSEUDOBULBAR  DISEASES. 
General  remarks — General  symptomatology — Progressive  bulbar  palsy — Acute 
( apoplectiform )  bulbar  paralysis — Bulbo-pontine  hemorrhage — ^Acute 
bulbar  apoplexy — Bulbo-pontine  softening — Bulbar  paralysis  from  other 
causes — Superior  encephalitis  (Wernicke)  chronic  ophthalmoplegia — 
Pseudo  bulbar  paralysis — ^Myasthenia  gravis — Paralyzing  vertigo  (Ger- 
lier's  disease. ) 434  to  464 

CHAPTER  XI. 

By  AVilliam  Hirsch,  M.D. 

ARTERIOSCLEROSIS— MULTIPLE     SCLEROSIS— PSEUDOSCLEROSIS  —  DIF- 
FUSE  SCLEROSIS— FRIEDREICH'S  DISEASE— PARALYSIS  AGITANS. 
465  to  477 

CHAPTER   XII. 
By  F.  X.  Dercum,  M.D. 

PARASYPHILITIC     AFFECTIONS,     INSANITIES,     AND     TOXIC     ENCEPH- 
ALOPATHIES. 

The  ocular  symptoms  of  paresis — Summary — The  ocular  phenomena  of  tabes — 
Summary — The  ocular  symptoms  in  functional  insanities — Summary — The 
ocular  symptoms  of  alcoholic  intoxication — The  ocular  symptoms  of  lead 
intoxication     478  to  531 

CHAPTER  XIII. 
By  B.  Sachs,  M.D. 
Amaurotic     family     idiocy — Infantile     cerebral     palsies     (spastic     hemiplegia, 

diplegia   and  paraplegia )     532  to  544 

CHAPTER  XIV. 
By  Charles  W.  Burr,  M.D. 
Abnormalities  in  the  development  of  the  brain  and  skull — Progressive  facial 
hemiatrophy — Progressive      facial      hemihypertrophy — Other      progressive 
hypertrophies — ^Acromegaly    545  to  564 

CHAPTER  X\. 
By  T.  H.  Weisenburg,  M.D. 
DISEASES  OF   THE   SPINAL  CORD  AND  SPINAL   NERVES. 
Myelitis — Syringo     myelia — Primary    degeneration    of    the    pyramidal     tracts 
(primary    lateral    sclerosis) — Amyotrophic    lateral    sclerosis — Progressive 
ascending  unilateral  paralysis — Posterolateral  sclerosis — Spinal  cord  symp- 
toms in  pernicious  anaemia — Injuries  of  the  spinal  cord — Acute  ascending 
paralysis    (Landry's  paralysis) — Diseases  of  the  nerves — ^Multiple  neuritis. 

565  to  613 


X  CONTENTS 

CHAPTER   XVI. 

By  G.  E.  de  SciiwEiNiTz,  A.M.,  M.D. 
NEUROSES  AND  PSYCHOSES. 
Ocular  manifestations  of  hysteria — Complete  antesthesia  of  the  visual  sense,  or 
hysterical  ainnurusis — Incomplete  antpsthesia  of  the  visual  sense,  or  hysteri- 
cal amblyopia — Defective  and  painful  visual  sensation,  or  hysterical  asthe- 
nopia— Anomalies  of  accommodation,  or  hysterical  cyclospasm  and  cyclo- 
plegia — Anomalies  of  the  iris  movements  or  hysterical  pupil  phenomena — 
Anomalies  of  the  eyelid  and  eye  muscle  movements,  or  hysterical  spasms, 
contractures,  pseudo-palsies  of  the  palpebral  and  bulbar  musculature — 
Anomalies  of  convergence — Previously  unclassified  hysterical  ocular  phe- 
nomena— Concerning  the  etiological  factors  in  hysterical  ocular  stigmata — 
Ocular  manifestations  of  neurasthenia  and  traumatic  neurosis — Treatment 
of  the  ocular  disorders  of  hysteria  and  neurasthenia — The  ocular  manifes- 
tation of  hypochondriasis — The  ocular  manifestation  of  hypnotism  and 
sleep— Tests  for  detecting  pretended  amblyopia   (malingering) 614  to  696 

CHAPTER   XVII. 
By  James  Uendrik  Lloyd,  A.M.,  M.D.  « 

MIGRAINE,      TETANUS,      TETANY,      EPILEPSY,     BLINDNESS     DUE    TO 
URAEMIA    AND    OTHER    BLOOD   STATES. 

Migraine — Ophthalmic  migraine — Ophthalmoplegic  migraine — Psychical  mi- 
graine— Tetanus — Tetany — Epilepsy — Blindness  due  to  ura-mia  and  other 
blood    states    697  to  7|2 

CHAPTER  XVIII. 

NEUROSES  AND  OTHER  CONDITIONS  OCCASIONED  BY  ERRORS  OF 
REFRACTION  AND  IMBALANCE  OF  THE  EXTRA-OCULAR 
MUSCLES,  AND  THE  EFFECT  OF  LENSES  AND  OPERATIVE  PRO- 
CEDURE  IN   RELIEVING    THE   SAME. 

Part  J. 

By  S.  D.  Risley,  M.D. 
Headache ;  neuralgia  743  to  774 

Part    II. 

By  Howard  F.  Haxsell,  A.M.,  M.D. 

EPILEPSY  ;  CHOREA  ;  VERTIGO;  GASTRIC  DISTURBANCES;  SPASMODIC 
MOVEMENTS  OF    THE    HEAD   OTHER   THAN   CHOREIFORM  ;  GEN- 
ERAL  ASTHENOPIA. 
775  to  827 


CHAPTER   XIX. 

By  Wm.  Campbell  Posey,  A.B.,  M.D. 
EXOPHTHALMIC   GOITRE. 
Symptomatology — The   ocular    symptoms — Course,    prognosis    and    frequency — 

Pathology — Treatment — Bibliography   828  to  851 


CONTEJ^TS  xi 

CHAPTER  XX. 
By  Wm.  Campbell  Posey,  A.B.,  M.D. 
THE     PSYCHOLOGICAL     EFFECTS     OF    OPERATIONS    ON    THE    EYES. 
The  relief  of  insanity  by  operations  upon  the  eyes — Mental  disturbances  after 

operation  upon  the  eyes — Bibliography 852  to  866 

CHAPTER   XXL 

By  Charles  H.  Frazier,  M.D. 

THE  SURGICAL    TREATMENT  OF  INTRACRANIAL    LESIONS    CAUSING 

DISTURBANCES  OF  VISION. 
Lesions  of  the  cranial  sinuses  as  affecting  the  contents  of  the  orbit — Thrombosis 
of  the  cavernous  sinus — Tumors  of  the  occipital  lobe — Preservation  of  vision 
in  cases  of  brain  tumors — Tumors  of  the  chiasm — Operations  upon  the  cere- 
bellum for  the  relief  of  choked  disks — Surgery  of  the  seventh  nerve — Opera- 
tive treatment  of  facial  palsy — Visual  disturbance  due  to  internal  hydro- 
cephalus— Surgery  of  the  fifth  nerve — Trifacial  neuralgia,  ticdouloureux, 
867  to  905 

CHAPTER  XXII. 
By  Joseph  Sailer,  M.D. 
TREMORS,    REFLEXES,    GAITS. 
Tremors — Classification — Reflexes — Reflexes  of  the  head — Reflexes  of  the  upper 
extremity — Reflexes   of   the   lower   extremity — Reflexes   of   the   trunk — The 
literature  on  reflexes — Gaits — The  gait  as  a  symptom  of  nervous  disease — 
Methods  of   study — The   paralytic  gaits — Gaits  due   to  disturbance  of   co- 
ordination— The  gaits  of  functional  nervous  disease — Delayed  walking. 
906  to  950 

CHAPTER   XXIII. 

By  G.  L.  Walton,  M.D. 

DEGENERACY     (DEGENERATION,    CONSTITUTIONAL    DEFECTS, 

DEVIATION). 
General  considerations — The  bearing  of  constitutional  deviation  upon  the  esti- 
mation of  eye  strain  symptoms — Deviation — The  bibliographical  study  of 
constitutional  deviations — Anatomical   stigmata — Physiological   stigmata — 
Psychic  and  psycho  neurotic  stigmata 951  to  971 


THE 
EYE  AND  NERVOUS  SYSTEM 


CHAPTER    I. 

THE  INTRACRANIAL  PORTION  OF  THE  ENCEPHALIC 
NERVES,  ESPECIALLY  OF  THOSE  RELATED  TO  THE 
EYE. 

By  WILLIAM  G.  SPILLER,  M.D. 

THE    OLFACTORY    NERVE. 

The  olfactory,  or  first,  nerve  has  its  origin  in  the  regio  olfactoria  of 
tlie  Schneiderian  membrane.  After  the  fibres  constituting  this  nerve 
have  entered  the  cranium  through  the  cribriform  plate,  they  penetrate 
the  olfactory  bulb  and  terminate  in  the  olfactory  glomeruli.  The 
olfactory  nerve,  therefore,  has  a  very  short  intracranial  course,  its  intra- 
cranial connections  belonging  to  the  very  complex  central  system  of 
fibres  of  the  olfactory  apparatus. 

THE   OPTIC   NERVE. 

The  optic,  or  second,  nerve  arises  and  terminates  within  the  retina, 
and  the  structure  commonly  called  the  optic  nerve  is  in  reality  a  part 
of  the  brain. 

Decussation  of  the  Visual  Fibres  in  the  Optic  Chiasm. — Were  it  not  for 
the  opinion  of  Kolliker,  probably  there  would  not  have  been  so  much 
doubt  concerning  the  decussation  in  man  of  the  nerve-fibres  in  the  optic 
chiasm.  Kolliker  believes  that  the  decussation  is  complete,  and  his 
views  have  justly  received  much  critical  attention.  The  subject  has 
been  presented  clearly  by  Warrington  and  Dutton  in  a  contribution  by 
which  they  try  to  show  that  Kolliker  is  in  error,  and  in  this  attempt 
they  are  aided  by  the  investigations  of  many  others.  Kolliker  admits 
the  existence  of  a  small  number  of  direct  fibres  which  pass  to  the  tract  of 

1 


2  THE    EYE    AND    NERVOUS    SYSTEM 

the  same  side  as  the  optic  nerve  from  which  they  are  derived.  He  is  not 
alone  in  his  views  because,  according  to  Warrington  and  Button,  Biesia- 
decki  in  1861,  Mandelstamm  in  1873,  Scheel,  W.  Krause  in  1897,  and 
Michel,  1873  to  1890,  also  have  favored  the  view  that  the  decussation 
is  complete.  Michel's  argument  that  if  homonymous  bilateral  hemi- 
anopsia is  to  be  explained  by  the  decussation  in  the  chiasm,  the  pro- 
portion of  crossed  to  uncrossed  fibres  should  be  as  1 :  1,  and  not,  as  it  is 
admitted  to  be  by  the  adherents  of  a  partial  crossing,  as  3 :  1,  or  4 :  1,  or 
even  as  5 :  3,  is  answered  by  Warrington  and  Button.  In  a  case  studied 
bv  them  there  was  not  any  marked  difference  between  the  amount  of 
degeneration  in  the  two  optic  tracts,  although  only  one  optic  nerve  was 
diseased.  Furthermore,  they  point  out,  that  owing  to  the  limitation 
of  the  field  of  vision,  the  outer  and  lower  parts  of  the  retina  are  prob- 
ably less  developed  than  the  inner  and  lower,  and,  as  Michel  himself 
has  found,  the  number  of  fibres  passing  to  different  sections  of  the  retina 
is  very  variable,  being  least  in  the  outer  segment.  They  refer  to 
Michel's  statement  that  no  anatomical  evidence  has  yet  been  produced 
demonstrating  the  double  representation  of  the  macula  in  the  cerebral 
cortex,  which  is  required  by  the  theory  of  semi-decussation  in  order  to 
explain  the  freedom  of  the  fixation  point  in  cases  of  hemianopsia,  and 
they  remark  tliat  Michel's  hypothesis  cannot  be  regarded  as  giving  a 
more  satisfactory  explanation.  They  refer  also  to  Griitzner's  work  on 
this  subject.  This  investigator  formed  models  of  the  chiasm  in  which 
one-half  of  the  fibres  were  made  to  cross  and  the  other  half  to  remain  on 
the  same  side.  The  models  were  then  embedded  in  paraffin  and  cut 
into  a  series  of  liorizontal  sections.  From  a  study  of  these  sections 
Grutzner  concluded  that  if  he  had  not  known  that  the  decussation  was 
partial  he  would  have  supposed  it  to  be  complete. 

Warrington  and  Button's  case  was  one  in  which  enucleation  of  the 
right  eyeball  had  been  performed  on  account  of  trauma.  The  chiasm 
with  the  optic  nerves  attached  was  cut  in  a  complete  series  of  about  500 
horizontal  sections.  In  the  Sections  about  the  median  plane  the  atrophy 
from  the  right  nerve  was  seen  spreading  in  the  central  portion  of  the 
same-sided  tract,  and  occupying  the  front  and  back  of  the  chiasm,  its 
extent  being  larger  in  the  latter  situation.  Even  though  Michel  has 
shown  that  the  fibres  of  the  optic  nerve,  as  they  enter  the  chiasm,  turn 
sharply  at  a  right  angle,  and  again  at  another  right  angle  when  entering 
the  tract  of  the  opposite  side,  Warrington  and  Button  believe  they  were 
able  to  detect  groups  of  fibres  passing  along  the  outer  border  of  the 
nerve  and  into  the  tract  of  the  same  side;  and  they  state  that  all  ob- 
servers (Purtsohor,  Wieting,  Siemerling,  Williamson)  with  the  modern 


THE  ENCEPHALIC  NERVES  3 

methods,  with  the  single  exception  of  Michel,  are  of  the  opinion  that 
degeneration  occurs  in  both  optic  tracts  after  destruction  of  one  optic 
nerve ;  and  the  crossed  fibres  usually  occupy  the  centre  of  the  tract,  the 
uncrossed  the  periphery.^ 

The  findings  that  Probst  obtained  in  his  experiments  on  the  optic 
fibres  resemble  very  closely  those  of  von  Monakow.  Probst  believes  that 
the  decussation  of  the  optic  fibres  in  the  dog  and  cat  is  a  partial  one. 
The  medial-central  fibres  of  the  optic  nerve  cross  first,  and  later  the 
dorsal  and  ventral  fibres,  while  the  lateral  fibres  do  not  decussate. 
Most  of  the  optic  fibres  cross  to  the  opposite  optic  tract. 

When  an  eyeball  is  removed  degenerated  optic  fibres  are  found  in 
both  external  geniculate  bodies,  but  more  are  found  in  the  opposite 
ganglion. 

The  arm  of  the  anterior  colliculus  of  the  corpora  quadrigemina 
contains  chiefly  crossed,  but  also  uncrossed,  optic  fibres. 

In  studying  the  optic  chiasm  of  a  new-born  child,  stained  by  the 
haematoxylin  method  of  Weigert,  a  partial  decussation  seems  to  me  very 
probable.  The  course  of  the  nerve-fibres  in  the  optic  nerves,  chiasm,  and 
optic  tracts  may  be  better  seen  in  specimens  obtained  from  a  new-born 
child  than  in  those  from  an  adult,  because  the  number  of  stained  fibres 
is  less  in  the  former.  A  careful  study  of  the  literature  on  decussation 
of  the  optic  fibres  seems  to  show  that  the  weight  of  evidence  is  in  favor 
of  a  partial  decussation,  and  in  support  of  this  view  are  the  important 
studies  of  Ramon  y  Cajal. 

Parsons,  from  his  investigations  on  monkeys,  concludes  that,  speak- 
ing generally,  the  fibres  derived  from  various  parts  of  the  retina  retain 
their  relative  position  in  the  optic  nerve, — i.e.,  temporal  fibres  remain 
external,  nasal  internal.  The  temporal  fibres  tend  towards  the  dorsal 
side  as  they  approach  the  chiasm,  and  the  nasal  towards  the  ventral 
side.  A  further  dislocation,  affecting  the  temporal  fibres  most,  occurs 
as  the  result  of  the  intercalation  of  the  papillomacular  bundle.  There 
may  be  some  variation  in  different  individuals.^ 

According  to  Henschen,  as  given  by  Wilbrand  and  Saenger,  the 
uncrossed  bundle  (not  papillomacular  fibres)  near  the  eyeball  is  divided 
into  two  parts,  between  which  are  placed  the  fibres  of  the  papillomacular 
bundle,  more  centrally  the  two  portions  of  the  uncrossed  bundle  ap- 
proach one  another  and  in  the  posterior  half  of  the  optic  nerve  in  trans- 
verse section  unite  and  assume  a  sickle  shape  and  are  situated  in  the 

^  Warrington  and  Dutton,  Brain,  1900,  vol.  xxiii.,  No.  92,  p.  642. 
'Lancet.  April  16,  1904.  p.  1029. 


4  THE   EYE   AND    NERVOUS    SYSTEM 

ventrolateral  jwrtion  of  the  nerve.  The  crossed  bundle  (not  papillo- 
niacular  bundle)  has  a  dorso-medial  position  throughout  the  optic  nerve. 

The  structure  of  the  chiasm  is  very  complicated.  Probably  the  most 
important  fact  is  that  the  uncrossed  fibres  are  mingled  with  the  crossed 
in  the  lateral  portion  of  the  chiasm,  so  that  nasal  hemianopsia  can  not 
be  caused  by  a  lesion  of  the  chiasm. 

There  is  much  diflFcrence  of  opinion,  as  shown  by  Wilbrand  and 
Saenger,  regarding  the  existence  of  a  separate  bundle  of  uncrossed  fibres 
in  the  optic  tract,  some  believing  that  the  crossed  are  mingled  with  the 
uncrossed  fibres.  Ilenschen  is  of  the  opinion  that  the  uncrossed  bundle 
occupies  the  superior  and  external  position  and  is  distinct  from  the 
crossed  fibres. 

Decussation  of  the  Macular  Fibres.  — Dean  and  Usher  have  attempted 
to  determine  the  position  of  the  optic  nerve-fibres  in  monkeys.  Vossius, 
in  a  case  of  symmetrical  central  scotoma  in  man,  found  that  the  macular 
fibres  lie  close  under  the  dorsal  margin  of  the  chiasm.  One  of  the  cases 
reported  by  Dean  and  Usher  corresponds  closely  with  Vossius's  descrip- 
tion. Bunge  found,  they  say,  that  the  papillomacular  bundle  main- 
tains a  dorsal  position  throughout  the  chiasm  in  man.  Dean  and  Usher 
give  the  course  of  the  macular  fibres  in  the  chiasm  in  the  monkey 
as  follows:  Anteriorly  in  the  chiasm  they  occupy  the  central  area  of 
both  halves.  Soon  after  entering  the  chiasm  they  pass  towards  the 
dorsum  before  any  crossing  has  occurred.  The  crossing  takes  place 
first  at  the  dorsal  periphery,  at  some  distance  behind  the  front  of  the 
chiasm.  On  j)roceeding  backward,  though  still  partly  remaining  dorsal, 
they  spread  from  the  dorsal  surface  in  a  ventral  direction.  The  macular 
fibres  never  reacli  the  ventral  periphery.  These  investigators  were 
unable  to  determine  whether  the  macular  fibres  cross  wholly  or  par^ 
tially  in  the  chiasm,  as  they  could  not  be  certain  whether  the  wound 
was  limited  to  the  macular  fibres,  or  whether  it  had  damaged  only  a 
portion  of  these.'' 

According  to  Ilenschen,  as  quoted  by  von  Monakow,  the  bundle  from 
the  macula  lutoa  between  the  retina  and  the  primary  optic  centres  lies 
in  the  middle  of  the  transverse  sections  of  the  optic  nerve  and  optic 
tract,  whereas  the  rest  of  the  retina  is  represented  in  the  periphery  of 
these  structures. 

Wilbrand  and  Saenger  describe  the  course  of  the  papillomacular 
bundle  according  to  Uhthoff  as  follows:  In  the  forward  portion  of  the 
optic  nerve  the  bundle  lies  in  wedge  form  in  the  outer  and  lower  part 


•Dean  an«l  Usher.  Brnin.  190.3.  vol.  xxvi.,  No.  104.  p.  524. 


Fig.  1.— Case  of  complete  absence  of  the  visual  system;  other  parts  of  the  body,  except  the 
external  genitalia,  were  well  developed.  The  age  of  this  patient  was  tweuty-two  years.  The  limbs 
were  sjiastic. 


Fig.  2.— Base  of  the  brain  from  the  case  of  complete  absence  of  the  visual  system, 
indication  of  optic  tracts,  chiasm,  or  optic  herves. 


There  is  no 


Fig.  3.— Portion  of  the  brain  from  the  case  of  complete  absence  of  the  visual  system.  A  part  of  the 
rJKht  temporal  lol)o  hiis  lx?en  cut  away,  in  order  to  show  the  absence  of  the  optic  tract  and  external 
geniculate  body.    The  internal  geniculate  Iwdy  is  well  developed. 


Fic.  4.— I'ortion  of  the  brain  from  a  case  of  complete  absence  of  the  visual  system.  The  pul- 
viiiar  is  of  good  size  on  each  side.  The  anterior  colliculi  of  the  corpora  quadrigemina  appear  smaller 
than  the  posterior,  because  they  were  further  from  the  camera. 


THE   ENCEPHALIC   NERVES  5 

of  the  nerve,  with  the  point  of  the  wedge  toward  the  central  vessels,  and 
from  here  posteriorly  gradually  assumes  a  crescent  shape,  the  ends  of 
the  crescent  withdrawing  from  the  periphery.  After  the  exit  of  the 
central  vessels  from  the  optic  nerve  the  bimdle  gradually  assumes  a 
vertically  oval  form,  and  becoines  centrally  placed  in  the  posterior  or- 
bital part  of  the  nerve.  There  are  variations  in  different  cases  as 
regards  the  point  at  which  the  bundle  becomes  central.  The  bundle  is 
central  also  in  the  intracranial  part  of  the  optic  nerve,  but  because  in 
this  portion  the  nerve  is  more  oval  the  bundle  has  a  greater  transverse 
than  a  vertical  diameter.     In  the  tract  the  bundle  occupies  the  centre. 

PRIMARY  OPTIC  CENTRES. 

The  fibres  of  the  optic  nerve  end  chiefly  in  the  external  geniculate 
body,  but  some  pass  to  the  posterior  part  of  the  optic  thalamus  known 
as  the  pulvinar  and  to  the  anterior  colliculus  of  the  corpora  quadri- 
gemina.  The  fibres  to  the  latter  structure  in  man  cannot  be  numerous, 
although  they  are  in  the  rabbit.  In  a  ease  studied  by  me  in  which 
the  visual  system  was  absent  in  an  adult  there  was  no  external  geniculate 
body  and  no  trace  of  either  optic  nerve ;  the  anterior  colliculi  of  the 
corpora  quadrigemina  and  the  pulvinar  on  each  side  were  well  formed. 

The  external  geniculate  body,  the  anterior  colliculus  of  the  corpora 
quadrigemina,  and  the  pulvinar  are  known  as  the  ])rimary  optic  centres, 
and  from  these  bodies  other  fibres  pass  to  the  visual  cortex  of  the  brain, 
although  they  contain  also  the  ramifications  of  fibres  that  arise  in  the 
visual  cortex  (Edinger).  The  optic  tract  is  formed  chiefly  by  nerve- 
fibres  that  terminate  in  the  primary  visual  centres,  but  according  to 
Edinger  they  contain  also,  certainly  in  the  rabbit  and  cat  and  probably 
in  man,  fibres  that  arise  in  the  corpora  quadrigemina.  These  fibres  frojn 
the  primary  visual  centres  terminate  in  the  retina.  According  tovonMon- 
akow  as  much  as  80  per  cent,  of  the  visual  fibres  in  man  terminate  in 
the  external  geniculate  body,  and  all  the  visual  fibres  of  the  optic  tract 
terminate  in  the  primary  visual  centres.  Von  Monakow  is  authority 
for  the  statement  that  hemianopsia  as  the  result  of  a  lesion  confined  to 
the  anterior  colliculus  of  the  corpora  quadrigemina  has  not  been  ob- 
served, and  it  is  doubtful  whether  a  lesion  confined  to  the  pulvinar  and 
not  implicating  the  external  geniculate  body  causes  hemianopsia. 

Some  optic  fibres  pass  above  and  below  the  external  geniculate  body 
as  stratum  zonale  to  the  pulvinar  of  the  optic  thalamus  and  near  to  the 
ganglion  habenulse,  with  which,  however,  they  do  not  come  in  contact 
(Probst)." 

*  Probst,  Monatsschrift  fiir  Psyehiatrie  und  Neurologic,  September,  1900,  vol. 
viii.  p.  165. 


6  THE    EYE    AND    NERVOUS    SYSTEM 

"PUPILLARY   FIBRES"  IN   THE   VISUAL    SYSTEM. 

Von  Gudden  showed  that  the  optic  nerve  contains  thick  and  slender 
nen'e-fibres,  and,  according  to  von  Monakow,  the  former  pass  almost 
entirely  to  the  anterior  colliculi  of  the  corpora  quadrigemina.  These 
thicker  fibres  are  supposed  to  serve  a  special  function, — viz.,  the  contrac- 
tion of  the  iris  in  the  light  reflex.  They  have  been  recently  studied  by 
Reichardt.  It  is  remarkable  that  these  "  pupillary  fibres"  should  be 
present  in  the  optic  nerve  and  optic  tract,  and  although  they  have  not 
been  actually  demonstrated  much  evidence  of  their  existence  has  been 
collected.  They  were  first  recognized  by  von  Gudden  and  von  Bech- 
terew,  and  are  believed  to  have  no  part  in  vision.  Their  function  is  to 
convey  centripetal  impulses  to  the  pupillary  reflex  centre  wherever  that 
is  located.  The  results  obtained  by  Massaut  in  his  study  of  the 
"  pupillary  fibres"  are  so  extraordinary  that  they  may  be  referred  to 
in  this  connection.  This  investigator  extirpated  the  iris  in  rabbits,  and 
found  degenerated  fibres  by  the  Marchi  method  in  the  optic  nerve, 
which  he  regarded  as  "  pupillary  fibres."  The  interpretation  of  this 
degeneration  offered  by  him  is  accepted  with  some  difficulty,  inasmuch 
as  a  degeneration  of  one  group  of  fibres,  the  centripetal,  is  supposed  to 
have  occurred  as  a  result  of  a  lesion  of  the  distal  ends  of  another  group 
of  fibres,  the  centrifugal.  This,  in  my  experience,  is  so  unlike  what 
occurs  elsewhere  in  the  nervous  system  that  I  can  accept  Massaut's 
interpretation  only  with  much  hesitation,  especially  as  it  is  well 
known  that  the  Marchi  method  is  at  times  the  source  of  many  erroneous 
conclusions.     (See  chapter  vi.,  p.  291.) 

Westphal  found  that  the  large  fibres  in  the  optic  nerve  receive  their 
medullary  sheaths  sooner  than  the  fine  fibres,  and  he  also  concluded  that 
the  former  are  "  pupillary  fibres."  Reichardt  points  out  that  disease 
of  the  optic  nerve  may  cause  complete  blindness  and  yet  leave  the  iritic 
reflex  intact,  and  this  seems  to  indicate  that  there  are  distinct  systems 
of  fibres  in  the  optic  nerve.  In  compression  of  the  optic  nerve  the  iritic 
reflex  is  much  less  affected  than  vision,  which  may  indicate  that  the 
"pupillary  fibres"  are  more  resistant.  When  acute  inflammation  of  the 
optic  nerve  is  disappearing  improvement  is  usually  noticed  in  the  reac- 
tion of  the  iris  before  vision  begins  to  return. 

Piltz  doscrilx^s  a  case  in  which  blindness  had  existed  nine  years 
and  was  caused  by  optic  atrophy,  and  yet  the  iritic  response  to  light  was 
preserved.  Bach  observed  a  case  of  tabes  in  which  the  reflex  action  of 
the  iris  to  light  was  better  in  the  eye  in  which  vision  was  more  affected 
and  the  optic  nerve  atrophy  was  more  advanced.  All  this  seems  to  indi- 
cate that  there  are  probably  special  fibres  in  the  optic  nerve  for  the 


THE  ENCEPHALIC  NERVES  7 

iritic  reflex.  Reichardt  also  has  observed  an  important  case.  A  man 
had  atrophy  of  the  left  optic  nerve,  and  until  four  weeks  before  the 
loss  of  sight  in  the  left  eye  became  complete  he  had  normal  pupillary 
reaction,  and  even  later  the  reaction  was  only  somewhat  tardy.  The 
left  optic  nerve  was  found  to  be  small,  and  the  reduction  in  size  was 
caused  chiefly  by  the  degeneration  of  the  fine  fibres.  This  case  seems 
to  afford  some  support  for  the  widely  accepted  teaching  that  the  fine 
fibres  especially  are  concerned  with  vision. 

Reichardt  believes  that  the  "  pupillary  fibres"  of  the  optic  nerve  may 
alone  degenerate,  or  at  least  be  more  affected,  and  in  such  a  case  vision 
would  be  preserved  although  the  iritic  reflex  would  be  lost. 

According  to  Bach  and  Meyer,  it  is  probable  that  the  pupillary  reflex 
may  be  stimulated  from  any  part  of  the  retina,  although  the  macula 
region  is  jirobably  the  most  important.  The  "  pupillary  fibres"  are 
mingled  with  the  visual  fibres  in  the  optic  nerve,  although  Bach  be- 
lieves a  certain  order  is  preserved,  so  that  those  fibres  that  come  from 
the  temporal  side  of  the  retina  remain  in  the  temporal  side  of  the  optic 
nerve.  In  man  the  decussation  of  the  "  pupillary  fibres,"  as  is  that  of 
the  visual  fibres,  is  partial,  although  most  of  the  fibres  of  each  variety 
cross.  The  visual  and  "  pupillary"  fibres  are  associated  in  the  optic 
tract  as  far  as  the  external  geniculate  body,  but  here  they  separate. 

It  may  be  said  that  no  one  has  ever  been  able  to  trace  the  connec- 
tion between  the  optic  nerve  and  the  oculomotor  nucleus.  It  has  been 
supposed  that  this  connection  is  made  by  means  of  Meynert's  fibres 
passing  in  a  curve  below  the  aqueduct  of  Sylvius.  Some  authors  have 
accepted  this  view  as  an  established  fact,  forgetting  or  being  in  igno- 
rance of  the  uncertainty  concerning  this  subject.  No  one  disputes  that 
these  fibres  of  Meynert  from  their  position  might  serve  as  the  connect- 
ing link,  but  there  are  other  fibres  in  this  region  which  might  have  this 
function. 

Probst,  whose  anatomical  studies  of  the  nervous  system  are  so  well 
kno^vn,  says  that  when  an  eyeball  has  been  enucleated  degenerated  fibres 
of  the  optic  nerve  cannot  be  followed  to  the  oculomotor  nucleus.  The 
connection  of  the  optic  fibres  with  the  oculomotor  nucleus  must  there- 
fore be  by  means  of  a  short  system  of  fibres  (Schaltsystem). 

It  is  supposed  that  the  "  pupillary  fibres"  pass  from  the  optic  tract 
to  the  corpora  quadrigemina  through  the  arm  of  the  anterior  colliculus 
of  this  body,  but  the  further  course  of  these  fibres  is  unknown.  Bern- 
heimer  believes  he  has  been  able  to  trace  these  fibres  to  the  region 
below  the  aqueduct  of  Sylvius  and  near  to  the  oculomotor  nucleus,  but 
Dimmer,  Bach,  and  others  have  followed  the  fibres  only  so  far  as  the 


8  THE    EYE    AND    NEKVOUS    SYSTEM 

armaudtheupperpartof  theanteriorcolliculus.  The  "  pupillary  fibres" 
are  believed  hy  some  to  be  in  relation  with  the  stratum  zouale  of  the 
pulvinar  of  the  optic  thalamus,  with  the  corpus  hypothalamicum,  and 
with  the  ganglion  habenula3,  but  this  relation  seems  improbable. 

Edinger,  in  the  latest  edition  of  his  lectures  upon  the  anatomy  of 
the  nervous  system,  expresses  himself  as  in  doubt  concerning  the  con- 
nection of  the  ''  pupillary  fibres"  of  the  optic  nerve  with  the  oculomotor 
nerve.  While  acknowledging  that  the  **  pupillary  fibres"  probably  pass 
through  the  anterior  arm  of  the  corpora  quadrigemina,  he  is  uncertain 
concerning  the  manner  of  their  connection  with  the  oculomotor  nerve. 
Von  Monakow  also  is  cautious  concerning  this  supposed  connection. 

THE   CORTICAL    CENTRES    OF   VISION. 

One  of  the  earliest  cases,  if  not  the  earliest  case,  of  bilateral  hemi- 
anopsia from  a  unilateral  lesion  was  that  published  by  Lewick;^  the 
lesion  was  an  abscess  of  the  right  occipital  lobe. 

Ilenschen  believes  that  only  a  lesion  of  the  cortex  of  the  calcarine 
fissure,  and  especially  of  its  anterior  two-thirds,  causes  persistent  bilat- 
eral homonymous  hemianopsia.  Lesions  of  other  parts  of  the  cortex  of 
the  occipital  lobe  may  exist  without  such  visual  disturbance.  Von 
Monakow  says  that  Huguenin  assumed  that  destruction  of  the  cortex  of 
the  calcarine  fissure  causes  hemianopsia.  Later  investigators  extended 
the  visual  cortex ;  von  Monakow  at  first  included  the  cuneus,  lingual 
lobule,  and  descending  gyrus.  Seguin  limited  the  visual  cortex  to  the 
cuneus;  Xothnagel  limited  it  to  the  cuneus  and  first  occipital  gyrus; 
Vialet  to  the  entire  medial  part  of  the  occipital  lobe;  Ferrier,  Ange- 
lucci,  Bianchi,  Reinhard,  Luciani,  Seppilli  included  the  cuneus  and 
the  medial  part  of  the  occi])ital  lobe;  the  first  extended  it  to  the  third 
occipital  gyrus  and  even  the  angular  gyrus. 

Ilenschen  says  that  all  the  lateral  surface  of  the  occipital  lobe  and 
the  angular  gyrus  may  be  destroyed  without  causing  hemianopsia.  In 
one  of  his  cases  a  lesion  was  confined  to  the  calcarine  cortex,  and  yet 
hemianopsia  was  caused  by  this  lesion.  In  another  case  the  calcarine 
cortex  was  destroyed  and  hemianopsia  was  caused  thereby,  while  ex- 
tensive destruction  of  the  lateral  surface  of  the  other  hemisphere,  pre- 
sumably the  occipital  lobe  is  meant,  did  not  cause  hemianopsia.  The 
cases  Ilenschen  refers  to  seem  to  show  that  anopsia  in  the  lower  quad- 
rant may  l)o  caused  by  a  lesion  of  the  dorsal  part  of  the  external 
geniculate  body,  and  that  in  such  cases  normal  fibres  may  be  traced 


'Anier.  Jour,  of  the  Med.  Sci..  180G. 


THE   ENCEPHALIC   NERVES  9 

from  the  lower  part  of  the  external  geniculate  body  to  the  inferior  lip 
of  the  ealcarine  fissure;  by  means  of  these  fibres  are  conveyed  the 
impressions  made  on  the  inferior  part  of  the  retina.  His  observations 
seem  to  show  that  the  fibres  of  the  superior  lip  of  the  ealcarine  fissure 
are  in  connection  with  the  superior  part  of  the  retina.  Henschen  com- 
bats Ferrier,  who  places  the  visual  centre  in  the  angular  gyrus,  and 
Nothnagel  and  Munk,  who  regard  the  first  occipital  convolution  as 
part  of  the  visual  centre.  He  has  observed  anopsia  of  the  superior 
quadrant  caused  by  softening  of  the  inferior  lip  of  the  ealcarine  fissure. 
In  another  case  horizontal  scotoma  was  caused  by  a  lesion  confined  to 
the  base  of  the  ealcarine  fissure. 

These  investigations  of  Henschen  are  extremely  important,  and 
seem  to  shoSv  that  the  upper  part  of  the  retina  is  represented  in  the 
upper  part  of  the  external  geniculate  body  and  in  the  upper  lip  of  the 
ealcarine  fissure ;  that  the  lower  part  of  the  retina  is  represented  in  the 
lower  part  of  the  external  geniculate  body  and  in  the  lower  lip  of  the 
ealcarine  fissure ;  and  that  the  horizontal  zone  of  the  retina,  midway 
between  the  upper  and  lower  portions,  is  represented  in  the  base  of  the 
ealcarine  fissure.  From  this  it  follows  that  the  fibres  from  the  different 
portions  of  the  retina  in  the  optic  nerve  and  optic  tract  must  preserve 
their  relative  positions,** 

The  case  of  quadrant  anopsia,  recently  reported  by  Beevor  and 
Collier  (Brain,  1904),  with  necropsy  and  careful  microscopical  study, 
is  exceedingly  valuable  in  determining  the  location  of  the  cortical  lesion 
causing  quadrant  anopsia.  A  case  similar  to  this  has  been  reported 
by  Hun.  The  only  symptom  of  nervous  disease  presented  while  Beevor 
and  Collier's  patient  was  under  observation  was  blindness  of  the  left 
upper  quadrants  of  both  visual  fields,  the  fixation  point  escaping.  This 
condition  remained  constant  nearly  two  years.  An  occlusive  lesion  of 
the  right  posterior  ealcarine  artery  had  caused  destruction  of  the  cor- 
tex (1)  of  the  right  fusiform  lobe  for  its  posterior  two  centimetres,  (2) 
of  the  right  lingual  lobe  from  the  junction  of  the  ealcarine  and  parieto- 
occipital fissures  to  the  pole  of  the  hemisphere,  (3)  of  the  whole  cortex 
in  the  depth  of  the  ealcarine  fissure,  (4)  of  the  greater  part  of  the 
inferior  cuneal  gyrus,  small  areas  only  at  the  anterior  and  posterior 
limits  of  this  gyrus  being  free.  The  necrosis  did  not  involve  the  optic 
radiations  at  any  point.  The  only  parts  of  the  cortex  of  the  mesial 
aspect  of  the  occipital  lobe  which  had  escaped  destruction  were  the 
upper  two-thirds  of  the  cuneus  and  the  anterior  and  ventral  portion  of 


« Henschen.  Scmainc  Mi'dinale.  April  22.  190.'}.  No.  10.  p.  125. 


10  THE    EYE    AND    NERVOUS    SYSTEM 

the  fusiform  gyrus.  The  lower  quadrants  of  the  visual  fields  were 
entirely  unaffected.  The  case  seems  to  show  that  the  cortex  of  the  upper 
two-thirds  of  the  cuneus  is  the  visual  centre  for  the  lower  quadrants. 

In  this  case  the  cortex  lining  the  calcarine  fissure  was  completely 
necrotic.  The  primary  half-vision  centre,  the  authors  think,  cannot 
therefore  be  limited  to  tlie  calcarine  cortex,  nor  the  macula  exclusively 
represented  in  the  anterior  part  of  this  region.  The  sharp  distinctions 
of  the  upper  and  lower  fields  as  related  to  the  calcarine  fissure,  made 
by  Ilenschen  would  seem  by  this  case  to  need  some  modification,  for  the 
lower  part  of  the  retina  would  appear  to  be  represented  in  the  upper 
lip  of  the  calcarine  fissure. 

Harris  "  thinks  it  is  possible  to  have  a  loss  of  the  lower  quadrant  on 
the  one  side  and  of  the  upper  quadrant  on  the  other  from  a  lesion  in 
each  cuneus,  and  he  says  such  a  case  has  been  recorded  by  Weymann, 
but  how  the  function  of  only  one  quadrant  of  the  retina  of  one  eye  can 
be  lost  by  a  lesion  of  the  visual  cortex  is  to  me  unintelligible.  Even 
granting  that  the  crossed  and  uncrossed  fibres  remain  separate  so  far  as 
the  optic  tract,  we  have  no  proof  that  they  are  ununited  in  the  optic 
radiations  and  visual  cortex.  A  lesion  in  these  parts  seems  to  cause 
disturbance  of  vision  in  each  eye. 

Wilbrand  thinks  that  every  part  of  the  macula  is  represented  both 
in  the  right  and  left  visual  cortical  areas,  but  in  cases  in  which  the 
dividing  line  in  hemianopsia  passes  directly  through  the  fixation  point 
the  double  representation  does  not  occur.  It  has  also  been  suggested 
(Forster)  that  the  macula  region  of  the  cortex  is  better  supplied  with 
blood-vessels  than  other  parts  of  the  visual  cortex,  and  receives  nour- 
ishment through  the  posterior  cerebral  artery  and  the  occipital  branch 
of  the  artery  of  the  Sylvian  fossa.  Von  Monakow  thinks  that  the  rep- 
resentation of  the  macula  is  not  confined  to  the  middle  of  the  visual 
centre,  nor  to  its  periphery,  but  he  believes  that  all  parts  of  the  occipital 
lobe  and  possibly  even  the  posterior  part  of  the  angular  gyrus  are  repre- 
sented in  the  macular  field,  and  that  the  macular  field  may  extend  far 
beyond  the  limit  of  the  area  usually  recognized  as  the  visual  region  of 
the  cortex.  lie  does  not  accept  Ilensclien's  views  that  the  macula  is 
represented  in  the  anterior  portion  of  the  cortex  of  the  calcarine  fissure. 

Von  Monakow  says  that  a  careful  examination  of  the  reported  cases 
shows  that  hemianopsia  is  most  likely  to  occur  and  to  be  persisting  per- 
haps only  from  a  lesion  in  the  calcarine  fissure,  but  there  are  some 
cases    in    which    hemianopsia    has    occurred    from    lesion    elsewhere, 

'  IJrain,  vol.  xx.,  1897,  p.  .308. 


THE  ENCEPHALIC   NERVES  11 

although  it  may  be  possible  that  in  these  cases  the  hemianopsia  was 
caused  by  implication  of  the  optic  radiations,  but  the  same  is  true  when 
the  lesion  is  in  the  calcarine  fissure.  Von  Monakow  thinks  that  in 
every  persisting  cortical  hemianopsia  there  is  partial  injury  of  some 
part  of  the  occipital  lobe  and — which  is  more  important — also  a  lesion 
of  the  optic  radiations.^ 

Few  anatomists  place  the  visual  centre  in  the  angular  gyrus,  but 
there  is  more  doubt  as  to  the  implication  of  the  lateral  surface  of  the 
occipital  lobe  in  the  visual  field,  and  as  to  the  extent  of  the  surface 
involved. 

Gallemaerts  has  found  that  after  enucleation  or  atrophy  of  an  eye- 
ball the  cells  of  the  visual  cortex  become  less  numerous,  and  this  is  to 
be  seen  in  both  occipital  lobes  after  a  lesion  of  one  eyeball.  This  find- 
ing confirms  the  partial  decussation  at  the  optic  chiasm.  The  crossed 
fasciculus  contains  more  fibres  than  the  direct  fasciculus ;  therefore  the 
greatest  diminution  of  nerve  cells  is  in  the  visual  cortex  opposite  to  the 
side  on  which  the  eyeball  has  been  lost.^ 

From  a  study  of  the  diminution  in  the  number  of  nerve-cells  Galle- 
maerts concludes  that  the  fusiform  lobule,  the  angular  gyrus,  and  the 
descending  gyrus  (which  is  on  the  outer  surface  of  the  occipital  lobe 
and  at  the  occipital  point)  are  not  part  of  the  visual  centre,  but  that  the 
visual  centre  consists  of  the  lingual  lobule  and  the  cuneus.  In  these 
convolutions,  especially  near  the  calcarine  fissure,  the  lesions  were  very 
distinct.  The  diminution  of  the  nerve-cells  in  the  visual  cortex  after 
destruction  of  the  eyeball  occurs  only  when  years  have  passed  since  the 
lesion  developed,  and  all  the  layers  of  nerve-cells  in  the  visual  cortex 
are  affected  after  a  sufficiently  long  period. 

Bernheimer  includes  the  cuneus,  the  cortex  of  the  calcarine  fissure, 
the  lingual  lobe,  and  the  descending  gyrus  in  the  visual  cortex. 

The  reader  is  referred  to  the  article  by  Dr.  Charles  K.  Mills  for 
further  remarks  on  the  lower  and  higher  cortical  visual  areas. 

THE   OCULOMOTOR   NERVE. 
The  nucleus  of  the  oculomotor,  or  third,  nerve  lies  below  the  aque- 
duct of  Sylvius.    This  group  of  cells  consists  of  a  median  nucleus  with 
a  lateral  nucleus  on  each  side  of  it.     Anterior  to  these  a  group  of  small 
cells  is  found  on  each  side  of  the  median  line,  and  is  known  as  the 


8  Von  Monakow,  Gehirnpathologie. 

"Gallemaerts,   Bulletin   de   I'Acadgmie   Royale  de  M6decine   de   Belgique,    1902, 
iv.  series,  vol.  xvi.  p.  267. 


12  THE    EYE    AND    NERVOUS    SYSTEM 

Edinger-Westphal  nucleus.  It  has  been  considered  as  the  source  of 
the  innervation  of  tlie  inner  muscles  of  the  eyeballs,  but  there  is  doubt 
whether  it  should  be  regarded  as  a  part  of  the  oculomotor  nucleus.  The 
fibres  of  the  oculomotor  nerve  pass  ventrallj  through  the  tegmentum  of 
the  cerebral  jx^duncle  and  reach  the  surface  at  the  inner  border  of  the 
foot  of  the  cerebral  peduncle  (crusta).  The  individual  fibres  forming 
this  nerve  are  not  closely  united  even  at  the  exit  of  the  nerve  from  the 
cerebral  j)eduncle,  and  it  is  because  of  this  that  paralysis  of  one  or 
more  of  the  muscles  innervated  by  the  oculomotor  nerve  may  be  caused 
by  basal  meningitis  without  paralysis  of  the  whole  muscular  supply  of 
this  nerve,  and  such  paralysis  may  mistakenly  be  attributed  to  nuclear 
disease. 

There  is  some  doubt  whether  the  muscles  of  the  eye  innervated  by 
the  oculomotor  nerve  are  separately  represented  in  the  nucleus  of  this 
nerve.  The  knowledge  concerning  this  subject  that  has  been  obtained 
depends  chiefly  on  investigations  upon  monkeys.  Siemerling  believes 
that  the  investigations  of  Bach,  Bernheimer,  van  Gehuchten,  and  van 
Biervliet  have  not  led  to  uniform  results.  The  oculomotor  nucleus, 
according  to  Siemerling,^''  is  merely  the  place  for  the  transference  of 
injpulses,  and  we  are  not  in  a  position  to  state  which  parts  are  concerned 
with  the  innervation  of  the  individual  muscles.  Edinger's  views  as 
contained  in  the  seventh  edition  of  his  lectures  upon  the  structure  of  the 
nervous  system  are  as  follows:  The  most  frontal  group  supplies  the 
levator  })alpebra}  and  rectus  superior  of  the  same  side.  From  the  caudal 
}X)rtion  arise  the  fibres  for  the  rectus  inferior  and  the  trochlearis  of 
the  opposite  side.  The  intervening  cells  give  origin  to  both  direct  and 
crossed  axis  cylinder  processes,  so  that  each  obliquus  inferior  and  each 
rectus  internus  receives  fibres  from  the  right  and  left  lateral  group  of 
cells.  The  nerve-cells  for  the  rectus  internus  are  on  each  side  so  near 
the  large  cell  median  nucleus  that  Edinger  believes  the  three  groups 
together  may  form  a  centre  for  convergence.  All  the  external  ocular 
muscles  he  believes  are  supplied  from  the  nuclei  of  the  oculomotorius, 
trochlearis  and  abducens.  The  fibres  for  the  inner  ocular  muscles  (iris 
and  ciliary  muscle)  arise  in  the  ciliary  ganglion,  and  fine  oculomotor 
fibres  terminate  in  this  ganglion ;  ])ossibly  these  fibres  arise  in  the 
small  cell  frontal  groups. 

Edinger  says  it  is  not  certain  whether  fibres  pass  directly  from  the 
oculomotor  nucleus  to  the  iris,  or  whether  they  are  interrupted  in  the 
ciliary  ganglion ;  the  latter  seems  the  more  probable.     Bernheimer  has 

'"  Archiv  fiir  Ppychiatrie,  vol.  xl..  No.  4,  p.  61. 


THE   ENCEPHALIC   NERVES  13 

caused  paralysis  of  the  iris  in  the  monkey  by  a  lesion  of  the  frontal 
portion  of  the  oculomotor  nucleus. 

The  connection  of  the  cortical  centres  with  the  nuclei  of  the  ocular 
nerves  has  not  yet  been  determined,  and  the  location  of  the  cortical  cen- 
tres is  still  very  uncertain. 

Marina's  investigations  have  made  it  doubtful  whether  a  centre  for 
convergence  in  the  oculomotor  nucleus  exists.  He  has  shown  that  when 
another  ocular  muscle  was  transplanted  and  allowed  to  unite  with  the 
tendon  of  the  internal  rectus,  contraction  of  the  pupil  in  convergence 
still  occurred,  although  the  internal  rectus  muscle  had  no  part  in  the 
convergence  movement  of  the  eyeball.  Contraction  of  the  pupil  oc- 
curred when  the  eyeball  w^as  drawn  inward  through  electrical  irritation 
of  the  internal  rectus,  and  even  when  it  was  drawn  inward  by  forceps 
without  muscular  action.  All  these  experiments  seem  to  show  that 
there  can  be  no  centre  for  convergence.^^ 

Van  Gehuchten  and  van  Biervliet,  from  their  studies  on  rabbits, 
conclude  that  the  uncrossed  fibres  of  the  oculomotor  nerve  arise  chiefly 
in  the  ventral  portion  of  the  nucleus  and  the  crossed  fibres  in  the  dorsal 
part  of  the  nucleus.  The  upper  end  of  the  nucleus,  therefore,  gives 
origin  only  to  direct  fibres.  The  nen^e-cells  scattered  between  the  fibres 
of  the  posterior  longitudinal  bundle  (the  lateral  cells  of  Bernheimer, 
the  aberrant  cells  of  Biervliet)  belong  to  the  oculomotor  nucleus  and 
give  origin  to  direct  fibres. 

Bernheimer  '^  believes  that  the  muscles  of  the  eyeballs  are  in- 
nervated from  the  lateral  nucleus  (chief  nucleus)  and  that  the  median 
nucleus  and  the  Edinger-Westphal  nucleus  innervate  only  the  inner 
muscles  of  the  eye. 

The  middle  portion  of  the  lateral  nucleus,  which  gives  origin  to 
crossed  fibres,  according  to  this  investigator,  innervates  the  inferior 
rectus:  further  forward  is  the  nucleus  of  the  internal  rectus  of  the 
same  side.  The  lateral  portion  of  the  nucleus  gives  origin  to 
fibres  for  the  internal  rectus.  The  group  of  cells  innervating  the 
superior  rectus  is  further  forward  than  that  innervating  the  internal 
rectus.  The  frontal  portion  of  the  lateral  nucleus  contains  the  nucleus 
of  the  levator  palpebrae  superioris.  Distal  to  the  lateral  nucleus  is  the 
group  of  cells  innervating  the  inferior  rectus  and  bordering  on  the 
nucleus  of  the  trochlear  nerve.    It  gives  origin  only  to  crossed  fibres. 


'^Marina,  Neurologisches  Centralblatt,  September  1,  1904,  p.  797. 
"Bernheimer,  Neurologisches  Centralblatt,  15,  11,  1899. 


14  TUE    EYE    AND    NERVOUS    SYSTEM 


Chief  Nucleus 


Levator  palpebrse 
Rectus  superior 
Rectus  internus 
Rectus  inferior 
Obliquus  superior 


Median  Line 


Most  writers  agree  that  the  Darkschewitch  nucleus  is  not  a  part  of 
the  oculomotor  nucleus.  No  scheme  of  the  divisions  of  the  oculomotor 
nucleus  can  as  yet  be  accepted  as  final. 

I  cannot  believe  that  the  centre  for  the  superior  rectus  is  so  far 
forward  as  is  usually  represented.  In  two  cases  of  tumor  under  my  ob- 
servation the  upward  movement  of  the  eyeballs  was  much  more  affected 
than  the  downward  movement.  A  necropsy  in  each  case  revealed  a 
tumor  of  the  pons. 

Some  investigators  doubt  the  existence  of  a  pupillary  reflex  centre 
in  the  oculomotor  nucleus,  and  they  point  out  that  no  explanation  for 
reflex  pupillary  rigidity  has  been  found  in  the  condition  of  the  anterior 
colliculi  of  the  corpora  quadrigemina  or  oculomotor  nucleus.  Patho- 
logical alteration  of  the  entire  corpora  quadrigemina  has  been  observed 
with  intact  pupils,  and  in  other  cases  this  region  has  been  found  to 
be  normal,  even  the  Edinger-Westphal  nucleus,  when  there  had  been 
reflex  rigidity  of  the  pupils  or  internal  ophthalmoplegia  of  years'  dura- 
tion. Reichardt  thinks  there  is  very  little  evidence  that  the  cause  of 
the  reflex  rigidity  of  the  pupil  is  to  be  found  in  the  region  of  the  oculo- 
motor nucleus,  or  even  in  the  small  cell  median  group  of  the  oculomotor 
nucleus ;  indeed,  contrary  to  such  an  opinion  are  the  cases  of  disease 
or  congenital  anomaly  of  the  region  of  the  oculomotor  nucleus  without 
reflex  pupillary  rigidity,  and  the  long  duration  of  reflex  pupillary 
rigidity  in  some  cases  as  the  only  ocular  sign  of  disease  and  without 
any  other  manifestation  of  disturbance  of  the  oculomotor  nucleus. 
Reichardt  thinks  there  is  more  reason  to  believe  that  a  lesion  causing 
reflex  rigidity  of  the  pupil  is  to  be  found  in  the  spinal  cord,  and  this 
would  explain  the  frequent  occurrence  of  the  sign  in  disease  of  the 
spinal  cord. 

Nine  cases  studied  clinically  and  pathologically  by  Wolff  seem  to 
show  that  degeneration  of  the  posterior  columns  in  the  upper  cervical 
region  is  in  close  relation  to  the  rigidity  of  the  pupils.^"'  Bach,  alone 
and  in  association  with  Meyer,  has  found  from  experiments  on  cats  that 
complete  even  repeated  division  of  the  cervical  part  of  the  spinal  cord 
several  millimetres  below  the  fourth  ventricle  causes  no  change  of  the 

"Wolff,  Arch,  fiir  Psychiatrie,  vol.  xxxii.,  No.  1,  p.  57. 


Fig.  5. — The  cerebral  peduncles  showing  the  oculomotor  nerves  at  their  origin  below  the  aqueduct 
of  Sylvius,  and  their  course  as  sepiarate  bundles  of  fibres  to  the  point  of  exit  at  the  inner  side  of  the 
crusta.    Photograph  of  a  section  from  a  new-bom  child. 


Fig.  C— The  trochlear  nerves  decussating  above  the  aqueduct  of  Sylvius.     Photograph  of  a  section 

from  a  new-born  child. 


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Fig.  9.— Photograph  of  a  section  from  the  medulla  oblongata  of  a  new-lDorn  child,  showing  the  cochlear 
branch  of  the  acoustic  nerve  at  each  side  deeply  stained  in  black. 


Fig.  10.— Photograph  of  a  section  from  the  medulla  oblongata  of  a  new-born  child,  showing  the  vestibu- 
lar branch  of  the  acoustic  nerve  at  each  side  deeply  stained  in  black. 


AHruLTihftf^^UAHf^'^    ^ 


Fui.  II.— Photograph  of  a  section  from  the  medulla  oblongata  of  a  new-born  child,  showing  on  the 
right  of  the  picture  the  fibres  of  the  glossopbaryngeus  or  vagus  entering  through  the  spinal  root  of  the 
trigeminal  nerve. 


:■  ^"n 


Alf^JJitff'i^'iAlltn . 


Fi(i.  li-riiotographof  a  section  from  the  medulla  oblongata  of  a  new-boni  child,  sliowlng  the 
hyjR^'lossus  nerve  on  each  side  passing  from  its  nucleus  below  the  floor  of  the  fourth  ventricle  and 
making  its  exit  on  the  inner  side  of  the  inferior  olive. 


THE   ENCEPHALIC   NERVES  15 

pupillary  reaction.  At  the  moment  the  division  occurs  the  pupils  be- 
come wider,  but  a  few  seconds  later  the  light  reflex  is  restored. 

Bilateral  division  of  the  medulla  oblongata  at  the  spinal  end  of  the 
fourth  ventricle,  at  a  definite  place,  causes  rigidity  of  both  pupils  to 
light. 

Division  of  the  right  half  of  the  medulla  oblongata  at  the  spinal 
end  of  the  fourth  ventricle  causes  rigidity  of  the  left  pupil  to  light, 
while  the  reaction  of  the  right  pupil  is  preserved. 

Mere  exposure  of  the  medulla  oblongata  is  sufficient  to  diminish  or 
even  abolish  the  light  reflex  of  the  pupils  for  a  quarter  of  an  hour  to 
one  hour,  and  when  this  has  occurred  the  rigidity  has  promptly  disap- 
peared when  a  cut  has  been  made  in  the  middle  of  the  fourth  ventricle 
or  on  each  side  in  the  upper  part  of  the  medulla  oblongata. 

These  experiments  seem  to  show  that  a  very  limited  area  near  the 
median  line  and  near  the  respiratory  centre,  at  the  spinal  end  of  the 
fourth  ventricle,  is  of  great  importance  for  the  light  reflex  of  the 
pupil.  This  seems  to  be  an  inhibitory  centre  for  the  pupil.  Cerebral 
to  this  area  is  a  subordinate  reflex  centre  of  the  pupil.  There  are, 
therefore,  probably  two  reflex  arcs  for  the  pupil ;  one  passing  through 
the  corpora  quadrigemina  and  one  through  the  spinal  end  of  the  fourth 

ventricle. 

TROCHLEAR  NERVE. 

The  trochlear,  or  fourth,  nerve  arises  in  a  distinct  group  of  cells 
situated  within  a  depression  of  the  posterior  longitudinal  bundle  and 
directly  posterior  to  the  oculomotor  nucleus.  It  is  separated  from  the 
latter  group  of  cells.  The  nerve  fibres  on  leaving  the  trochlear  nucleus 
pass  around  the  aqueduct  of  Sylvius  on  the  same  side,  and  somewhat 
spinalwards,  decussate  above  the  aqueduct  in  the  anterior  medullary 
velum,  and  leave  the  brain  stem  as  a  delicate  bundle  directly  behind 
the  posterior  colliculus  of  the  corpora  quadrigemina  of  the  opposite 
side.  The  nerve  then  descends  along  the  outer  border  of  the  cerebral 
peduncle. 

TRIGEMINAL   NERVE. 

The  trigeminal,  or  fifth,  nerve  is  chiefly  sensory,  although  it  has  also 
an  important  motor  function  in  the  innervation  of  the  muscles  of  masti- 
cation. The  motor  and  sensory  nuclei  are  situated  side  by  side,  the 
motor  to  the  inner  side,  in  the  lateral  part  of  the  tegmentum  of  the 
pons  about  midway  between  the  cerebral  and  spinal  ends  of  the  pons. 
The  sensory  and  motor  bundles  of  this  nerve  are  closely  associated 
within  the  pons,  so  that  separate  strands  of  fibres,  such  as  are  seen  in 
the  intramedullary  portion  of  the  oculomotor  and  abducens  nerves,  are 


1«;  THE    EYE    AND    NERVOUS    SYSTEM 

not  found  in  the  course  of  the  trigeminal  nerve.  The  motor  portion  of 
the  nerve  receives  a  small  bundle  of  fibres  from  a  group  of  large  cells 
situated  laterally  to  the  aqueduct  of  Sylvius  and  on  the  same  side. 

The  sensory  fibres  arise  in  the  Gasserian  ganglion  and  after  enter- 
ing the  pons  terminate  only  partially  in  the  sensory  nucleus,  the  larger 
number  of  them  bending  downward  at  the  level  of  the  sensory  nucleus 
to  form  the  descending  or  spinal  root,  which  may  be  traced  so  far  as 
the  second  cervical  segment  of  the  spinal  cord.  Within  the  spinal  cord 
it  occupies  a  position  behind  the  posterior  horn.  The  long  course  of 
the  descending  spinal  root  explains  the  implication  of  the  trigeminal 
nerve  in  certain  cases  of  lesion  not  extending  above  the  medulla  ob- 
longata, as  in  syringomyelia. 

The  origin  of  the  fibres  of  the  spinal  root  in  the  Gasserian  ganglion 
is  shown  by  the  intense  degeneration  that  occurs  in  these  fibres  when 
the  sensory  root  of  the  nerve  is  cut  between  the  pons  and  the  Gasserian 
ganglion. 

The  sensory  fibres  on  entering  the  pons  pass  dorsally  and  medially 
to  the  sensory  nucleus.  The  motor  root  exterior  to  the  pons  remains 
distinct  until  it  becomes  united  with  the  third  division  of  the  trigeminal 
nerve  at  the  peripheral  end  of  the  Gasserian  ganglion. 

ABDUCENS    NERVE. 

The  abducens,  or  sixtli,  nerve  has  its  nucleus  beneath  the  floor  of 
the  fourth  ventricle  in  the  knee  made  by  the  fibres  of  the  facial  nerve 
as  they  pass  upward  and  then  outward.  The  nucleus  is  well  defined, 
almost  spherical,  and  contains  numerous  cells.  It  is  not  probable  that 
any  of  the  axis  cylinder  processes  of  these  cells  are  associated  with  the 
fibres  of  the  facial  nerve. 

The  fibres  of  the  abducent  nerve  on  leaving  the  dorsal  and  inner 
sides  of  the  nucleus  pass  ventrally  and  make  their  exit  at  the  junction 
of  the  pons  with  the  medulla  oblongata  and  near  the  median  line.  The 
nucleus  is  situated  a  little  more  cerebralward  than  is  the  point  of  exit 
of  the  nerve,  consequently  the  fibres  pass  a  little  spinalward  in  their 
exit  from  the  brain  stem.  The  fibres  are  not  united  into  one  bundle  in 
their  course  through  the  tegmentum  of  the  pons  and  transversely 
through  the  pyramidal  tract.  They  do  not  decussate  on  leaving  the 
nucleus,  although,  according  to  some  anatomists,  they  may  receive  a 
small  addition  from  the  nucleus  of  the  other  side. 

The  close  association  of  the  abducent  nucleus  with  the  fibres  of  the 
facial  nerve  explains  the  occurrence  of  paralysis  in  the  distribution  of 
these  two  nerves  when  a  lesion   is  situated  in  the  lower  part  of  the 


PLATE  B. 


a,  olfactory  nerve ;  b,  optic  nerve ;  c,  optic  tract ;  d,  oculomotor  nerve ;  e,  trochlear  nerve ;  /,  sensory 
portion  of  trigeminal  nerve ;  g,  motor  portion  of  trigeminal  nerve ;  h,  abducens  nerve ;  i,  facial  and  acous- 
tic nerves ;  j',  glossopharyngeal,  vagus  and  accessorius  nerves;  k,  hypoglossus  nerve ;  I,  first  cervical  root; 
m,  second  cervical  root. 


THE   EXCEPHALIC   NERVES  17 

tegmentum  of  tlie  pons.  The  nearness  of  the  two  abducent  nerves  to 
one  another  at  tlieir  point  of  exit  from  the  pons  explains  the  occurrence 
of  paralysis  of  both  external  rectus  muscles  in  basal  meningitis. 

The  abducent  nucleus  receives  fibres  from  the  superior  olive,  which 
is  believed  to  be  in  connection  with  the  acoustic  nerve,  and  in  this  way 
a  functional  relation  may  be  established  between  the  two  nerves.  It  is 
also  in  connection  with  that  portion  of  the  oculomotor  nucleus  which 
controls  the  internal  rectus  muscle  of  the  opposite  side.  This  connec- 
tion is  probably  by  means  of  the  posterior  longitudinal  bundle. 

At  the  point  of  exit,  at  the  junction  of  the  pons  and  medulla  ob- 
longata, the  fibres  of  the  abducent  nerve  become  united  into  one  bundle. 
From  here  to  the  exit  from  the  skull  through  the  sphenoidal  fissure  the 
course  of  the  nerve  is  long,  and  consequently  paralysis  of  the  external 
rectus  muscle  is  not  uncommon  from  intracranial  lesions. 

FACIAL    NERVE. 

The  facial,  or  seventh,  nerve  arises  from  a  large  group  of  cells  situ- 
ated in  the  upper  part  of  the  medulla  oblongata  at  about  where  this 
structure  passes  into  the  pons.  The  nucleus  is  situated  postero-laterally 
to  the  upper  end  of  the  lower  olive.  The  fibres  arising  in  the  cells  of 
this  nucleus  jiass  posteriorly  and  medially  ununited  toward  the  raphe 
to  just  below  the  floor  of  the  fourth  ventricle.  Here  the  fibres  ascend 
and  bending  again  so  as  to  form  a  knee,  in  which  is  situated  the  nucleus 
of  the  sixth  nerve,  pass  ventro-laterally  and  on  the  inner  side  of  the 
spinal  root  of  the  fifth  nerve  to  their  exit  point  at  the  junction  of  the 
medulla  oblongata  and  pons.  The  fibres  of  this  nerve  at  their  exit 
from  the  pons  lie  on  the  inner  side  of  the  acoustic  nerve.  The  loop 
made  by  the  facial  nerve  within  the  lower  part  of  the  tegmentum  of 
the  pons  is  one  of  the  most  remarkable  formations  within  the  central 
nervous  system,  and  seems  to  serve  no  definite  purpose.  The  glosso- 
pharyngeus  and  vagus  nerves  have  a  similar  course.  From  the  knee 
onward  the  fibres  of  the  facial  nerve  are  united  in  a  distinct  bundle. 

It  has  not  been  possible  to  distinguish  a  nucleus  for  the  upper 
■distribution  of  the  facial  nerve  separate  from  that  for  the  lower  dis- 
tribution ;  indeed,  it  seems  probable  that  the  entire  facial  nerve  has  one 
common  nucleus  in  the  lower  part  of  the  pons,  although  different 
branches  of  the  nerve  may  be  represented  in  different  portions  of  the 

nucleus. 

ACOUSTIC    NERVE. 

The  acoustic,  or  eighth,  nerve  consists  of  two  distinct  parts,  the  coch- 
lear nerve  concerned  with  hearing  and  the  vestibular  nerve  concerned 
2 


18  THE    EYE    AND    NERVOUS    SYSTEM 

witli  coordination.  The  latter  enters  the  medulla  oblongata  on  the 
inner  side  of  the  restiform  body;  the  former  passes  on  the  outer  side 
of  this  structure.  The  cochlear  nerve  extends  further  spinalward  than 
does  the  vestibular  branch,  but  the  latter  extends  further  cerebral  ward 
than  does  the  cochlear  branch.  The  fibres  of  the  cochlear  nerve  termi- 
nate al)out  the  cells  of  the  ventral  nucleus  and  the  acoustic  tubercle. 
The  ventral  nucleus  lies  on  each  side  of  and  within  the  substance  of 
the  cochlear  nerve  at  its  entrance  into  the  medulla  oblongata.  The 
acoustic  tubercle  lies  a  little  dorsally  to  the  ventral  nucleus,  and  in  man 
is  not  sliar})ly  separated  from  it.  The  trapezoid  body  and  the  acoustic 
stria^  form  a  part  of  the  system  connecting  the  cochlear  ners'c  with 
structures  situated  higher  in  the  brain.  The  former  passes  ventrally 
to  the  restiform  body  towards  the  raphe;  the  latter  pass  around  the 
restiform  body.  The  trapezoid  body  is  much  less  important  in  man 
than  in  some  of  the  lower  animals.  It  is  in  connection  with  the  trape- 
zoid nucleus  and  superior  olive,  and  by  another  system  of  fibres,  the 
lateral  fillet,  is  brought  into  connection  with  the  posterior  colliculus  of 
the  corpora  quadrigemina. 

The  vestibular  nerve  enters  the  medulla  oblongata  between  the 
restiform  body  and  tiie  descending  spinal  root  of  the  trigeminal  nerve. 
The  fibres  of  this  nerve  terminate  about  the  cells  of  the  posterior  nu- 
cleus below  the  floor  of  the  fourth  ventricle,  and  in  the  nuclei  of  Deiters 
and  von  Bcchtcrew,  below  and  lateral  to  the  floor  of  the  fourth  ventricle. 
The  vestibular  nerve  possesses  also  a  descending  root,  between  the 
fibres  of  which  are  numerous  nerve-cells. 

GLOSSOPHARYNGEAL  AND  VAGUS  NERVES. 

The  glossopharyngeus,  the  vagus,  and  the  cerebral  portion  of  the 
accessorius  cannot  1x3  sharply  separated  at  their  exit  from  the  medulla 
oblongata.  The  fibres  of  these  nerves  are  found  directly  dorsal  to  the 
lower  olive  and  in  the  continuation  downward  of  this  line  of  exit.  The 
uppermost  fibres  belong  to  the  glossopharyngeus;  the  middle  to  the 
vagus,  and  the  lowest  to  the  accessorius.  The  fibres  are  not  united  into 
three  nerve  bundles  at  their  point  of  exit  from  the  medulla  oblongata. 

The  sensory  glossopharyngeus  and  sensory  vagus  nerve-fibres  enter 
the  medulla  oblongata  laterally,  passing  through  the  spinal  root  of  the 
trigeminal  nerve.  Some  of  these  terminate  in  the  dorsal  nucleus  below 
the  floor  of  the  fourth  ventricle  and  lateral  to  the  hypoglossus  nucleus. 
Others  bend  downward  near  the  lateral  part  of  the  fourth  ventricle  to 
form  the  descending  root.  This  root  is  accompanied  by  a  small  amount 
of  gray  matter,  and  about  the  nerve-cells  of  this  many  of  the  fibres  of 


THE   ENCEPHALIC   XERVES  19 

the  descending  root  terminate.  In  the  opinion  of  some  anatomists,  the 
descending  root  contains  chjefly  glossopharyngeus  fibres  and  few  vagns 
fibres. 

The  posterior  nucleus  is  regarded  by  some  anatomists  as  motor  in 
function  and  not  sensory.  The  ventral  nucleus,  or  nucleus  ambiguus, 
is  in  all  probability  motor.  This  nucleus  is  not  sharply  defined.  It 
lies  dorso-laterally  to  the  lower  olive.  In  some  transverse  sections  only 
a  few  large  cells  belonging  to  this  nucleus  may  be  seen ;  in  other  sec- 
tions no  cells  at  all  are  found.  The  nerve-fibres  arising  in  this  nucleus 
pass  separately  dorsally  and  medially,  much  in  the  same  way  as  do  the 
fibres  of  the  facial  nerve,  but  before  reaching  the  fourth  ventricle  bend 
to  form  a  knee,  and  join  the  sensory  fibres  passing  obliquely  through 
the  medulla  oblongata. 

ACCESSORIUS    NERVE. 

The  accessorius,  or  eleventh,  nerve  has  been  described  so  far  as  its 
cerebral  portion  is  concerned  in  connection  with  the  glossopharyngeus 
and  the  vagus.  The  spinal  portion  arises  in  the  lateral  hoi'n,  of  the 
spinal  cord,  and  the  nerve-fibres  passing  from  the  cells  in  this  situa- 
tion reach  the  periphery  of  the  cord  through  the  lateral  column.  The 
nerve-fibres  can  be  observed  leaving  the  cord  as  separate  strands  as  low 
as  the  fifth  or  sixth  cervical  segment. 

The  upper  fibres,  or  cerebral  portion,  of  the  accessorius  probably 
belong  to  the  vagus  nerve. 

HYPOGLOSSAL  NERVE. 

The  hypoglossus,  or  twelfth,  nerve  has  its  nucleus  just  below  the  floor 
of  the  fourth  ventricle  close  to  the  raphe.  It  is  a  well  defined  group  of 
large  nerve-cells,  resembling  closely  those  of  the  anterior  horns  of  the 
spinal  cord.  The  nerve-fibres  arising  in  these  cells  pass  a  little 
obliquely  forward  in  straight  lines  until  they  reach  the  lower  olive,  but 
they  are  more  or  less  diverted  from  their  course  in  passing  through  this 
structure.  They  leave  the  medulla  oblongata  lateral  to  the  pyramid, 
although  some  of  the  nerve-fibres  pass  directly  through  the  pyramid. 

The  nucleus  of  the  hypoglossus  is  one  of  the  easiest  to  find  of  all  the 
motor  nuclei  in  the  medulla  oblongata  and  pons,  and  is  about  18  milli- 
metres in  length,  extending  upward  about  as  far  as  the  striae  medullares. 


CHAPTER    II. 

SIGHT  :  THE  STRUCTURE  AND  OPTICAL  PROPERTIES 

OF  THE  EYE. 

By  WILLIAM  NORWOOD  SOUTER,  M.D. 

Sight,  or  vision,  is  that  sense  by  which  we  appreciate  the  form  and 
color  of  objects  through  the  action  of  the  physical  energy,  light,  on  the 
retina.  In  other  words,  sight  is  the  consciousness  which  results  from 
stimulation  of  the  retina  by  light.  The  visual  apparatus,  therefore, 
consists  of  two  distinct  parts:  (1)  the  eye,  which  is  comparable  to  a 
photograph  camera,  the  retina  (which  receives  and  transforms  the  light- 
energj'  into  a  nerve-impulse)  corresponding  to  the  sensitized  plate  of 
the  camera;  and  (2)  the  conducting  and  interpreting  mechanism,  by 
means  of  which  the  nerve-impulse  is  conducted  to  the  visual  areas  of  the 
brain  and  thence  to  the  centres  of  consciousness  where  the  impulse  is 
manifested  as  sight. 

The  manner  in  which  the  light-stimulus  is  transformed  into  a  nerve- 
impulse  is  but  little  understood ;  it  is  only  known  that  in  this  trans- 
formation a  certain  chemical  change  (destruction  of  the  retinal  purple) 
takes  place  in  the  retina.  The  second  transformation,  that  of  the  nerve- 
impulse  into  the  consciousness  of  vision,  is  also  beyond  our  compre- 
hension. So  much  as  is  known  of  the  mental  process  of  vision  will  be 
considered  in  a  subsequent  chapter;  it  is  the  province  of  the  writer  of 
the  present  chapter  to  consider  only  the  structure  of  the  eye,  and  its 
adaptation  and  imperfections  as  an  optical  mechanism.    (See  chapter  iii.) 

THE  STRUCTURE  OF  THE  EYE. 

The  eyeball  is  spheroidal  in  shape.  Its  normal  diameter  varies  be- 
tween 23  millimetres  and  2.^)  millimetres.  The  antero-posterior  diam- 
eter is  slightly  greater  than  the  transverse  diameter,  while  the  vertical  is 
the  least  of  tlio  three  dimensions.  The  difference  between  the  greatest 
and  the  least  diameter  is  about  1  millimetre. 

As  externally  viewed  the  eye  is  divisible  into  two  distinct  portions : 
an  anterior,  transparent  portion,  or  cornea,  and  a  posterior,  larger, 
opaque  portion,  the  f>rlem.  The  curvature  of  the  cornea  is  greater  than 
that  of  the  sclera,  so  that  at  the  junction  of  these  two  structures  there  is 
formed  a  groove  or  sulcus— the  sulcus  cornece.  On  antero-posterior 
20 


STRUCTURE    AND    OPTICAL    PROPERTIES  21 

meridional  section  of  the  eye  the  corneal  portion  comprises  about  one- 
sixth  and  the  scleral  portion  the  remaining  five-sixths  of  the  circum- 
ference. 

The  eyeball  is  protected  posteriorly  by  the  bony  walls  of  the  orbit, 
but  antero-exteriorly  there  is  no  other  protection  than  that  afforded  by 
the  lids.  The  latter  serve,  when  partially  or  completely  closed,  to  pro- 
tect the  eye  from  excessive  light,  and  to  some  extent  from  injury  by 
foreign  bodies.  The  inner  surface  of  the  lids  is  covered  with  mucous 
membrane,  the  conjunctiva,  which  is  reflected  at  the  upper  and  lower 
fornices  (the  upper  and  lower  culs-de-sac)  upon  the  eyeball,  and  the 
epithelium  of  this  membrane  is  continued  over  the  anterior  surface 
of  the  cornea.  The  conjunctiva  thus  forms  a  sac,  open  anteriorly  at  the 
lid-margins. 

There  are  three  concentric  coats  or  tunics  of  the  eye :  the  external, 
the  middle,  and  the  inner  coat. 

The  External  Coat. — The  external  coat  consists  of  the  transparent 
cornea  anteriorly  and  the  opaque  sclera  posteriorly.  The  structure  of 
these  two  membranes  is  essentially  the  same,  both  being  of  a  fibrous 
nature.  The  transparency  of  the  cornea  is  the  result  of  close  union  and 
regular  arrangement  of  the  fibres,  and  of  homogeneity  of  refractive 
index  of  the  component  corneal  tissues. 

The  Cornea  is  covered  anteriorly  by  epithelium,  which  is  continuous 
with  the  epithelium  of  the  conjunctiva.  Immediately  behind  the  epi- 
thelium is  the  anterior  limiting  membrane  (Bowman's  membrane), 
which  is  a  dense,  structureless  membrane.  Next  to  this  is  the  corneal 
stroma,  which  consists  of  regularly  arranged  lamellae  of  fibrous  tissue 
having  lymph  spaces  or  lacunce  connected  by  small  canals  or  canaliculL 
In  each  lacuna  there  is  situated  a  cell  whose  processes  extend  along  the 
canaliculi  so  as  to  communicate  with  adjacent  cells.  These  are  the  fixed 
cells  of  the  cornea,  in  contradistinction  to  the  leucocytes  or  movable 
cells  which  are  carried  along  the  lymph  channels.  The  fourth  layer 
of  the  cornea  is  a  thin,  homogeneous,  very  resistant  membrane,  known 
as  Descemefs  membrane.  It  is  continuous  with  the  ligamentum  pecti- 
natum  (p.  23),  which,  together  with  Descemet's  membrane,  is  embryo- 
logically  a  part  of  the  middle  coat  of  the  eye.*  The  fifth  and  innermost 
layer  is  the  endothelium,  or  layer  of  polygonal  cells,  which  is  continuous 
with  the  endothelium  of  the  iris,  and  which,  like  the  preceding  layer,  is 
embryologically  a  part  of  the  middle  coat.  There  are  no  blood-vessels  in 
the  healthy  cornea,  nourishment  being  supplied  through  lymph  channels. 

The  corneal  nerves  are  derived,  from  the  ciliary  plexus.  They  pene- 
trate the  sclera  on  the  outer  side  of  Schlemm's  canal  and  form  a  net- 


22  THE  EYE  AND  NERVOUS  SYSTEM 

work  in  the  vicinity  of  the  corneal  margin.    From  this  plexus  branches 
are  given  off  which  supply  the  several  layers  of  the  cornea. 

The  radius  of  curvature  of  the  anterior  surface  of  the  cornea  is  about 
8  millimetres,  7.8  millimetres  being  the  commonly  accepted  average  for 
the  nonnal  eye.  Tlie  radius  of  curvature  of  the  posterior  surface  is 
about  6  millimetres.    The  thickness  of  the  cornea  at  its  centre  is  about  1 

millimetre. 

The  sclera  is,  as  previously  stated,  composed  of  dense  fibrous  tissue 
irregularly  arranged.  It  is  of  a  glistening  whitish  color,  which  indi- 
cates its  scant  blood  supply.  The  sclera  is  pierced  by  a  number  of 
openings  for  blood-vessels  and  nerves.  The  anterior  ciliary  arteries, 
veins,  and  nerves  enter  the  eyeball  near  the  margin  of  the  cornea.  In 
the  equatorial  region  there  are  four  or  five  openings  for  the  passage  of 
the  vena"  vorticosa*.  Posteriorly  there  are  a  number  of  small  openings 
for  the  posterior  ciliary  arteries  and  veins,  and  a  large  opening  for  the 
optic  nerve.  This  oi)ening  is  about  1.5  millimetres  in  diameter,  and  is 
situated  slightly  to  the  nasal  side  of  the  posterior  pole  of  the  eye ;  it  is 
traversed  by  connective-tissue  fibres,  continuous  with  the  inner  layers 
of  the  sclera,  which  are  known  as  the  lamina  cribrosa.  The  outer  layers 
of  the  sclera  are  continuous  wdth  the  sheath  of  the  optic  nerve. 

The  greatest  thickness  of  the  sclera  is  about  1  millimetre,  near 
the  posterior  pole.  From  this  region  it  becomes  somewhat  thinner 
toward  the  equator,  and  is  again  thickened  anteriorly  where  the  tendons 
of  the  extraocular  muscles  blend  with  the  sclera. 

Anteriorly,  near  the  sclero-corneal  junction  and  concentric  with  it 
is  found  the  circular  sinus,  knowTi  as  Schlemms  canal.  The  nature  of 
this  was  for  a  long  time  in  doubt,  but  it  is  now  regarded  as  a  venous 
channel,  into  which  drains  also  the  aqueous  humor  through  the  ligamen- 
tum  pectinatum. 

The  sclera  is  covered  externally  by  a  thin  layer  of  loose  connective 
tissue,  the  episclera,  which  is  more  freely  supplied  with  blood-vessels 
than  the  sclera  proper. 

The  anterior  jwrtion  of  the  sclera  is  covered  by  conjunctiva  w^hich 
is  reflected  from  the  lids  at  the  upper  and  lower  culs-de-sac ;  the  poste- 
rior portion  is  embedded  in  Tenon's  capsule,  from  which  it  is  separated 
by  a  lymph  space,  thus  allowing  the  eye  to  be  freely  movable  in  the 
capsule. 

The  Uvea. — The  middle  coat  or  uvea  is  so  named  from  its  resem- 
blance (when  stripped  of  the  sclera)  to  a  grape. 

The  iris  or  anterior  portion  of  the  uvea  is  a  diaphragm  having  in  it 
a  circular  opening  (the  pupil)  for  the  passage  of  light  to  the  retina. 


STRLXTURE    AND    OPTICAL    PROPERTIES  23 

From  the  free  pupillary  border  the  iris  extends  peripherally  to  the 
ciliary  body,  to  which  it  is  attached  slightly  behind  the  sclero-comeal 
junction.  The  angle  between  the  sclero-cornea  and  the  root  of  the  iris 
is  bridged  over  by  a  loose,  spongy  tissue,  called  the  ligamentum  pectina- 
tum.  The  patulency  of  this  structure  is  of  great  importance  in  the 
regulation  of  intraocular  tension,  since  through  its  meshes  the  aqueous 
humor  is  drained  off  into  Schlemm's  canal,  of  which  the  ligamentum 
pcctinatum  forms  the  inner  wall. 

The  iris  is  composed  of  an  endothelial  layer  in  front  (continuous 
with  the  endothelium  of  the  cornea),  a  pigment  layer  behind  (continu- 
ous with  the  pigment  layer  of  the  retina),  and,  between  these  two,  the 
stroma.  The  latter  is  a  network  of  blood-vessels  and  fibrous  tissue,  in 
which  are  embedded  the  two  muscles  of  tlie  iris,  the  sphincter  and  the 
dilator.  The  former  is  a  circular  band  of  muscular  tissue  about  1 
millimetre  in  width,  which  surrounds  the  pupil  and  causes  contraction 
of  this  opening.  The  dilator  muscle  consists  of  longitudinal  or  radial 
fibres  which  assist  in  dilating  the  pupil.  These  two  muscles  regulate 
the  size  of  the  pupil,  which  contracts  in  brilliant  illumination  to  cut  off 
the  excess  of  light,  and  expands  in  feeble  illumination  to  allow  sufficient 
light  to  enter  the  eye. 

The  bluish  color  of  the  iris  in  blondes  is  due  to  the  pigment  con- 
tained in  the  posterior  or  retinal  pigment  layer ;  in  dark  eyes  there  is 
additional  pigment  in  the  stroma. 

The  ciliary  body,  the  middle  portion  of  the  uvea,  is  ring-shaped,  tri- 
angular on  transverse  section,  with  its  broadest  side  or  base  in  apposi- 
tion with  the  inner  surface  of  the  sclera  (Fig.  1).  This  body  extends 
from  the  sclero-comeal  junction  backward  for  a  distance  of  about  2 
millimetres,  being  merged  in  the  chorioid  at  the  ora  serrata,  or  anterior 
extremity  of  the  retina.  The  ciliary  body  is  composed  of  the  ciliary 
processes  and  the  ciliary  muscle.  The  ciliary  processes  are  connective- 
tissue  elements  in  which  are  embedded  a  large  number  of  blood-vessels 
and  pigment-cells.  There  are  from  fifty  to  seventy  of  these  processes 
longitudinally  arranged  with  their  larger  ends  projecting  anteriorly. 
The  ciliary  muscle  lies  beneath  the  processes  and  consists  of  two  parts. 
The  larger  part  is  formed  of  meridional  fibres  {BrucTce^s  muscle)  which 
are  attached  anteriorly  with  firm  union  to  the  sclero-comeal  junction 
and  neighboring  sclera.  The  fibres  of  this  part  pass  backward  and  are 
inserted  into  the  anterior  portion  of  the  chorioid.  This  is  the  most 
external  part  of  the  muscle.  To  its  inner  side  and  adjoining  the  pro- 
cesses is  the  second  or  transverse  part,  the  annular  muscle  of  Muller, 
which  consists  of  a  band  of  muscular  fibres  surrounding  the  periphery 


24  THE  EYE  AND  NERVOUS  SYSTEM 

of  the  iris.  This  part  constitutes  about  one-tenth  of  the  entire  muscle 
in  the  normal  or  emmetropic  eye.  In  hyperopia  the  annular  muscle  is 
larger  and  in  myopia  it  is  smaller  than  in  emmetropia.  The  ciliary 
muscle  is  an  essential  factor  in  the  accomplishment  of  accommodation. 
By  contraction  of  this  muscle  the  crystalline  lens  becomes  more  convex, 
so  as  to  enable  near  objects  to  be  seen  clearly. 

The  chorioid  is  that  part  of  the  uvea  which  is  posterior  to  the  ciliary 
body.  It  is  dark  brown  in  color,  thus  differing  from  the  ciliary  body, 
which  is  black ;  its  inner  surface  is  smooth,  while  the  outer  surface 
when  torn  from  the  sclera  presents  a  shaggy  ap]X?arance.  In  this  respect 
and  also  in  the  abundance  of  blood-vessels  the  chorioid  resembles  the 


Canal  of  Schlemm 

Spaces  of  Funtana 
Aut.  Ciliary  Veins 

Conjunctiva 


Sclera 


k\ 


Fig.  1.— Diagram  of  anterior  segment  of  eye,  drawn  to  accurate  scale.     (Flemming.) 

embryonic  chorion,  from  which  the  name  is  derived.  The  thickness  of 
the  chorioid  varies  from  .08  millimetre  near  the  optic  nerve  to  .01 
millimetre  at  the  ora  serrata. 

In  structure  the  chorioid  consists  of  a  network  of  blood-vessels, 
intervascular  connective  tissue,  and  pigment.  It  is  usually  described 
as  consisting  of  five  layers,  which,  beginning  with  the  external  layer, 
are:  (1)  The  supra-chorioid,  (2)  the  layer  of  large  vessels,  (3)  the 
layer  of  medium  vessels,  (4)  the  layer  of  capillaries,  and  (5)  the 
lamina  vitrea. 

The  supra-chorioid  is  composed  of  non-vascular  but  richly  pigmented 
lamelljp,  which  lie  Ix-tween  the  sclera  and  the  chorioid  proper.  When 
stripped  from  the  sclera  these  lamellsp  are  torn  in  shreds,  from  which 
the  chorioid  acquires  its  shaggj'  appearance. 


STRUCTURE    AXD    OPTICAL    PROPERTIES  25 

The  layer  of  large  vessels,  consisting  chiefly  of  veins,  is  placed  in  a 
close  network,  with  many  anastomoses.  The  intervascular  spaces  are 
liighly  pigmented. 

The  layer  of  medium-sized  vessels  is  thin  and  only  slightly  pig- 
mented. 

The  capillary  layer  consists  of  large  capillaries,  closely  arranged. 
This  layer  contains  no  pigment. 

The  lamina  vilrea  is  a  thin  homogeneous  membrane  which  coats  the 
inner  surface  of  the  capillary  layer. 

The  blood  supply  of  the  uvea  is  derived  from  the  anterior  and  the 
posterior  ciliary  arteries.  The  anterior  ciliary  arteries,  four  in  num- 
ber, arise  from  the  four  arteries  supplying  the  recti  muscles,  pierce  the 
sclera  near  the  cornea,  and  terminate  in  the  greater  arterial  circle  of 
the  iris.  From  this  circle  radial  branches  are  given  off  to  supply  the 
iris,  and  these,  by  reunion,  form  the  lesser  arterial  circle  near  the 
pupillary  border  of  the  iris.  The  posterior  ciliary  arteries  consist  of  the 
long  and  the  short  ciliary  arteries.  Of  the  former  there  are  two,  which 
arise  from  the  ophthalmic  artery,  pierce  the  sclera  near  the  optic  nerve, 
and  continue  forward,  one  on  each  side  of  the  eyeball,  between  the 
sclera  and  chorioid  as  far  as  the  ciliary  muscle.  Each  artery  then  divides 
into  two  branches,  which  form,  with  the  aid  of  the  anterior  ciliary 
arteries  already  mentioned,  the  greater  arterial  circle  of  the  iris.  It 
thus  appears  that  the  anterior  and  the  long  posterior  ciliary  arteries  sup- 
ply chiefly  the  iris  and  ciliary  body.  The  short  posterior  ciliary  arteries, 
from  twelve  to  fifteen  in  number,  arise  from  the  ophthalmic  or  some 
of  its  branches,  and  surround  the  optic  nerve  as  they  pass  to  the  posterior 
part  of  the  eyeball,  where  they  pierce  the  sclera  and  supply  the  chorioid 
and  ciliary  processes. 

The  greater  part  of  the  venous  blood  from  the  uvea  leaves  the  eye 
through  the  venae  vorticosa?,  of  which  there  are  from  four  to  six  pene- 
trating the  sclera  in  the  equatorial  region  of  the  eyeball.  A  much 
smaller  proportion  of  the  venous  blood,  chiefly  that  from  the  ciliary 
muscle,  leaves  the  eye  through  the  anterior  ciliary  veins,  which  pierce 
the  sclera  near  the  cornea  and  pass  backward  under  the  conjunctiva. 
These  veins  anastomose  with  the  conjunctival  veins  and  also  with 
Sehlemm's  canal. 

The  nerves  of  the  uvea  are  the  long  and  the  short  ciliary  nerves. 
The  former,  two  or  three  in  number,  are  derived  from  the  nasal  branch 
of  the  fifth  nerve.  The  short  ciliary  nerves,  from  ten  to  twelve  in 
number,  are  derived  from  the  ciliary  ganglion ;  they  contain  motor 
fibres  from  the  third  nerve,  sensory  fibres  from  the  fifth  nerve,  and 
fibres  from  the  svmpathetic  system. 


26 


THE  EYE  AND  NERVOUS  SYSTEM 


The  Retina.-^The  retina  is  the  third  or  innermost  eye-tunic.  It  is 
formed  by  the  terminal  expansion  of  the  optic  nerve,  and,  with  the 
exception  of  its  outer  or  pig^ient  layer,  is  transparent  during  life,  hav- 
ing only  a  slight  purplish  tinge  {the  retinal  purple),  which  fades  on 
exposure  to  light. 

The  retina  extends  forward  as  far  as  the  ora  serrata,  beyond  which 
it  exists  only  in  rudimentary  form.  In  the  latter  condition  it  extends 
to  the  border  of  the  pupil,  furnishing  the  posterior  pigment  layer  of 
the  ciliary  body  and  iris  (pars  ciliaris  retince  and  pars  iridica  retirus), 


Fio.  2.— Section  of  human  retina,  showing  the  usual  appearance  of  the  component  layers  in  ordinary 
preparations.— «.  pigrment  layer ;  6,  rotjs  and  cones ;  c,  external  limiting  membrane ;  d,  outer  nuclear 
layer ;  e,  outer  plexiform  layer ;  /,  inner  nuclear  layer ;  g,  inner  plexiform  layer ;  h,  layer  of  ganglion- 
cells  :  i,  fibre  layer ;  k,  internal  limiting  membrane ;  /,  base  of  Miller's  fibre ;  t>,  blood-vessel.  Magnified 
475  diametere. 


which  is  continuous  with  the  pigment  layer  of  the  retina  proper  (pars 
optica).  This  pigment  layer  is  embryologically  a  part  of  the  retina, 
but  it  adheres  to  the  chorioid  when  the  retina  is  lifted  from  the  former 
membrane. 

The  fibres  of  the  optic  ner\'e,  which  within  the  eye  consist  only  of 
axis-cylinders    (except   in  the  anomalous  condition   known   as  opaque 


STKLTTURE    AXD    OPTICAL    PROPERTIES  27 

nerve-fibres),  radiate  from  the  optic  disk,  or  entrance  of  the  optic  nerve, 
and  form  the  inner  layer  of  the  retina.  The  optic  disk  is  situated' 
slightly  to  the  nasal  side  of  the  posterior  pole  of  the  eye.  At  the  poste- 
rior polar  region  is  situated  the  macula  lutea  or  yellow  spot.  This  is 
described  as  an  annular  or  oval  area  from  1  millimetre  to  2  millimetres 
in  diameter,  at  the  centre  of  which  lies  a  minute  depression,  the  fovea 
centralis. 

^  u 

Adjacent  to  the  chorioid. 


Adjacent  to  the  vitreous. 

Fig.  3. — Scheme  of  the  structure  of  the  human  retina. 

A.  Horizontal  section,  hsematoxylin  stain.  I.  Pigment  epithelial  layer.  II.  Layer  of  rods  and 
cones:  a.  external;  b.  internal  elements.  III.  External  limiting  membrane.  IV.  External  molecu- 
lar layer :  c,  fibre  layer.  V.  External  granular  layer.  VI.  Internal  molecular  layer :  d,  spongio- 
blasts ;  e,  supporting  fibres  of  Miiller ;  /,  nuclei  of  the  same.  VII.  Internal  granular  layer.  VIII. 
Layer  of  ganglion  cells.    IX.  Nerve-fibre  layer.    X.  Internal  limiting  membrane. 

B.  Demonstration  after  the  method  of  Golgi.  I.  Pigment  epithelial  layer.  II.  Layer  of  rods  and 
cones.  III.  Molecular  and  visual  cells.  IV.  External  plexiform  layer.  V.  Layer  of  horizontal  cells. 
VI.  Layer  of  bipolar  cells.  VII.  Layer  of  amacrine  cells.  VIII.  Internal  plexiform  layer  (fibre  layers). 
IX.  Layer  of  ganglion  cells.  X.  Nerve-flbre  layer :  1,  diffuse  amacrine  cells:  2,  diffuse  ganglion  cells ; 
-3,  centrifugal  nerve  fibres ;  4,  association-amacrine  cells ;  5,  neuroglia  cells ;  6,  supporting  fibres  of 
Miiller. 


In  structure  the  retina  is  composed  of  an  outer  and  an  inner  lamina. 
The  outer  lamina  constitutes  the  retinal  pigment  layer.  The  inner 
lamina  constitutes  the  rest  of  the  fully  formed  retina  (Fig.  2).  The 
inner  lamina  is  subdivided  into  two  layers,  the  neuro-epithelial  and 
the   cerebral    (Fig.    3).       The   neuro-epithelial   layer  consists   of   the 


28 


THE  EYE  AND  NERVOUS  SYSTEM 


retinal  rods  and  cones,  whicli  constitute  the  light-receiving  and  trans- 
forming mechanism.  The  cerebral  layer,  which  is  the  impulse-trans- 
mitting part  of  the  retina,  contains  a  layer  of  bipolar  cells  and  a  layer 
of  ganglion-cells  and  nerve-fibres.  The  bipolar  cells  connect  by  their 
processes  the  rods  and  cones  on  the  one  hand  with  the  ganglion-cells  of 
the  nerve-fibres  on  the  other,  and  thus  form  the  connecting  link  between 
the  peripheral  recipient  elements  and  the  cerebral  conducting  nerve- 
fibres.  The  bipolar  cells  are  not  united  to  the  rods  and  cones  or  to  the 
ganglion-cells,  but  their  process-terminals  are  in  close  apposition  with 
the  terminals  of  the  elements  which  they  connect. 

In  addition  to  the  bipolar  cells  just  described  there  are  also  found 
similar  cells,  except  that  their  processes  extend  horizontally.     From 


Extrrnal  surface 


Fif},  4.— Diagrammatic  secUonof  the  human  fovea.  Magnified  375  diameters.  (Golding-Bird  and 
Schiifer.)-2.  ganglion  layer :  4,  inner  nuclear  layer ;  6.  outer  nuclear  layer,  the  cone-flbres  forming  the 
so-called  external  fibrous  layer  of  Henle ;  7.  cones ;  v,  section  of  a  blood-vessel ;  M,  membrana  limitans 
interna ;  og,  ig,  outer  and  inner  granules  (cone-nuclei  and  bipolars)  at  the  centre. 


their  apparent  nerve  connections  these  cells  are  called  association  cells, 
though  their  function  is  not  definitely  known.  Still  other  cells  are 
found  which  apparently  have  no  nerve  connections,  and  whose  function 
is  likewise  not  known.  They  are  called  spongioblasts  (Muller)  or  ama- 
crine  cells  (Cajal). 

The  supporting  neuroglia  or  susientacular  tissue  of  Muller  consists 
of  transverse  fibres  and  an  outer  and  an  inner  membrane,  the  external 


STRUCTURE    AXD    OPTICAL    PROPERTIES  29 

and  the  internal  limiting  membrane,  respectively.  These  two  mem- 
branes, together  with  the  retinal  pigment  layer  and  the  various  sub- 
divisions of  the  neuro-epithelial  and  cerebral  layers,  make  up  the  ten 
layers  into  which  the  retina  is  usually  divided. 

The  macula  lutea,  that  part  of  the  retina  which  is  concerned  in  dis- 
tinct vision,  requires  special  description.  In  this  region  the  rod-ele- 
ments are  replaced  by  cones,  so  that  well  within  the  border  of  the  macula 
there  are  no  rods,  while  the  number  of  cones  is  very  great.  The  layer  of 
ganglion-cells  is  also  much  thickened  in  this  area,  except  at  the  fovea, 
the  region  of  greatest  visual  acuteness,  where  the  ganglion-cell  layer 
becomes  very  thin ;  at  the  foveal  centre  these  cells  are  not  found,  the 
connection  between  the  cones  and  the  nerve-fibres  being  made  by  lat- 
erally extending  processes  of  the  bipolar  cells.  Transversely  the  pro- 
cesses of  these  cells  are  much  shorter  at  the  fovea  than  elsewhere.  This 
fact,  together  with  the  diminution  of  the  ganglion-cells,  makes  the 
cone-elements  the  most  prominent  feature  at  the  fovea.  The  cone- 
fibres  and  nuclei,  constituting  the  outer  nuclear  layer  (iv.  Fig.  3,  A) 
are  relatively  conspicuous ;  they  form  the  external  fibrous  layer  of 
Ilenle.  The  fibres  are  situated  obliquely  at  the  sides  of  the  fovea,  but 
perpendicularly  at  its  centre  (Fig.  4).  In  the  latter  situation  the 
fibres  are  much  shorter  than  at  the  sides,  thus  adding  another  factor  in 
the  causation  of  the  depression  at  the  fovea ;  the  principal  other  factors 
are  the  thinning  of  the  nerve-fibre  layer  (ganglion-cells)  and  the  sessile 
character  of  the  bipolar  cells. 

Blood  Supply  of  the  Retina. — The  outer  layers  of  the  retina  receive 
their  blood  supply  from  the  capillaries  of  the  adjacent  chorioid.  The 
inner  layers  are  supplied  by  the  central  artery  of  the  retina.  This 
artery  is  derived  from  the  ophthalmic  artery;  it  penetrates  the  optic 
nerve  about  5  millimetres  behind  the  eyeball  and  emerges  at  the  optic 
disk,  slightly  on  the  nasal  side  of  its  centre.  It  is  divided  at  its  point 
of  emergence  or  slightly  behind  the  disk  into  two  branches,  an  upper 
and  a  lower  branch.  These  branches  traverse  the  disk  giving  off  small 
branches,  and  again  divide  just  within  or  without  the  border  of  the 
disk  into  temporal  and  nasal  branches.  The  further  subdivisions  are 
illustrated  in  Fig.  5.  The  branches  of  the  retinal  artery  are  terminal, 
that  is,  there  are  no  anastomoses,  and  consequently  embolism  of  a 
branch  is  followed  by  loss  of  function. 

The  efferent  veins,  slightly  larger  than  the  arteries,  follow  the  same 
general  course  as  the  arteries.  Although  there  are  no  large  vessels  at 
the  macula,  this  region  is  abundantly  supplied  with  capillaries,  except 
at  the  fovea,  where  there  are  no  blood-vessels. 


30  THE  EYE  AND  NERVOUS  SYSTEM 

Refractive  and  Distensive  Contents  of  the  Eye. — Within  the  inner 
tunic  of  the  eye  just  described  are  included  the  aqueous  humor,  the 
crystalline  lens,  and  the  vitreous  body.  These  substances  serve  the  dou- 
ble jnirpose  of  keeping  the  otherwise  collapsible  eyeball  distended  and 
at  the  same  time  of  acting  as  refractive  media. 

The  aqueous  humor  is  a  transparent,  slightly  alkaline  fluid,  com- 
posed chiefly  of  water  and  sodium  chloride ;  it  is  secreted  by  the  ciliary 


Fig  .  5.— Diagnun  of  the  blood-vessels  of  the  human  retina.  ( Leber,  after  Jaeger. ) — ant,  vn»,  superior 
iia.sal  artery  and  vein ;  at*,  vtg,  superior  temporal  artery  and  vein ;  ant,  vni.  Inferior  nasal  vessels ; 
ali,  vti,  inferior  temporal  ves-sels ;  ame,  ime,  median  vessels ;  am,  tin.  macular  vessels. 


body,  passes  forward  through  the  pupil  (Fig.  1),  and  is  carried  off 
through  the  interspaces  of  the  ligamentum  pectinatum  into  Schlemm's 
canal. 

The  crystalline  lens  is  formed  of  delicate  fibres  closely  united.  In 
childhood  the  lens  substance  is  gelatinous.  In  early  adult  life  the 
central  part  or  nucleus  is  solid,  while  the  outer  or  cortical  part  is  soft. 
In  old  ago  the  entire  lens  is  solid.  The  fibres  are  arranged  in  regular 
layers,  and  the  lens  is  divisible  into  three  principal  segments.  With 
the  diminution  of  transparency  which  takes  place  in  old  age,  the  lines 
of  union  of  these  segments  l)ccome  visible  when  the  lens  is  examined 
with  the  aid  of  oblique  illumination. 

The  lens  is  enclosed  in  a  delicate  capsule,  to  which  is  attached, 
anteriorly  and  posteriorly,  near  the  periphery  or  equator  of  the  lens, 


STKUCTURE    AND    OPTICAL    PIfOPERTlES  31 

the  suspensory  ligament  {zonule  of  Zinn).  By  this  lig;ament,  the  outer 
border  of  which  is  attached  to  the  ciliary  body,  the. lens  is  held  in  posi- 
tion (Fig.  1 ). 

Tn  shape  the  lens  is  biconvex,  its  anterior  surface  being  less  curved 
than  its  posterior  surface.  The  radius  of  curvature  of  the  anterior 
surface  is  about  10  millimetres,  while  that  of  the  posterior  surface  is 
about  G  millimetres.  In  childhood,  and  to  a  less  degree  at  a  later  age, 
prior  to  complete  solidification,  the  curvature  of  the  anterior  surface  of 
the  lens  is  capable  of  increase  by  contraction  of  the  ciliary  muscle  and 
consequent  relaxation  of  the  suspensory  ligament.  In  this  way  the  eye 
is  adapted  for  different  distances  as  is  required  in  near  vision. 

The  lens  is  a  bloodless  body  after  birth,  and  its  metabolism  is  ex- 
tremely slow,  especially  in  its  inner  or  nuclear  portion.  It  is  probable 
that  as  much  nutritive  fluid  as  is  required  is  supplied  from  the  adjacent 
ciliary  body. 

The  vitreous  body,  which  occupies  the  larger  and  posterior  part  of 
the  eye,  consists  of  a  transparent,  semi-fluid,  colloid  substance,  together 
with  trabecular  of  supporting  tissue.  The  whole  is  enclosed  in  a  cap- 
sule, the  hyaloid  membrane,  on  the  anterior,  concave  surface  of  which 
the  crystalline  lens  rests.  The  vitreous  also  contains  floating  cells 
(muscce  voUtaiites)  which  can  usually  be  seen  entoptically  when  one 
looks  at  a  uniformly  bright  light,  as  the  sky.  In  disease  these  cells 
sometimes  become  greatly  multiplied,  so  as  seriously  to  interfere  with 
vision. 

Like  the  lens,  the  vitreous  is  devoid  of  blood  after  birth,  and  conse- 
quently nuist  receive  its  nourishment  from  surrounding  tissues,  the 
ciliary  body  and  chorioid.  There  is  also  a  central  canal  (which  lodges 
the  pre-natal  hyaloid  artery)  running  from  the  posterior  pole  of  the 
lens  to  the  optic  disk  and  communicating  with  the  lymph  spaces  of  the 
optic  nerve.  This  canal  doubtless  plays  an  important  part  in  the  metab- 
olism of  the  vitreous. 

THE  EYE  AS  A  REFRACTIVE  APPARATUS. 

In  order  that  we  may  perceive  the  form  of  an  object  by  the  visual 
sense  the  stimulated  area  on  the  retina  nmst  constitute  an  image  of 
that  object.  This  image  is  formed  by  the  refracting  apparatus  of  the 
eye.  It  is  well  known  that  light  appears  to  travel  in  straight  lines 
(rays)  as  long  as  the  medium  is  unchanged,  but  when  light  passes  from 
one  medium  to  another  of  different  density  or  refractive  index  the 
path  of  all  rays  which  meet  the  separating  surface  obliquely  undergoes 
a  change  of  direction.     This  change  in  direction  is  called  refraction;   it 


32  THE  EYE  AND  NERVOUS  SYSTEM 

is  explained  by  assuming  that  light  travels  in  waves  and  that  the  progress 
of  these  waves  is  less  rapid  in  dense  than  in  rare  substances. 

Bv  means  of  a  properly  curved  surface  separating  two  media  a 
considerable  portion  (a  pencil)  of  the  rays  proceeding  from  a  point  may 
be  united  in  another  point  or  focus  after  refraction,  as  is  illustrated  in 
Fig.  6,  in  which  SAS'  represents  a  section  of  a  spherical  surface  sap- 


s's' 


arating  two  media,  the  second  medium  (that  on  the  right  of  the  figure) 
being  denser  than  the  first,  while  SOS'  represents  a  section  of  a  pencil 
of  light  proceeding  from  0.  That  portion  of  the  wave  which  travels 
along  OA  encounters  the  denser  substance  sooner  than  the  portions 
which  travel  along  OS  and  OS',  ^^^lile  the  first  portion  travels  the 
distance  from  ^1  to  //  in  the  denser  substance,  the  peripheral  portions 
travel  the  greater  distances  BS  and  B'S'  in  the  rarer  medium.  Wlien 
the  whole  pencil  has  entered  the  denser  medium  SHS'  represents  the 
form  of  the  wave  front,  and  SI,  01,  and  S'l  represent  rays  (perpendic- 
ular to  the  wave  front),  all  meeting  in  the  focus,  I.  The  ray  01.  which 
meets  the  separating  surface  perjx^ndicularly  and  which  consequently 
undergoes  no  refraction,  is  called  the  optic  axis. 

The  position  of  the  focus  /  depends  upon  the  degi'ee  of  refraction, 
— that  is,  upon  the  curvature  of  the  surface  and  the  relative  densities 

Fig.  7. 


(refractive  indices)  of  the  two  media.  For  the  same  surface  and  media 
the  position  of  the  focus  also  varies  with  the  jwint  of  origin  of  the 
incident  jxjncil.  As  the  point  0  becomes  more  distant  from  the  sur- 
face the  f(K*us  /  approaches  the  surface,  and  vice  versa.  The  two  points 
O  and  /  are  called  conjugate  foci.  When  the  anterior  focus  0  is  so  far 
distant  that  the  wave  front  may  be  regarded  as  plane  and  the  rays  as 


STRUCTURE    AXD    OPTICAL    PROPERTIES  33 

parallel,  the  conjugate  focus  is  called  the  posterior  principal  focus 
(Fig.  7).  When,  on  the  other  hand,  the  point  0  approaches  so  near 
the  surface  that  the  rays  are  parallel  after  refraction,  that  is,  when  the 
conjugate  /  is  infinitely  distant,  the  point  from  which  the  rays  proceed 
is  called  the  anterior  principal  focu^  (Fig.  8).  When  the  point  of 
origin  is  nearer  than  the  first  principal  focus  the  rays  will  remain 


divergent  after  refraction,  but  the  divergence  will  be  less  than  before 
refraction.  In  this  case  the  rays  appear  to  proceed  from  a  point,  /,  on 
the  left  of  0  (Fig.  9),  but  since  they  do  not  actually  pass  through  this 
apparent  point  of  origin,  this  is  not  an  illuminated  point  as  is  the  focus 
/  in  Fig.  6.  Hence  the  latter  is  called  a  real  focus,  while  the  former  is 
a  virtual  focus.  So  also,  if  the  rays  have  been  rendered  convergent  by 
previous  refraction,  so  that  they  are  already  converging  toward  a  focus, 
the  convergence  of  the  rays  will  be  increased  by  the  refraction  at  the 
convex  surface  (provided  the  focus  toward  which  the  rays  converge  is 
beyond  the  centre  of  the  surface),  so  that  after  this  refraction  the  rays 
will  meet  in  a  real  focus  which  is  nearer  the  surface  than  the  principal 

Fig.  9. 


I-^^s^ 


focus.  This  condition  is  represented  in  the  correction  of  hyperopia  by 
a  convex  lens,  as  is  illustrated  in  Fig.  14,  p.  41.  The  path  of  the 
light  motion  may  be  reversed  in  any  of  the  preceding  diagrams ;  that 
is,  these  diagrams  may  be  used  to  illustrate  the  refraction  which  takes 
place  when  light  passes  from  a  dense  to  a  rare  medium  at  a  concave 
surface.  On  the  other  hand,  it  is  apparent  that  the  refraction  which 
takes  place  when  light  passes  from  a  rare  to  a  dense  medium  at  a  con- 
cave surface  or  from  a  dense  to  a  rare  medium  at  a  convex  surface  is 
3 


34 


THE  EYE  AND  NERVOUS  SYSTEM 


opposite  to  that  hitherto  descrilied ;  that  is,  such  refraction  has  the 
effect  of  increasing  the  divergence  or  diminishing  the  convergence  of 
rays. 

As  the  various  rays  proceeding  from  0  (Fig.  10)  are  concentrated 
at  /,  so  the  rays  from  0,  are  focused  at  /,,  and  those  from  Og  are  focused 
at  I».  Hence  IJ2  is  the  image  of  OiOj.  All  the  rays  which  pass 
through  the  centre  C  meet  the  surface  perpendicularly  and  consequently 
are  unrefracted.  The  point  C  is  called  the  nodal-point  and  the  unre- 
fracted  rays  are  called  nodal-rays,  or  optic  axes,  01  being  the  principal 
axis  and  Oj/j  and  Oo/g  secondary  axes. 

Since  0x0 .yC  and  IiI-XJ  are  similar  triangles,  we  know  that  the 
linear  dimensions  O^O^  and  I^I^  are  proportional  to  the  distances  OC 


and  /C,  or  each  linear  dimension  of  tlie  image  bears  the  same  propor- 
tion to  the  corresponding  dimension  of  the  object  as  does  the  distance 
of  tiie  image  from  the  nodal-point  to  the  distance  of  the  object  from  this 
I)oint.  Hence  it  is  apparent  that  the  nearer  an  object  is  to  the  refract- 
ing surface  (as  the  eye)  the  larger  will  be  the  image  as  formed  after 
refraction. 

Tlie  cornea  of  the  eye  presents  a  curved  (approximately  spherical) 
surface,  convex  to  incident  light,  and  the  refractive  index  of  the  cornea 
and  aqueous  humor  is  greater  than  that  of  air;  ^  hence  the  condition  of 
refraction  when  light  enters  the  cornea  is  similar  to  that  represented  in 
Fig.  10.  The  corneal  refraction,  however,  is  not  sufficiently  great  to 
bring  even  parallel  rays  to  a  focus  on  the  retina  of  the  normal  eye,  and 
still  less  would  it  be  able  to  focus  rays  from  a  near  object.  The  de- 
ficiency is  supplied  by  the  crystalline  lens,  whose  refractive  index  is 
higher  than  that  of  the  aqueous  and  vitreous,  and  which  is  biconvex  in 


'  The  refractive  index  of  the  aqueous  is  practically  identical  with  that  of  the 
vitreous,  while  the  index  of  the  cornea  is  slightly  greater  than  that  of  the  aqueous 
and  vitreous;  btit  for  the  sake  of  simplicity  a  common  index  (1.337)  may  be 
assumed  for  these  three  media. 


STRUCTURE    AXD    OPTICAL    PROPERTIES  35 

form.  By  the  additional  convergence  given  rays  of  light  in  passing 
through  this  lens  they  are  hroiight  to  a  focus  on  the  retina  in  the  normal 
eye,  and  consequently  a  well-defined  image  of  the  object  of  vision  is 
formed  on  the  retina. 

A  combination  of  several  surfaces  and  media,  such  as  is  formed  by 
the  cornea,  aqueous,  lens,  and  vitreous,  constitutes  a  compound  optical 
system.  Such  a  system  is  analogous  to  a  simple  system  of  one  surface 
and  two  media,  except  that,  instead  of  both  conjugate  focal  distances 
being  measured  from  the  same  point  (where  the  surface  and  principal 
axis  intersect),  the  anterior  and  posterior  focal  distances  are  measured 
respectively  from  the  first  and  the  second  principal  point,  and  instead 
of  a  single  nodal-point  there  are  two  nodal-points,  separated  by  the  same 
interval  as  the  principal  points.  In  a  compound  system  the  nodal-ray 
does  not  form  a  continuous  straight  line,  but  its  characteristic  is  that 
after  the  last  refraction  its  direction  is  parallel  to  that  which  it  had 
before  entering  the  refracting  system.  In  the  eye  the  lateral  displace- 
ment of  the  nodal-ray  is  so  slight,  the  two  nodal-points  being  separated 
by  an  interval  of  only  .37  millimetre,  that  it  would  escape  observation 
in  a  diagrammatic  drawing  of  the  natural  size.  For  practical  pur- 
poses, therefore,  we  may  regard  the  eye  as  having  a  single  nodal-point. 
By  calculation  it  is  shown  that  this  point  lies  very  near  the  posterior 
surface  of  the  crystalline  lens,  about  7  millimetres  behind  the  cornea. 

Inversion  of  the  Image. — It  is  apparent  from  Fig.  11  that  the  retinal 

Fig.  11. 


image  is  always  inverted  with  respect  to  the  object  of  vision.  The 
mind,  however,  takes  no  cognizance  of  this  inversion,  since  it  possesses 
the  power  of  external  projection,  so  that  we  see,  not  the  image,  but  the 
object  in  its  true  position.  This  power  has  dovibtless  been  derived 
through  association  with  the  sense  of  touch.  We  have  learned  that  a 
stimulus  conveyed  to  the  brain  from  the  upper  part  of  the  retina  pro- 
ceeds from  an  object  situated  beloAv  the  eye,  and  rice  versa,  and  that  a 
stimulus  on  the  temporal  side  of  the  retina  must  proceed  from  an  object 
on  the  nasal  side  of  the  eye,  and  vice  versa. 

Aberration. — We  have  assumed  that  all  the  rays  proceeding  from  a 
point  are  united  in  its  conjugate  focus  after  refraction  at  a  spherical 


36  THE  EYE  AND  NERVOUS  SYSTEM 

surface.  This  is  true  only  of  small  pencils  of  light  whose  peripheral 
rays  are  not  much  removed  from  the  optic  axis.  It  is  found  by  calcu- 
lation and  experiment  that  the  peripheral  rays  are  relatively  too  strongly 
refracted,  and  consequently  meet  the  axis  sooner  than  the  more  central 
rays.  This  constitutes  spherical  aberration;  it  is  apparent  that  the 
effect  of  aberration  is  to  mar  the  sharpness  of  images.  The  refracting 
surfaces  of  the  eye,  though  not  strictly  spherical,  may  practically  be  so 
regarded,  at  least  at  their  central  portions,  which  alone  are  normally 
concerned  in  refraction,  since  the  iris  prevents  the  more  peripheral 
rays  from  reaching  the  retina. 

In  certain  cases,  especially  in  conical  cornea,  the  peripheral  refrac- 
tion is  relatively  insufficient,  the  focal  distance  for  peripheral  rays 
being  longer  than  that  for  the  more  central  rays.  This  is  called  nega- 
tive aberration. 

There  is  still  another  kind  of  aberration,  which  is  due  to  the  fact 
that  the  various  colors  are  not  equally  refracted,  violet  being  the  color 
of  greatest  and  red  that  of  least  refraction.  This  is  chromatic  aberra- 
tion. 

Aberration  does  not  ordinarily  interfere  with  vision,  partly  l)ecause 
the  iris  cuts  off  the  more  peripheral  rays,  and  partly  because  the  retina 
is  so  constructed  that  a  well-defined  visual  impression  is  conveyed  to 
the  brain  only  when  the  stimulus  falls  upon  the  macula  lutea,  which  is 
so  situated  as  to  receive  images  only  from  points  lying  near  the  optic 
axis  of  the  eye.  While  the  peripheral  portion  of  the  retina  is  very 
useful  in  extending  the  field  of  vision,  it  is  only  the  macula  and  more 
especially  the  fovea  that  is  directly  concerned  in  distinct  vision. 

Angles  Alpha  and  Gamma. — In  addition  to  aberration,  the  eye  is  sub- 
ject to  certain  other  imperfections.  The  various  surfaces  are  not  per- 
fectly centred  on  a  single  line  or  axis,  as  in  a  perfectly  constructed 
optical  apparatus,  and  the  fovea  does  not  lie  exactly  on  the  line  which 
passes  through  the  middle  of  the  cornea  and  the  centre  of  rotation  of 
the  eye, — the  line  which  most  nearly  represents  a  common  optic  axis 
of  the  various  surfaces.  Since  the  image  must  fall  u|X)n  the  fovea  in 
direct  vision,  it  is  apparent  that  the  line  connecting  the  point  of  most 
acute  vision  with  its  image  on  the  fovea  and  passing  through  the  nodal- 
point  of  the  eye  must  be  obliquely  inclined  to  the  axis ;  that  is,  the  axis 
of  direct  vision  is  a  secondary  axis.  This  line  is  called  the  line  of 
vision.  The  angle  which  the  line  of  vision  makes  with  the  optic  axis  at 
the  nodal-point  is  called  gamma.  The  angular  distance  of  the  fovea 
from  the  optic  axis  usually  ranges  from  2°  to  4°,  chiefly  in  the  hori- 
zontal meridian,  the  fovea  being  usually  displaced  temp)rally  as  regards 


STRUCTUKE    AND    OPTICAL    PROPERTIES  37 

the  optic  axis.  When  this  is  the  ease  it  is  apparent  that  the  visual  line 
must  intersect  the  cornea  on  the  nasal  side  of  the  optic  axis.  Further- 
more, it  is  found  by  keratometric  examination  that  the  cornea  is  approx- 
imately a  segment  of  the  small  end  of  an  ellipsoid,  and  that  the  corneal 
summit,  or  point  where  the  major  axis  of  the  ellipsoid  cuts  the  cornea, 
usually  lies  slightly  on  the  temporal  side  of  the  optic  axis.  The  angle 
which  the  major  axis  of  the  cornea  makes  with  the  visual  line  is  called 
alpha. 

Accommodation. — We  have  learned  that  as  an  object  approaches  a 
refracting  surface  the  conjugate  image  on  the  opposite  side  of  the  surface 
recedes  from  the  surface.  Hence  if  the  eye  is  of  such  length  that  light 
from  a  distant  object  is  focused  on  the  retina,  it  is  evident  that  light 
from  a  near  object  must  impinge  upon  the  retina  before  the  rays  have 
been  united  in  a  focus,  unless  the  refractive  power  of  the  eye  can  be 
suitably  increased.  The  eye  does  possess  the  property  (except  in  old 
age)  of  increasing  its  refractive  power.  It  accomplishes  this  by  an 
increase  in  the  convexity  of  the  crystalline  lens,  chiefly  of  the  anterior 
surface.  This  power  which  the  eye  possesses  of  varying  its  refractive 
power  to  adapt  itself  for  vision  at  different  distances  is  called  accommo- 
dation. So  long  as  the  object  of  vision  is  distant  more  than  about  6 
metres  (20  feet)  no  accommodation  is  required;  that  is,  for  all  dis- 
tances not  less  than  this  limit  the  posterior  conjugate  focus  coincides 
practically  with  the  principal  focus ;  or  all  distances  no":  less  than  6 
metres  may  be  regarded  as  infinite  in  our  study  of  the  refraction  of 
the  eye. 

Accommodation  is  effected  by  contraction  of  the  ciliary  muscle  as 
the  result  of  reflex  stimulation  received  by  the  accommodation  centre 
from  its  connection  with  the  optic  nerve,  probably  through  the  corpora 
quadrigemina.  The  accommodation  centre  constitutes  the  anterior  "por- 
tion of  the  nucleus  of  the  third  nerve.  From  this  centre  an  efferent 
impulse  is  transmitted  through  the  ciliary  ganglion  to  the  ciliary 
muscle,  and  at  the  same  time  an  impulse  is  sent  to  the  sphincter  pupillae, 
so  that  contraction  of  the  pupil  takes  place  simultaneously  with  accom- 
modation. 

There  are  two  theories  as  to  the  manner  in  which  contraction  of 
the  ciliary  muscle  produces  an  increase  of  convexity  of  the  crystalline 
lens.  According  to  Helmholtz's  theory,  which  until  recently  was  uni- 
versally accepted,  contraction  of  this  muscle  draws  forward  the  anterior 
part  of  the  chorioid  and  reduces  the  circumference  of  the  ciliary  muscle 
with  a  resultant  relaxation  of  the  suspensory  ligament  of  the  lens.  The 
latter  then  by  the  inherent  property  of  soft  matter  inclosed  in  a  capsule 


38  THE  EYE  AND  NERVOUS  SYSTEM 

and  subjected  to  a  uniform  outside  pressure  assumes  a  more  spheroidal 
sha|)e  with  a  marked  increase  of  convexity  of  its  anterior  surface. 

Tscherning,  on  the  other  hand,  has  endeavored  to  prove  that  con- 
traction of  the  ciliary  muscle  causes  (through  the  meridional  fibres)  an 
increased  tension  upon  the  suspensory  ligament,  and  that  because  of 
the  resistance  of  the  nucleus  of  the  lens  there  is  a  resulting  increase  of 
curvature  at  the  axial  part  of  the  lens  with  a  diminution  of  curvature 
at  tlie  periphery.  Since  this  theory  requires  the  assumption  of  a  firm 
nucleus,  it  would  seem  untenable,  for  we  know  that  no  such  resistant 
nucleus  exists  in  ehihlhood, — the  period  of  greatest  accommodative 
activity. 

Range  of  Accommodation. — The  nearest  point  for  which  an  eye  can 
adapt  itself  is  called  the  near-point  of  the  eye,  and  the  distance  through 
which  distinct  vision  is  possible  is  called  the  range  or  amplitude  of 
accommodation.  In  the  normal  eye  this  embraces  the  whole  region 
from  infinity  to  the  near-point.  The  farthest  point  of  distinct  vision  is 
called  the  far- point  of  the  eye. 

Measurement  of  Accommodation. — Accommodation  may  be  measured 
by  the  convex  lens  which,  when  placed  in  front  of  and  as  near  the  eye 
as  possible,  enables  light  from  a  point  to  be  focused  on  the  retina  with- 
out the  exercise  of  accommodation.  This  lens  is  the  equivalent  of  the 
accommodation  which  must  be  exercised  to  enable  the  light  from  the 

Fig.  12. 


same  point  to  be  focused  on  the  retina  without  the  lens.  Thus  if  £",  Fig. 
12,  represents  an  eye  adapted  for  an  infinite  distance,  parallel  rays 
will  l>e  focused  on  its  retina,  and  in  order  that  rays  from  a  near-point, 
P,  may  1k'  focused  on  the  retina,  the  eye  must  exercise  accommodation, 
or  else  a  convex  lens,  L,  whose  focal  length  is  LP,  must  be  placed  before 
the  eye.  Since  P  is  the  anterior  principal  focus  of  the  lens,  rays  from 
P  will  l)e  parallel  after  passing  through  the  lens,  and  will,  consequently, 
be  focused  at  /*'  on  the  retina  of  the  eye,  which  is  adapted  for  parallel 
rays.  Hence  the  lens  whose  focal  length  is  LP  is  the  equivalent  of  the 
accommodation  which  must  be  exercised  by  a  normal  eye  in  order  that 
rays  from  P  may  be  focused  on  the  retina.     If  P  is  the  nearest  point 


STEUCTURE    AND    OPTICAL    PROPERTIES  39 

for  which  the  eye  can  accommodate,  the  lens  whose  focal  length  is  LP 
is  the  lens-equivalent  of  the  accommodative  power  of  the  eye.^ 

Presbyopia. — As  the  lens  hardens  with  increase  of  age  there  is  a 
gradual  diminution  of  accommodative  power.  The  following  table 
(Bonders)  gives  the  average  accommodative  power  at  different  ages: 


Age 

10  15  20  25 

30  35 

40 

45 

50 

55    60  65    70 

Diopters 

14  12  10   8.5 

7   5.5 

4.5 

3.5 

2.5 

1.75   1   0.75   0.25 

It  is  shown  in  this  table  that  at  45  years  of  age  the  amplitude  is 
3.5  D.,  which  represents  in  a  normal  or  emmetropic  eye  (adapted  for 
an  infinite  distance  without  accommodation)  the  power  of  focusing 
on  the  retina  rays  from  all  objects  at  or  beyond  a  distance  of  ^  metre 
(9  inches).  The  image  of  an  object  nearer  than  this  limit  will  be 
blurred.  In  order  that  a  very  small  object,  such  as  fine  printed  matter, 
may  be  clearly  seen  it  must  be  held  near  the  eye,  so  as  to  form  on  the 
retina  an  image  large  enough  to  be  deciphered,  and  when  the  focusing 
power  does  not  afford  a  clear  image  at  a  distance  of  22  centimetres,  or 
9  inches,  the  reading  of  very  small  print  becomes  difficult.  Furthermore, 
it  is  not  possible  to  use  continuously  all  the  accommodative  power.  It 
is  found  that  one  can  use  for  a  prolonged  period  only  about  two-thirds  of 
the  total  amplitude ;  when  he  has  not  one-third  in  reserve  great  fatigue 
(asthenopia)  quickly  ensues.  Hence,  in  order  that  any  one  may  use 
his  eyes  continuously  for  work  at  a  distance  of  one-third  of  a  metre 
(33  centimetres,  or  13  inches)  he  must  have  a  total  accommodative 
power  of  4.5  D. — 3  D.  for  use  and  1.5  D.  in  reserve.  When  the  am- 
plitude falls  below  this  amount  the  condition  is  called  presbyopia 
(old  sight).  Presbyopia  is,  therefore,  that  condition  in  which,  from 
physiological  sclerosis  of  the  lens,  the  accommodative  power  is  less  than 
4.5  D.  It  is  shown  in  the  table  that  this  condition  makes  its  onset 
about  the  fortieth  year.  Usually,  however,  artificial  assistance  is  not 
required  before  the  forty-fifth  year.  At  this  age  a  convex  spherical 
lens  of  1  D.  generally  affords  the  necessary  assistance.  The  probable 
strength  of  lens  required  to  overcome  the  presbyopia  at  different  ages  is 
given  in  the  following  table: 

Age  45     50     55  60  65        70        75 

Diopters         1       2       2.75       3.25       3.5       3.5       3.5 

'  The  power  of  a  lens  is  inversely  proportional  to  its  focal  length,  which  is  the 
distance  from  the  lens  of  the  focus  for  parallel  rays;  the  shorter  the  focal  length, 
the  greater  is  the  refractive  power  of  the  lens.  The  unit  of  lens  measure  in  oph- 
thalmology is  the  Diopter,  which  expresses  the  power  of  a  lens  whose  focal  length 
is  one  metre.  Similarly,  2  D.  is  the  power  of  a  lens  whose  focal  length  is  i/^  metre; 
3  D.  is  the  power  of  a  lens  whose  focal  length  is  1/3  metre,  etc. 


40  THE  EYE  AND  NERVOUS  SYSTEM 

Obviously  this  table  serves  only  as  a  guide.  It  is  not  to  be  strictly 
followed  in  the  selection  of  working-glasses.  The  determination  of  the 
latter  depends  not  only  upon  the  distance  at  which  work  is  most  con- 
veniently done,  but  also  upon  the  refractive  condition  of  the  eye ;  that 
is,  the  strength  of  the  working-lens  depends  upon  the  position  of  the  far- 
point,  or  point  of  adaptation  in  relaxation  of  the  accommodation.  In 
hyperopia,  as  will  be  subsequently  shown,  a  convex  lens  is  required 
to  adapt  the  eye  for  distant  vision  without  accommodation.  It  is  evi- 
dent, therefore,  that  in  the  selection  of  a  glass  for  near  work  the  correc- 
tion of  the  hyperopia  must  be  added  to  that  of  the  presbyopia.  In 
myopia,  on  the  other  hand,  a  concave  lens  is  required  to  adapt  the  eye 
for  distant  vision,  and  in  this  case  the  difference  between  the  distance- 
glass  and  the  presbyopic  correction  represents  the  strength  of  lens 
required  for  near  work. 

ANOMALIES  OF  REFRACTION. 

In  emmetropia,  the  typically  normal  optical  condition,  parallel  rays 
are  focused  on  the  retina  without  accommodation ;  that  is,  the  eye  is 
adapted  for  distant  vision,  and  by  the  exercise  of  accommodation  it  is 
adapted  for  near  objects.  This  is  the  most  favorable  condition  for  the 
varying  requirements  of  civilized  life,  but  since  it  necessitates  an  exact 
correspondence  between  the  refractive  power  of  the  eye  and  the  length 
of  the  eyeball,  emmetropia,  as  determined  by  refined  methods  of  meas- 
urement, is  the  exception  rather  than  the  rule.  When  the  posterior  prin- 
cipal focus  does  not  fall  upon  the  retina  in  relaxation  of  the  accommoda- 
tion the  eye  is  said  to  be  ametropic  or  to  have  an  error  or  anomaly  of 
refraction. 

There  are  three  kinds  of  ametropia :  hyperopia  or  hypermetropiaj 
in  which  the  posterior  principal  focus  lies  behind  the  retina;  myopia, 
in  which  the  focus  lies  in  front  of  the  retina ;  and  astigmia,^  in  which, 
owing  to  asymmetry  or  irregularity  of  one  or  more  of  the  refracting 
surfaces  or  to  heterogeneity  of  structure,  the  rays  are  not  brought  to 
a  focus  at  any  point. 

Hyperopia. — Ilyjieropia  may  be  due  to  any  one  of  three  causes: 
(1)  deficient  curvature  of  the  cornea  or  lens  (curvature  hyperopia)', 

'  The  writer  has  pledged  his  support  to  the  endeavor  now  being  made  by  Dr. 
Swan  Burnett  and  others  to  replace  the  commonly  employed  word  astigmatism  by 
the  more  euphonious  and  correct  word  here  used.  The  impropriety  of  the  former 
word  has  long  been  known.  The  argument  in  favor  of  making  a  change  at  this 
time  is  ably  presented  by  Dr.  Burnett  in  the  American  Journal  of  Ophthalmology, 
December.  1003. 


STRUCTUKE  AND  OPTICAL  PROPERTIES  41 

(2)  abnormal  refractive  index,  sucli  that  the  total  refractive  power  of 
the  eye  is  too  low  (index  hyperopia — very  rare)  ;  and  (3)  abnormally 
short  antero-posterior  diameter  of  the  eyeball  (axial  hijperopia).  By 
means  of  many  measurements  which  have  been  made  it  has  been  proved 
that  axial  hyperopia  is  by  far  the  most  common  variety. 

In  hyperopia  the  refractive  power  of  the  eye  in  relaxation  of  the 
accommodation  is  insufficient  to  focus  rays  from  any  point,  distant  or 
near,  on  the  retina  (Fig.  13).     The  deficiency  of  refraction  may  be 

Fig.  13. 


overcome  by  means  of  a  convex  spherical  lens  placed  in  front  of  the 
eye.  This  is  illustrated  in  Fig.  14,  in  which  parallel  rays  from  a  dis- 
tant point,  0,  would  be  focused  by  the  hyperopic  eye,  E,  at  some  point 
behind  the  retina,  but  with  the  aid  of  the  lens,  L,  the  rays  are  focused 
on  the  retina,  R.  It  is  apparent  from  this  diagram  that  LP  is  the 
focal  length  of  the  lens,  L,  which  corrects  the  hyperopia,  and  that  this 
focal  length  increases  as  the  lens  is  moved  farther  'from  the  eye,  since 
P  is  a  fixed  point,  conjugate  to  the  retina.  That  lens  which,  when 
placed  as  near  as  possible  to  the  eye  (the  position  in  which  spectacles 
are  worn),  enables  rays  from  a  distant  point  to  be  focused  on  the  retina 
is  taken  as  the  measure  of  the  hyperopia.  Thus,  if  LP  is  1  metre  there 
is  hyperopia  of  1  D. ;  if  LP  is  i  metre  there  is  hyperopia  of  2  D.,  etc. 


^^F 


In  the  study  of  accommodation  we  learned  that  the  eye  is  able  to 
increase  its  refractive  jwwer  by  increasing  the  convexity  of  the  crystal- 
line lens.  In  emnietropia  this  power  is  called  into  play  only  in  near 
vision,  but  in  hyperopia,  when  uncorrected  by  a  lens,  accommodation 
must  be  exercised  to  render  distant  vision  distinct,  and  in  near  vision 
the  same  degree  of  accommodation  which  would  be  required  of  a  nor- 
mal eye  must  be  used  in  addition  to  that  required  for  distant  vision.     It 


42  THE  EYE  AND  NERVOUS  SYSTEM 

is  apparent,  therefore,  that  the  unaided  hyperopia  eye  has  an  abnormal 
task  imposed  ujx)n  it,  both  in  distant  and  in  near  vision.  In  childhood 
when  the  accommodative  power  is  at  its  maximum  a  moderate  degree 
of  hyi^eropia  is  very  often  not  attended  by  any  disturbance,  the  accom- 
modation being  ample  for  near  as  well  as  for  distant  vision,  but  when 
with  advancing  age  the  accommodative  power  diminishes,  the  tax  im- 
posed upon  the  ciliary  muscle  gives  rise  to  various  symptoms  of  nerve- 
exhaustion  (asthenopia). 

So  easy  is  accommodation  in  childhood  that  it  is  usually  not  possi- 
ble for  a  hyperope  at  this  period  of  life  to  relax  all  of  his  accommoda- 
tion when  using  his  eyes.  Hence  a  portion  of  the  hyperopia  will  be 
masked  by  accommodative  action,  and  it  is  only  by  the  employment  of 
a  cycloplegic  or  by  examination  in  a  dark  room  that  the  full  extent 
of  the  hyjxjropia  can  be  ascertained.  That  portion  of  the  hyperopia 
which  is  masked  by  accommodation  is  called  latent  hyperopia.  Hyper- 
opia which  exists  in  excess  of  what  is  latent  is  called  manifest;  that  is, 
manifest  hyjieropia  is  measured  by  the  strongest  convex  lens  which  does 
not  impair  distant  vision.  The  sum  of  the  latent  and  manifest  hyper- 
opia constitutes  the  total  hyperopia.  In  childhood  hyperopia  of  a  low 
or  moderate  degree  is  very  frequently  all  latent,  but  sooner  or  later,  the 
age  varying  with  the  degree  of  hyperopia  and  with  the  physical  condi- 
tion, a  portion  becomes  manifest,  and  this  portion  continually  increases 
at  the  exjjense  of  the  latent  hyperopia. 

There  is,  further,  a  division  of  manifest  hyperopia  into  that  which 
can  be  overcome  by  accommodative  action  to  render  distant  vision  dis- 
tinct (facultative  hyperopia),  and  that  which  cannot  be  so  overcome 
(absolute  hyperopia).  In  old  age  the  total  hyperopia  is  absolute,  so 
that  distant  vision  can  be  made  distinct  only  by  the  help  of  a  convex 
lens.  In  near  work  an  absolute  hyperope  would  require  the  appropriate 
presbyopic  correction  added  to  the  correction  of  the  hyperopia.  Thus 
in  hyperopia  of  2D.,  a  lens  of  5  D.  would  be  required  for  reading  at 
a  distance  of  ^  metre  (13  inches). 

Hyperopia  is  the  normal  condition  in  infancy,  but  with  the  increase 
of  the  antero-posterior  diameter  of  the  eye  which  takes  place  in  the 
process  of  growth,  hyperopia  is  succeeded  by  emmetropia  or  myopia 
prior  to  adult  life  in  about  one-half  of  all  persons  in  civilized  countries. 
Among  savages  liy])eropia  is  the  rule  at  all  ages. 

It  is  apparent,  therefore,  that  hyperopia  (except  in  the  higher 
grades)  is,  like  presbyopia,  a  physiological  condition ;  yet,  owing  to  the 
great  preponderance  of  near-woyk  which  results  from  the  requirements 
of  civilization,  it  is  in  most  cases  necessary  to  correct  all  or  a  portion 


STKUCTURE  AN^D  OPTICAL  PROPERTIES  43 

■of  the  defect  before  the  hyperope  has  advanced  far  in  adult  life.  In 
fact,  even  a  moderate  hyperopia  may  require  correction  in  childhood  to 
relieve  the  strain  which  is  imposed  by  the  unnatural  near-work  incident 
to  school  life. 

In  the  treatment  of  hyperopia  a  number  of  questions  arise,  the  most 
important  of  which  may  be  summarized  as  follows:  (1)  As  to  when 
it  is  advisable  to  correct  the  defect;  (2)  as  to  the  portion  of  the  defect 
to  be  corrected;  and  (3)  as  to  whether  the  glasses  should  be  worn  con- 
stantly or  for  near-work  only. 

As  to  the  first  question,  hyperopia  does  not  require  correction  unless 
it  gives  rise  to  some  physical  disturbance,  as  inability  to  see  clearly, 
asthenopia,  headache,  or  convergent  strabismus ;  but  since  few  persons 
consult  a  physician  except  when  suffering  from  physical  disability,  it  is 
apparent  that  almost  all  cases  of  hyperopia  which  are  brought  to  the 
notice  of  the  physician  require  correction. 

As  to  the  portion  of  error  to  be  corrected,  it  is  not  possible  to  give  a 
fixed  rule.  Usually  in  childhood  the  lens  should  not  exceed  one-half  or 
two-thirds  of  the  total  error  as  determined  with  the  eye  under  the  in- 
fluence of  a  cycloplegic.  The  chief  exception  to  this  rule  is  when  the 
hyperopia  is  complicated  with  convergent  strabismus,  when  all  or  nearly 
-all  the  error  should  be  corrected.  In  adults  correction  of  the  greater  part 
of  the  hyperopia  will  usually  be  tolerated. 

Still  less  is  it  possible  to  answer  the  third  question  by  a  fixed  rule. 
In  general,  children  who  suffer  from  asthenopia  resulting  from  a  low 
^ade  of  hyperopia  should  wear  correcting  glasses  during  school  and 
study  hours  only;  but  when  the  error  reaches  2.5  D.  or  3D.  it  is 
usually  found  that  the  ciliary  muscle  will  not  adapt  itself  to  the  use  of 
glasses  unless  the  latter  are  worn  constantly.  Similarly  in  strabismus 
relief  is  not  to  be  expected  unless  the  glasses  are  worn  constantly.  In 
adult  hyperopes  who  suffer  from  nerve-strain,  as  the  result  of  prolonged 
near-work,  the  same  general  rule  is  applicable ;  that  is,  the  glasses  may 
be  worn  for  near-work  only,  if  the  error  is  slight,  and  especially  if  it  is 
latent,  but  for  higher  grades  of  the  defect  the  glasses  must  be  worn 
constantly. 

Myopia. — Myopia  is  divisible  into  curvature  myopia,  index  myopia, 
^nd  axial  myopia.  Curvature  myopia  results  from  keratoconus  or  conical 
cornea,  or  from  increased  convexity  of  the  lens  in  partial  dislocation 
(when  released  from  traction  by  the  suspensory  ligament).  Myopia 
also  occurs  not  infrequently  as  a  premonitory  sign  of  senile  cataract,  and 
in  diabetes.  This  may  be  due  either  to  increase  of  convexity  of  the 
lens  from  swelling  or  from  increase  of  refractive  index  of  the  nuclear 


44  THE  EYE  AND  NERVOUS  SYSTEM 

portion  of  tlie  lens.  With  these  exceptions  myopia  is  almost  always 
due  to  excessive  length  of  the  antero-posterior  diameter  of  the  eyeball. 
In  myopia,  whatever  its  cause,  the  refractive  power  of  the  eye  is 
too  great  in  proportion  to  the  axial  length,  and  consequently  rays  from 
a  distant  object  (parallel  rays)  will  be  united  in  a  focus  before  they 
reach  the  retina,  and  the  image  of  a  distant  object  as  formed  on  the 
retina  will  be  blurred    (Fig.    15).       The   predominant  symptom  of 

Fig.  15. 


myopia  is,  therefore,  the  inability  to  see  distant  objects  clearly,  and 
since  only  near  objects  are  clearly  distinguished,  the  affection  is  popu- 
larly called  near-sightedness. 

An  object  situated  at  P  (Fig.  16),  which  is  conjugate  to  the  retina, 
and  which  is  the  far-point  of  the  eye,  can  be  clearly  seen  without  any 
accommodation,  and  an  object  nearer  the  eye  than  this  point  can  be 
seen  with  less  accommodation  than  would  be  required  by  a  normal  eye, 
whose  far-point  is  at  infinity.  This  is  not,  however,  as  it  might  appear, 
always  an  advantage  in  near-work,  for  it  not  infrequently  leads  to 
severe  strain  on  the  convergence  centres,  the  nervous  mechanism  being 
so  constituted  that  exercise  of  accommodation  is  required  as  an  incentive 

Fig.  16. 


JX-jig 


for  the  proper  convergence  of  the  visual  lines  of  the  two  eyes,  so  that  the 
image  may  in  each  case  fall  upon  the  fovea.  Furthermore,  when  the 
myopia  exceeds  3.5  I),  or  4  D.,  the  farthest  point  of  distinct  vision  is 
so  near  the  eyes  that  the  amount  of  convergence  required  is  too  great  to 
be  comfortably  maintained ;  in  uncorrected  high  myopia,  vision  is  per- 
formed with  one  eye,  while  the  other  assumes  a  position  of  relative  or 
absolute  divergence  (divergent  strabismus). 


STKUCTURE  AND  OPTICAL  PROPERTIES  45 

Axial  myopia,  which,  as  previously  stated,  is  the  typical  form  of 
myopia,  is  divisible  into  two  classes.  The  first  class  embraces  those 
cases  in  which  the  eyes  are  hyperopic  prior  to  the  beginning  of  school 
life.  In  the  natural  course  of  growth  there  occurs  an  enlargement  of 
the  eyes  with  consequent  diminution  of  hyperopia,  and,  normally,  hy- 
peropia passes  into  emmetropia  before  adult  life.  But  in  a  certain  pro- 
portion of  cases  the  increase  in  the  axial  length  is  not  arrested  when 
emmetropia  is  reached,  and  a  condition  of  myopia  resu],ts.  This  is 
probably  caused  by  stretching  of  the  sclera  by  muscular  pressure  and 
traction  in  the  convergence  required  in  school  work.  This  kind  of 
myopia  does  not  reach  a  very  high  degree  and  it  ceases  to  advance,  after 
the  sclera  has  acquired  its  normal  resisting  power,  in  adult  life.  It  is, 
therefore,  called  benign  or  school  myopia. 

The  second  class  of  axial  myopia  is  due  to  defective  development 
of  the  sclera ;  the  myopic  condition  is  present  at  an  early  age — ^prior  to 
the  beginning  of  school  life.  It  may  r^^ach  a  very  high  degree  (posterior 
staphyloma),  and  together  with  the  distention  of  the  sclera  there  may 
occur  atrophy  of  the  chorioid,  vitreous  opacities,  hemorrhages,  or  de- 
tachment of  the  retina,  with  partial  or  total  loss  of  vision.  This  kind 
of  myopia  is,  therefore,  appropriately  called  malignant  myopia. 

The  malignant  variety  is  fortunately  much  less  common  than  the 
benign.  While  the  latter  is  confined  chiefly  to  the  student  classes,  the 
former  occurs  in  a  relatively  greater  proportion  among  the  poorly  de- 
veloped lower  classes.  The  proportion  of  myopes  varies  in  different 
countries.  In  America  statistics  have  shown  a  proportion  of  about 
25  per  cent,  among  college  students,  while  in  Germany  the  proportion 
among  the  same  class  of  persons  reaches  50  per  cent. 

As  in  hyperopia  the  deficiency  of  refraction  is  overcome  by  the 
convergent  action  of  a  convex  lens,  so  in  myopia  the  excess  of  refrac- 
tion may  be  overcome  by  the  divergent  action  of  a  concave  lens.  Thus  if 
L  (Fig.  16)  is  a  concave  lens,  the  parallel  rays  which  meet  it  are  ren- 
dered divergent  so  that  they  appear,  after  passing  through  the  lens,  to 
proceed  from  the  point  P.  Hence  if  P  is  conjugate  to  the  retina  of  the 
myopic  eye  E,  it  is  apparent  that  parallel  rays  which  are  made  to  pass 
through  the  lens  will  enter  the  eye  E  as  if  coming  from  P,  and  will  con- 
sequently be  focused  on  the  retina.  An  appropriate  concave  lens  will, 
therefore,  make  distant  objects  appear  distinct  to  the  myopic  eye.  The 
eye  may  then  see  near  objects  with  the  aid  of  accommodation,  or  the 
lens  may  be  removed  in  near  vision. 

It  is  apparent  from  Fig.  16  that  parallel  incident  rays  will  never 
be  united  in  a  focus  as  the  result  of  refraction  by  a  concave  lens,  but  P 


46  THE  EYE  AND  NERVOUS  SYSTEM 

is  the  point  on  the  axis  through  which,  after  refraction,  the  rays  appear 
to  pass.  The  point  P  is,  therefore,  the  virtual  focus  (for  parallel  rays) 
of  the  concave  lens,  in  contradistinction  to  the  real  focus  of  the  convex 
lens.  The  focal  length  of  the  lens  L  is  LP;  it  is  expressed  in  diopters, 
as  was  explained  for  the  convex  lens ;  but  since  P  lies  on  the  left  of  the 
lens,  instead  of  on  the  right  as  in  the  convex  lens,  the  focal  length  is 
regarded  as  negative,  and  the  refractive  value  is  expressed  by  the  minua 
(-)  sign,  which  is  used  before  the  dioptric  number. 

The  degree  of  myopia  is  measured  by  the  focal  length  or  dioptric 
power  of  the  lens,  which,  when  placed  as  near  as  possible  to  the  eye, 
focuses  parallel  rays  on  the  retina.  Thus  if  LP  is  1  metre,  the  eye  has 
1  D.  of  myopia ;   if  LP  is  ^  metre,  the  eye  has  3  D.  of  myopia,  etc, 

Tn  the  adaptation  of  concave  lenses  to  the  correction  of  myopia  the 
general  rules  differ  somewhat  from  those  given  for  the  correction  of 
hyperopia.  ^Myopia  occurring  in  young  children  should  be  corrected  at 
as  early  an  age  as  is  compatible  with  tlie  wearing  of  spectacles,  not  only 
for  the  beneficial  effect  in  retarding  the  advance  of  the  myopia  (prob- 
ably by  relieving  the  eyes  of  the  necessity  of  excessive  convergence),  but 
also  for  the  purpose  of  placing  the  child  under  favorable  circumstances 
in  cultivating  his  powers  of  observation.  All,  or  all  but  a  small  frac- 
tion, of  the  myopia  should  be  corrected,  and  the  glasses  should  be  worn 
both  for  distance  and  near-work.  Tn  the  case  of  adults,  however,  when 
the  myopia  has  not  l)een  corrected  in  childhood,  the  ciliary  muscle  is 
poorly  developed,  and  the  full  amplitude  of  accommodation  is  not 
attained.  In  such  cases  it  often  hapj^ens  that  the  glasses  which 
are  suitable  for  distance  will  not  be  tolerated  for  near-work;  for  the 
latter  a  weaker  concave  lens,  or  no  lens,  or  a  weak  convex  lens  may 
be  required,  according  to  the  degree  of  myopia  and  the  age  of  the 
individual. 

In  the  highest  grades  of  myopia  (exceeding  15  D.)  the  myopia 
may  be  overcome  or  greatly  reduced  by  extraction  of  the  crj'stalline 
lens. 

Of  no  less  imi>ortance  than  the  correction  of  myopia  is  its 
prophylaxis  by  the  inculcation  of  proper  hygienic  principles,  such  as 
tlie  avoidance  of  close  application  of  the  eyes  at  a  very  early  age,  the 
necessity  of  good  illumination  of  school  rooms,  and  of  large  and  clear 
print  for  young  children. 

Astigmia. — Astigmia  is  that  condition  in  which  rays  proceeding 
from  a  point  are  not  united  in  a  focus  at  another  point  after  refraction. 
There  are  two  kinds  of  astigmia  of  the  eye:  (1)  That  which  is  due  to 
asymmetrical,  but  regular,  curvature  or  oblique  position  of  the  cornea 


STRUCTUKE  AND  OPTICAL  PROPERTIES  47 

or  lens;*  and  (2)  that  which  is  due  to  imevenness  or  irregularity  of 
one  or  more  of  the  surfaces,  or  to  heterogeneity  of  composition  of  the 
crystalline  lens.  The  first  kind  constitutes  regular  and  the  second 
irregular  astigmia, 

Astigmia  exists,  to  a  slight  extent,  as  a  physiological  imperfection 
in  all  eyes.  The  curvature  of  the  cornea  is,  as  a  rule,  somewhat  greater 
in  the  vertical  than  in  the  horizontal  meridian.  On  the  other  hand,  the 
crystalline  lens  presents  the  opposite  condition,  for,  on  account  of  a 
slightly  oblique  position,  its  meridian  of  greatest  refraction  is  usually 
horizontal.  These  two  defects  of  curvature  tend  to  neutralize  each 
other,-  but  as  a  rule  the  vertical  refraction  predominates,  thus  giving  rise 
to  a  slight  degree  of  regular  astigmia.  Furthermore,  the  normal  crys- 
talline lens  is  not  free  from  heterogeneity  of  structure,  for,  being  com- 
posed of  a  number  of  segments,  and  each  segment  of  a  number  of 
fibres,  its  refraction  is  considerably  marred  by  irregularity,  which  is 
the  chief  cause  of  the  normal  irregular  astigmia. 

Regular  astigima,  exceeding  the  amount  which  may  be  regarded  as 
normal  or  physiological,  may  be  due  to  excess  of  vertical  over  the  hori- 
zontal curvature  (astigmia  with  the  rule);  or  to  excess  of  horizontal 
curvature  (astigmia  against  the  rule);  or  to  asymmetry  in  which  the 
meridians  of  greatest  and  least  curvature  (these  being  at  right  angles 
to  each  other)  are  neither  vertical  nor  horizontal  (oblique  astigmia). 
As  may  be  inferred  from  the  normal  asymmetry  of  the  cornea  and  lens, 
astigmia  with  the  rule  is  more  commonly  the  result  of  corneal  asym- 
metry (corneal  astigmia),  while  astigmia  against  the  rule  is  more  com- 
monly due  to  asymmetry  of  the  lens  (lenticular  astigmia)  ;  but  this  is 
by  no  means  always  the  case,  for  quite  often  the  meridian  of  greatest 
corneal  curvature  is  horizontal,  thus  giving  rise  to  astigmia  against  the 
rule.  This  is  notably  so  after  cataract  operations,  since  the  section  of 
the  cornea  leaves  a  resulting  diminished  curvature  in  the  vertical  direc- 
tion withoijt  materially  altering  the  curvature  of  the  horizontal 
meridian. 

Regular  astigmia,  since  it  results  from  a  regular  asymmetry  of 
curvature,  in  which  the  meridians  of  greatest  and  least  curvature  (the 
principal  meridians)  are  at  right  angles  to  each  other,  may  be  overcome 
by  a  cylindrical  lens,  which  has  the  property  of  refracting  rays  in  the 

*  The  astigmia  which  results  •from  obliquity  of  the  cornea  or  lens  with  refer- 
ence to  the  optic  axis  is  usually  classed  as  regular  since  the  defect  of  vision  result- 
ing therefrom  can  be  improved  by  a  cylindrical  lens;  but  from  a  mathematical 
point  of  view  the  condition  differs  somewliat  from  that  which  results  from  asym- 
metrical curvature. 


48  THE  EYE  AND  NERVOUS  SYSTEM 

meridian  of  its  curvature  without  altering  their  direction  in  the  plane 
of  the  axis  of  the  cylinder.  Hence  if  the  refraction  of  an  eye  is  such 
tliat  rays  proceeding  from  a  distant  }X)int  are,  in  the  vertical  meridian, 
focused  on  the  retina,  while  in  the  horizontal  meridian  of  less  curva- 
ture the  rays  are  intercepted  hy  the  retina  before  they  have  reached 
their  focus,  a  suitable  convex  cylindrical  lens,  having  its  refracting  me- 
ridian horizontal  (its  axis  vertical),  will  so  add  to  the  refraction  in  the 
horizontal  meridian  that  the  rays  will  be  focused  on  the  retina  in  this 
as  well  as  in  the  vertical  meridian ;  in  other  words,  the  rays  will  be 
focused  in  a  point  on  the  retina  and  the  astigmia  will  be  overcome. 
Wlien,  as  in  this  case,  the  eye  is  emmetropic  in  one  meridian  and  hy- 
peropic  in  the  other,  the  condition  is  called  simple  hyperopic  astigmia. 

If  with  the  same  asymmetry  of  curvature  the  eyeball  is  slightly 
larger  than  in  the  former  case,  so  that  the  horizontal  focus  falls  on  the 
retina  while  the  vertical  focus  is  in  front  of  the  retina,  the  condition  is 
simple  myopic  astigmia.  In  this  case  rays  will  be  focused  in  a  point 
on  the  retina  with  the  aid  of  a  suitable  concave  cylindrical  lens,  axis 
horizontal,  since  this  will  throw  the  vertical  focus  back  to  the  retina 
without  aflFecting  the  rays  in  the  emmetropic  horizontal  meridian. 

If  both  ffX'i  are  behind  the  retina,  one  farther  than  the  other  (com- 
pound hyperopic  astigmia),  a  convex  spherical  lens  must  be  added  to 
the  approj)riate  cylindrical  lens  in  order  to  bring  the  focus  on  the 
retina.  If,  on  the  other  hand,  both  foci  are  in  front  of  the  retina  (com- 
pound  myopic  astigmia)  a  concave  spherical  lens  must  be  added  to  the 
appropriate  cylinder.  It  may  hapix»n  evidently  that  the  retina  is  situ- 
ated between  the  two  foci,  one  of  these  being  in  front  of  the  retina  and 
the  other  behind  it  {mixed  astigmia).  In  this  case  a  convex  cylinder, 
such  as  will  equalize  the  refraction  in  the  two  principal  meridians  and 
thus  bring  the  rays  to  a  focus  in  front  of  the  retina,  may  be  combined 
with  a  concave  spherical  lens  of  sufficient  strength  to  throw  the  focus 
back  u)X)n  the  retina ;  or  a  concave  cylinder,  such  as  will  equalize  the 
refraction  but  throw  the  focus  l)ehind  the  retina,  may  be  combined  with 
a  convex  spherical  lens  to  bring  the  focus  forward  upon  the  retina.*^ 

•  Sphero-cylindricnl  lenses  for  the  correction  of  compound  and  mixed  astigmia 
have  the  spherical  element  ground  upon  one  side  of  the  glass  and  the  cylindrical  upon 
the  other.  In  tlie  correction  of  compound  astigmia  both  surfaces  should  be  convex 
or  both  concave,  according  as  the  condition  is  that  of  hyperopic  or  myopic  astigmia, 
but  in  the  correction  of  mixed  astigmia  one  surface  must  be  convex  and  the  other 
concave.  The  sphero-cylinder  is  the  common  form  of  lens  used  in  the  correction  of 
these  errors,  but  it  is  possible  to  substitute  for  this  form  of  glass  a  crossed- 
cylindcr  (one  cylinder  on  each  side  of  the  glass,  the  two  axes  being  at  right 
angles),  or  a  toric  lens,  in  which  the  proper  asymmetrical  curvature  is  ground  upon 
one  face  of  the  glass. 


STRUCTURE  A:N^D  OPTICAL  PROPERTIES  49 

The  degree  of  astigmia  is  measured  by  the  dioptric  power  (in  the 
refracting  meridian)  of  the  cylindrical  lens  which  equalizes  the  refrac- 
tion. The  degree  may  vary  from  .12  D.  (the  weakest  lens  commonly 
made)  to  15  D.  or  more.  Almost  all  eyes  have  as  much  asymmetry  as  is 
indicated  by  the  former  limit,  but  the  higher  degrees  are  fortunately 
uncommon.  In  fact,  astigmia  of  more  than  5  D.  or  6  D.  is  not  often 
encountered.  In  the  lower  grades  of  asymmetry  the  effect  upon  vision 
is  very  slight,  and  in  many  such  cases  no  disturbance  of  any  kind 
results ;  on  the  other  hand,  in  persons  who  are  engaged  in  close  applica- 
tion of  the  eyes  astigimia  of  .25  D.  or  even  .12  D.  may  produce  asthen- 
opia, which  is  relieved  only  by  the  use  of  correcting  lenses. 

The  treatment  of  regular  astigmia  consists  in  the  adaptation  (as  will 
subsequently  be  explained)  of  the  appropriate  cylindrical  lens.  In  the 
lower  grades  the  lens-correction  may  be  worn  only  in  close  application 
of  the  eyes,  but  in  the  higher  grades,  preferably  in  all  reaching  1  D., 
the  correction  should  be  worn  constantly. 

Irregular  astigmia  exceeding  the  physiological  amount  is  most  com- 
monly due  to  scar  formation  in  the  cornea  or  to  irregular  swelling  of 
the  crystalline  lens  in  the  premonitory  stage  of  cataract.  This  kind  of 
astigmia  is  incapable  of  correction. 

Anisometropia. — Since  each  individual  is  normally  endowed  with 
a  pair  of  eyes,  which  work  together  in  the  function  of  vision,  the  rela- 
tive refractive  condition  of  the  tw'o  eyes  is  a  matter  of  great  importance. 
The  ideal  condition  is  evidently  that  in  which  the  refraction  is  alike  in 
the  two  eyes ;  this  is  called  isometropia.  But  more  commonly  an  abso- 
lute correspondence  of  the  two  eyes  is  not  found ;  one  eye  may  be  emme- 
tropic and  the  other  hyperopic,  myopic,  or  astigmic;  or  both  eyes  may 
have  the  same  defect,  the  degree  being  higher  in  one  eye  than  in  the 
other ;  or  one  eye  may  have  hyperopia  and  the  other  myopia.  Any  such 
deviation  from  isometropia  is  called  anisometropia,  and  the  special  con- 
dition in  which  one  eye  is  hyperopic  and  the  other  myopic  is  usually 
called  antimetropia.  Wliile  it  is,  in  most  cases,  possible  to  detect  a 
slight  difference  between  the  two  eyes,  it  is  only  w^hen  the  disparity  is 
marked  that  anisometropia  should  be  regarded  as  an  anomaly,  capable 
of  giving  rise  to  asthenopia  or  other  visual  disturbance. 

In  the  treatment  of  anisometropia  the  ophthalmologist  endeavors  to 
equalize  the  condition,  as  far  as  practicable,  in  the  two  eyes  by  giving 
to  each  eye  its  appropriate  correcting  lens.  In  childhood  quite  a  high 
disparity  may  thus  be  overcome,  but  in  those  cases  which  have  not  been 
corrected  prior  to  adult  life  the  correction  of  the  defect  will,  in  the  more 
marked  cases,  rarely  be  tolerated.  In  some  of  these  cases  vision  is 
4 


50  THE  EYE  AND  NERVOUS  SYSTEM 

performed  with  both  eyes,  the  clear  image  formed  in  one  eve  being 
fused  with  tlie  blurred  image  formed  in  the  other,  but  in  very  many 
cases  vision  is  uniociilar,  the  better  eye  being  used,  while  the  poorer  eye 
falls  into  a  state  of  relative  deviation  or  squint.  On  this  account  it  is 
of  the  utmost  importance  that  anisometropia  should  be  corrected  in 
early  childhood. 

Determixatiox  of  Visual  Acuteness  and  of  the 
Refractive  Condition  of  an  Eve. 
The  visual  power  is  measured  by  the  least  interval  which  permits 
the  eye  to  distinguish  as  such  two  bright  or  white  points  on  a  black 
ground.  In  the  normal  eye  this  minimum  interval  subtends  an  angle 
of  about  one  minute  at  the  nodal-point.  This  angle  is  called  the  mini- 
mum visual  angle.  For  practical  vision-testing  it  is  more  convenient 
to  use  a  series  of  test-letters,  so  constructed  that  when  they  are  placed 
at  the  proper  distance,  the  breath  of  each  stroke  of  the  letter  sub- 
tends the  minimum  visual  angle  at  the  nodal-point  of  the  eye  under- 
going examination,  while  each  dimension  of  the  whole  letter  subtends 
an  angle  of  five  minutes  (Fig.  17).     For  determining  visual  acuteness 

Fig.  17. 


cards  are  furnished  having  printed  on  them  letters  of  various  sizes,  the 
distance  at  which  each  letter  subtends  the  five-minute  angle  being  noted 
on  the  card.  Thus,  if  at  a  distance  of  six  metres,  a  person  can  dis- 
tinguish those  letters  which,  as  marked  on  the  card,  subtend  the  five- 
minute  angle  at  this  distance,  the  visual  acuteness  is  normal  (F^| 
or  y  =  1)  ;  but  if  at  this  distance  the  smallest  distinguishable  letters 
are  tliose  which  sul)tend  the  five-minute  angle  at  twelve  metres,  the 
visual  power  is  only  j%  of  the  normal  acuteness  {V  =  y^).  The  visual 
acuteness  of  any  eye  is  therefore  measured  by  a  fraction  whose  numer- 
ator is  the  distance  at  which  the  test  is  conducted  and  whose  denominator 
is  the  distance  at  which  the  smallest  distinguishable  letters  subtend  the 
five-minute  angle. 

It  not  infrequently  happens,  especially  in  young  persons,  that  the 
visual  power  exceeds  the  standard  wliicli  lias  l)een  accepted  as  the  aver- 


STJIUCTURE  A:N'D  OPTICAL  PROPERTIES  51 

age  vision  of  normal  eyes.     We  thus  have  the  expressions  F  ^^  f ,  or 

T7 6 

\  — -f. 

The  minimum  visual  angle  is  the  basis  of  the  most  valuable  method 
of  determining  the  refractive  condition  of  the  eye.  The  refraction  of 
the  eye  indicates  the  relation  between  the  position  of  the  retina  and  pos- 
terior focus  for  parallel  rays,  when  the  accommodation  is  relaxed.^ 
Hence  in  determining  the  refraction  the  test-letters  must  be  at  such  a 
distance  that  the  rays  proceeding  therefrom  may  be  regarded  as  parallel ; 
that  is,  the  letters  must  be  five  or  preferably  six  metres  (20  feet)  from 
the  eye  undergoing  examination.  With  the  letters  at  this  distance  nor- 
mal vision  is  possible  only  in  emmetropia  or  in  hyperopia  with  use  of 
accommodation,  since  full  visual  acuteness  necessitates  an  accurate  focus 
of  the  image  on  the  retina.  To  decide  between  these  two  conditions  we 
place  a  weak  convex  spherical  lens  before  the  eye  under  examination 
(the  other  eye  being  excluded  from  vision  by  an  opaque  disk)  ;  if  this 
lens  does  not  render  vision  worse  (hazy)  accommodation  has  been  re- 
placed by  the  convergent  action  of  the  lens.  By  trying  successively 
stronger  lenses  we  find  the  strongest  lens  which  does  not  impair  vision. 
This  measures  the  manifest  hyperopia.  In  old  persons  it  also  measures 
the  total  hyperopia,  but  in  children  and  young  adults  a  certain  portion  of 
the  hyperopia  is,  as  we  have  learned,  latent.  In  fact  the  whole  of  the 
liyperopia  may  be  latent,  so  that  the  weakest  convex  lens  will  make  vision 
worse.  AVhen  latent  hyperopia  cannot  be  otherwise  excluded  a  cyclo- 
plegic  must  be  instilled  into  the  conjunctival  sac,  and  the  examination 
made  while  the  eye  is  under  the  influence  of  the  drug.  If  under  this 
condition  vision  remains  normal,  the  eye  is  emmetropic;  but  if  the 
vision,  which  before  was  normal,  falls  below  normal  during  cycloplegia, 
the  convex  spherical  lens  which  affords  maximum  vision  measures  the 
total  hyperopia. 

ITomatropin-hydrobromate  is  the  most  suitable  cycloplegic  for  gen- 
eral use ;  one  drop  of  a  one-and-one-half  per  cent,  solution  instilled  at 
ten-minute  intervals  until  six  applications  have  been  made  will,  except 
in  rare  instances,  produce  complete  paralysis  of  the  accommodation  in 
one  hour  after  the  last  instillation.  The  effect  of  the  drug  passes  off  in 
about  twenty-four  hours.  In  young  children  and  in  other  persons  in 
whom  a  prolonged  enforced  rest  of  the  accommodation  is  desirable 
atropin  may  be  used.  To  insure  complete  paralysis,  in  execessive  accom- 
modative activity,  one  drop  of  a  one  per  cent,  solution  of  atropin-sul- 
phate  may  be  instilled  three  times  a  day  for  several  days,  though  usually 

•  This  is  the  static  refraction,  in  contradistinction  to  the  dynamic  refraction, 
which  refers  to  the  accommodative  power. 


52  THE  EYE  AND  NERVOUS  SYSTEM 

complete  paralysis  occurs  in  one  and  one-half  hours  after  the  first  in- 
stillation. The  full  accomnioclative  activity  is  not  restored  until  about 
two  weeks  after  the  last  instillation. 

The  use  of  a  cycloplegic  is  contraindicated  when  there  is  any  ten- 
dency to  glaucoma ;  but  as  this  affection  seldom  occurs  in  young  per- 
sons, who  alone  require  a  cycloplegic  for  the  determination  of  refraction, 
there  is  ordinarily  no  danger  in  the  use  of  a  cycloplegic  for  this  purpose. 

If  vision  is  less  than  normal  at  the  first  examination  (without  cyclo- 
plegia),  the  defect  of  vision  is  due,  if  to  any  error  of  refraction,  either 
to  hyperopia  without  sufficient  accommodation  to  overcome  it,  or  to 
myopia,  or  to  astigmia.  The  first  of  these  conditions  is  determined  by 
the  improvement  effected  by  placing  a  convex  spherical  lens  before  the 
eye.  On  the  other  hand,  the  presence  of  myopia  is  revealed  by  the  fact 
that  vision  is  rendered  worse  by  a  convex  lens  and  improved  by  a  con- 
cave lens.  But  while  in  hyperopia  the  strongest  acceptable  lens  measures 
the  ametropia,  in  myopia  the  weakest  lens  which  affords  maximum 
vision  must  be  selected,  for  if  a  stronger  lens  is  taken,  normal  vision 
may  still  be  possible  with  the  aid  of  accommodation.  In  fact,  so  active 
is  accommodation  in  young  subjects  that  overaction  of  the  ciliary  muscle 
(spasm  of  accommodation)  sometimes  gives  rise  to  an  apparent  myopia, 
whereas  the  true  condition  is  that  of  emmetropia  or  hyperopia.  Wlien 
there  is  any  reason  for  suspecting  an  overaction  of  the  ciliary  muscle 
the  refractive  condition  should  be  determined  while  the  eye  is  under  the 
influence  of  a  cycloplegic.  In  fact  it  is  better  in  most  cases  to  use  a 
cycloplegic  for  the  determination  of  refraction  in  persons  under  thirty 
years  of  age,  since  by  this  means  only  can  the  static  refraction  be  de- 
termined with  absolute  certainty. 

Wlien  neither  a  convex  nor  a  concave  spherical  lens  brings  the  vision 
up  to  the  normal  standard,  we  suspect  the  presence  of  astigmia.  If  this 
is  regular  it  may  be  corrected  by  a  cylindrical  lens.  If  a  spherical  lens 
has  been  found  which  improves  vision,  this  should  remain  before  the 
eye  in  the  trial  frame  in  conducting  the  examination  for  the  determina- 
tion of  the  existence  of  astigmia.  In  the  latter  procedure  we  place  a 
weak  convex  cylindrical  Ions  before  tlio  eye;  and,  since  more  commonly 
the  eye  has  its  greatest  curvature  in  the  vertical  meridian,  we  place  the 
axis  of  the  lens  in  this  meridian.  If  in  this  position  it  does  not  improve 
vision,  we  rotate  the  lens  to  ascertain  whether  in  any  other  position  it 
effects  an  improvement."^  If  we  thus  succeed  in  finding  a  position  in 
which  the  lens  improves  vision,  we  try  stronger  lenses  until  we  find 

^The  position  of  a  cylindrical  lens  is  registered  in  accordance  with  the  direc- 
tion of  its  axis,  as  indicated  by  the  angular  marking  on  the  trial  frame. 


STKUCTURE  A^B  OPTICAL  PROPERTIES  53 

that  lens  which  gives  normal  vision.  If  the  weak  convex  cylinder  is  not 
accepted  in  any  meridian,  we  next  try  a  concave  cylinder.  In  this  case 
we  first  place  the  axis  in  the  horizontal  meridian,  since  the  probability  is 
that  the  vertical  curvature  is  excessive.  We  then  proceed  as  with  convex 
cylinders  until  we  find  tlie  position  and  the  lens  which  give  best  vision. 
If  we  have  difficulty  in  determining  the  directions  of  the  principal  me- 
ridians by  the  foregoing  method,  we  may  make  use  of  the  clock-face 
chart  (Fig.  18),  which  consists  of  a  series  of  radiating  lines,  numbered 


in  the  same  manner  as  a  clock  face.  If  (after  correction  of  any  exist- 
ing hyperopia  or  myopia)  one  group  of  these  lines  appears  very  dis- 
tinct while  the  other  lines,  and  especially  the  group  at  right  angles  to 
the  group  of  greatest  distinctness,  are  blurred,  the  eye  is  astigmic,  and 
the  lines  of  greatest  distinctness  lie  in  the  meridian  which  requires 
correction.^  Hence  the  axis  of  the  cylindrical  lens  should  be  placed  in 
the  direction  of  the  most  blurred  lines.  If  a  convex  lens  having  its 
axis  in  this  direction  does  not  improve  vision,  we  try  a  concave  lens 
with  its  axis  in  the  same  direction.  The  lens  which  equalizes  all  the 
lines  and  at  the  same  time  affords  normal  vision  is  the  correction  of 
the  astigmia. 

While  simple  refractive  errors  in  eyes  having  normal  visual  acute- 
ness  may  be  easily  measured  by  the  method  of  examination  just  de- 
scribed, those  cases  in  which  hyperopia  or  myopia  is  complicated  by 
astigmia,  and  especially  in  eyes  having  imperfect  visual  acuteness,  much 
time  may  be  saved  and  gi'eater  accuracy  secured  by  the  aid  of  objective 

*  If,  for  instance,  the  eye  is  emmetropic  in  the  horizontal  meridian,  but  not  so 
in  the  vertical  meridian,  a  distant  point  will  have  its  horizontal  focus  on  the 
retina,  while  in  the  vertical  meridian  the  rays  will  be  diffused  on  the  retina,  that 
is,  the  image  of  a  distant  point  will  be  a  vertical  line;  therefore  since  every  point 
of  a  vertical  line  will  have  a  corresponding  vertical  line  as  its  image,  the  image 
will  be  a  sharply  defined  line.  But  if  the  line  is  horizontal  instead  of  vertical, 
that  is.  if  the  line  lies  in  the  emmetropic  meridian,  every  point  of  the  horizontal 
line  will  have  a  short  vertical  diffusion  line  as  its  image,  and  the  image  of  the 
whole  line  will  be  diffused  and  blurred. 


n-t  THE  EYE  AND  NERVOUS  SYSTEM 

methods  of  examination.  The  most  valuable  of  these  methods  are 
ophthalmoscopy,  skiascopy,  and  keratometry. 

The  refractive  condition  may  be  determined,  with  a  degree  of  accu- 
racy varying  witli  the  experience  and  skill  of  the  examiner,  by  direct 
ophthalmoscopy.  If  the  examiner  is  emmetropic  (or  has  his  ametropia 
corrected  by  a  lens)  the  hyperopia  of  the  examined  eye  is  measured  by 
the  strongest  convex  ophthalmoscopic  lens  with  which  the  small  retinal 
vessels  can  be  distinctly  seen ;  while  myopia  is  measured  by  the  weakest 
concave  lens  with  which  these  vessels  are  distinctly  seen.  Astigmia  is 
measured  by  the  difference  between  the  lens  which  corrects  the  ame- 
tropia in  one  principal  meridian  (as  determined  by  distinctness  of  the 
retinal  vessels  at  right  angles  to  this  meridian)  and  the  lens  which 
corrects  the  ametropia  in  the  other  principal  meridian.  The  accurate 
estimation  of  astigmia  in  this  way  is  very  difficult ;  in  fact,  it  is  not 
usually  attempted,  since  the  astigmia  can  be  much  more  easily  de- 
termined by  skiascopy. 

Skiascopy  consists  in  the  estimation  of  the  refractive  condition  from 
the  movement  of  the  shadow  which  passes  across  the  pupil  of  the  ex- 
amined eye  when  the  illuminating  mirror  is  rotated.  The  movement  of 
this  shadow  varies  with  the  position  of  the  observer  (who  looks  through 
the  sight  hole  of  the  mirror),  with  reference  to  the  far-point  of  the 
examined  eye.  If  the  examiner  is  at  this  point  there  is  no  perceptible 
movement  of  the  shadow,  but,  as  the  mirror  is  shifted,  the  whole  pupil 
is  simultaneously  enshrouded  in  darkness.  If  the  examiner  is  nearer 
the  eye  than  the  far-point,  he  sees  a  shadow  move  across  the  pupil  in  the 
direction  in  which  he  rotates  the  mirror,  the  rate  of  movement  diminish- 
ing as  he  departs  from  the  far-point.  If,  on  the  other  hand,  he  is 
farther  from  the  e^e  than  the  far-point,  he  sees  the  shadow  move  in  the 
opposite  direction  to  the  mirror  rotation.^  The  explanation  of  this 
change  of  movement  is,  found  in  the  fact,  that  the  rays  proceeding  from 
any  point  of  the  retina  meet  at  the  far-point  of  the  eye  and  have  their 
relative  position  reversed  in  passing  this  point.  On  this  account  the 
far-point  is  called  the  point  of  reversal  of  the  eye.  We  know  that  in 
emmetropia  the  far-point  (the  anterior  conjugate  to  the  retina)  is  at 
infinity,  and  in  hyperopia  it  is  negative  or  behind  the  retina ;  hence  it  is 
only  in  myopia  that  the  examiner  can  place  himself  at  the  point  of 
reversal.  If,  for  instance,  he  finds  that  there  is  no  perceptible  shadow 
at  a  distance  of  one  metre,  while  the  shadow  moves  opposite  to  the 

•The  movements  as  described  are  those  that  take  place  with  a  plane  mirror; 
if  a  concave  mirror  is  used,  the  direction  of  the  movement  is  in  all  cases  opposite 
to  that  described  for  the  plane  mirror. 


STRUCTURE  AND  OPTICAL  PROPERTIES  55 

mirror  when  he  moves  to  a  greater  distance,  and  in  the  same  direction 
as  the  mirror  when  he  moves  nearer  the  eye  than  the  one-metre  limit, 
he  knows  that  the  eye  has  1  D.  of  myopia,  since  the  degree  of  myopia  is 
measured  by  the  distance  of  the  far-point  from  the  eye  (Fig.  16).  But 
if  as  the  examiner  moves  away  from  the  eye  the  shadow  continues  to 
move  in  the  same  direction  as  the  mirror,  he  may  place  a  convex  spherical 
lens  before  the  eye  so  as  to  render  it  artificially  myopic.  Thus,  if  with 
a  convex  lens  of  1  D.  the  shadow  movement  is  annulled  at  a  distance  of 
1  metre,  the  eye  must  be  emmetropic,  since  one  diopter  of  myopia  is 
produced  by  a  lens  of  1  D.  But  if  it  requires  a  lens  of  2  D.  to  bring  the 
point  of  reversal  to  the  one-metre  distance,  the  eye  must  have  1  D.  of 
hyperopia.  In  other  words,  if  the  examiner  places  himself  at  a  dis- 
tance of  one  metre  from  the  eye,  the  hyperopia  is  always  1  D.  less  than 
the  dioptric  power  of  the  lens  which  annuls  the  shadow-movement.  In 
myopia  of  more  than  1  D.  the  examiner  may  approach  the  eye  until  he 
reaches  the  point  of  reversal  and  thus  measure  the  distance  of  the  far- 
point  directly,  or  he  may  (as  is  generally  preferable)  remain  at  the  one- 
metre  position  and  place  before  the  eye  such  a  concave  lens  as  will  bring 
the  point  of  reversal  to  this  position.  The  lens  which  effects  this,  neu- 
tralizes all  but  1  D.  of  the  existing  myopia ;  that  is,  the  myopia  is  1  D. 
^eater  than  the  dioptric  power  of  the  lens  which  annuls  the  shadow- 
movement. 

If  in  the  application  of  skiascopy  we  find  (either  with  or  without  a 
spherical  lens)  no  shadow-movement  in  one  meridian,  while  a  shadow 
appears  to  move  across  the  pupil  in  the  meridian  at  right  angles  to  this, 
the  existence  of  astigmia  is  demonstrated,  and  the  meridian  in  which 
there  is  no  movement  is  one  principal  meridian,  while  the  other  is  the 
meridian  in  which  the  shadow  appears  to  move.  The  degree  of  astigmia 
is  then  measured  by  the  cylindrical  lens,  having  its  axis  in  the  meridian 
of  no  movement,  which,  either  alone  or  together  with  a  spherical  lens, 
annuls  the  movement  in  all  meridians. 

Keratometry,  or  ophthalmometry,  consists  in  the  measurement,  by 
means  of  images  reflected  at  the  anterior  surface  of  the  cornea,  of  the 
curvature  of  this  surface,  and  the  deduction  therefrom  of  the  corneal 
asymmetry  in  terms  of  the  dioptric  equivalent  of  the  resulting  astigmia ; 
but  since  the  corneal  astigmia  is  to  a  variable  extent  modified  by  the 
lenticular  astigmia,  which  is  not  revealed  by  keratometry,  this  method 
is  less  valuable  than  skiascopy;  it  is,  nevertheless,  very  useful  as  a 
corroborative  test. 

Determination  of  the  Accommodative  Power. — The  accommodative 
power  may  be  most  conveniently  measured  by  means  of  small  test-letters 


56  THE  EYE  AND  NERVOUS  SYSTEM 

constructed  on  the  five-minute-angle  principle,  as  have  been  devised  by 
Oliver.  If  the  eye  is  emmetropic,  the  accommodative  power  is  measured 
by  the  shortest  distance  from  the  eye  at  which  the  smallest  distinguish- 
able letters  can  be  read.  If  the  eye  is  ametropic,  its  refractive  error 
should  be  first  corrected,  and  the  examination  then  conducted  as  in 
emmetropia. 


CHAPTER  III. 

THE  PSYCHOLOGY  OF   THE  VISUAL  ACT  AND  THE  FOCAL 
DISEASES   OF   THE  VISUAL   COETEX. 

By  CHARLES  K.  MILLS,  M.D. 

INTRODUCTORY  OUTLINE. 

The  visual  act  may  be  described  in  general  terms  as  that  process  by 
which  luminosity,  form  or  color  is  recognized  by  the  organs  of  vision. 
Usually  the  act  is  concerned  with  the  visual  recognition  of  a  definite 
object.  The  term  has,  however,  an  application  to  subjective  visual 
processes  as  illustrated,  for  instance,  by  hallucinations  of  sight  either  in 
the  normal  or  in  the  insane,  and  by  the  recognition  of  phosphenes  due 
to  mechanical  or  electrical  stimulation.  My  concern  in  the  present 
chapter  is  chiefly  with  the  visual  act  as  related  to  the  cerebral  cortex,  and 
especially  to  the  higher  cortical  areas  and  centres  of  vision.  The  con- 
sideration of  the  subject  must  necessarily  include  to  a  certain  extent 
both  the  physiology  and  the  patholog}^  of  the  visual  cortex.  The  cortical 
visual  act  may  be  simple  or  complex  according  to  the  simplicity  or  com- 
plexity of  the  object  recognized,  which  may  vary  from  a  luminous  point 
of  weak  intensity  and  brief  duration  through  a  series  including  forms 
of  all  grades  of  regularity  or  irregularity,  colors  differing  in  hues  and 
in  shades,  natural  objects  of  diverse  sort,  particular  landscapes,  persons 
with  individuality  of  face  and  figure,  designs  originating  in  the  arts  and 
sciences,  and  numbers,  letters  and  words. 

Under  the  influence  of  the  recent  discovery  of  radium  and  its  effects, 
some  tendency  is  shown  among  physicists  to  return  to  the  corpuscular 
theory  of  light ;  nevertheless  the  still  most  universally  accepted  doctrine 
is  that  which  is  based  upon  the  Newtonian  hypothesis  of  luminous  trans- 
mission by  Avaves  of  the  ether  which  permeates  all  space. 

Unlike  sound  vibrations,  the  direction  of  the  waves  of  the  vibrating 
ether  is  transverse  to  the  direction  of  the  rays  of  light.  Only  those  ethe- 
real vibrations  which  have  a  velocity  of  between  four  hundred  billions  on 
the  one  hand  and  a  little  more  than  nine  hundred  billions  on  the  other 
hand  are  effective  in  giving  rise  to  sensations  of  sight. 

To  others  in  this  work  are  assigned  the  tasks  of  not  only  describing 
and  explaining  the  anatomy  and  methods  of  action  of  the  components 
of  the  eye  itself,  but  also  of  many  of  the  effects  which  result  from  the 

5.7 


68  THE    EYE    AXD    NERVOUS    SYSTEM 

reception  of  light  by  the  nervous  apparatus  of  vision.  I  shall  consider 
only  the  ultimate  cerebral,  and  therefore  psychical,  effects  of  the  light 
transmitted  through  the  eye  to  the  nervous  system.  In  the  retina  are 
certain  photo-chemical  substances  and  a  complex  anatomical  nervous 
structure,  the  chief  constituents  of  which  are  known  as  the  rods  and 
cones.  To  and  from  these  rods  and  cones  come  and  go  transmitting  ner- 
vous structures  which  put  the  retinal  elements  in  communication  with 
lower  and  higher  levels  of  the  brain. 

A  light  wave  starts  on  its  journey  through  the  ether  from  the  lumin- 
ous object  which  is  its  source.  The  first  step  towards  its  becoming 
a  visual  impulse  is  taken  when  it  decomposes  the  photo-chemical  sub- 
stances of  the  retina,  thus  setting  up  vibrations  in  the  extreme  periphery 
of  the  end  organs  of  this  membrane.  The  initial  psychical  step  is  taken 
when  it  reaches  its  first  stopping  j)lace  in  the  brain  cortex. 

The  excitation  received  by  the  retinal  substances  and  structures  is 
conveyed  by  fibres  of  the  optic  nerve  back  to  centres  at  the  base  of  the 
brain,  and  either  directly  or  by  new  relays  of  fibres  to  the  visual  cortex. 
Definite  portions  of  the  retina  are  related  to  equally  definite  portions  of 
the  visual  centre  which  first  received  the  projected  retinal  excitations — a 
matter  which  will  later  receive  attention  in  more  detail.  The  result  in 
the  cortical  centres  first  receiving  the  impulses  is  a  visual  sensation  or 
percept.  Up  to  this  point,  however,  an  idea  of  the  object  perceived  is  not 
obtained.  In  order  that  this  shall  come  to  pass  the  cortical  excitation 
which  has  been  evoked  must  be  transmitted  to  the  cellular  elements  of 
another  region  of  the  brain  surface ;  in  other  words,  from  a  simple  sen- 
sory or  percei)t  centre  to  a  memory  centre.  A  memorial  image  is  de- 
posited in  the  centre  placed  at  some  distance  from  the  cell  or  cell-group 
in  which  the  excitation  is  received. 

WTien  such  focal  diseases  of  the  visual  cortex  as  mind-blindness,  word- 
blindness  and  letter-blindness  are  considered,  the  fact  that  visual  sensa- 
tions or  percepts  and  visual  concepts  or  appercepts  are  dependent  upon 
separate  processes  and  have  distinct  cortical  habitations,  will  more 
clearly  api)car. 

One  who  is  mind-blind  or  word-blind  so  that  he  may  not  be  able  to 
recognize  persons  or  objects  in  their  real  characters,  or  w^ords  and  letters 
in  their  true  significance,  may  yet  have  visual  sensations  with  their  cor- 
tical correlatives,  as  illustrated  by  the  fact  that  he  can  avoid  or  seek  the 
objects  which  he  does  not  recognize  and  that  he  may  see  the  words  and 
the  letters  composing  them  and  yet  not  be  able  to  tell  what  they  import, 
although  he  knows  that  they  are  different  from  the  other  objects  in  his 
field  of  vision. 


THE  PSYCHOLOGY  OF  VISION  59 

LIMITS  AND   SUBDIVISIONS   OF  THE  VISUAL   CORTEX. 

I  shall  next  define  the  limits  and  subdivisions  of  the  visual  cortex  as 
I  understand  them.  The  visual  cortex,  that  part  of  the  convoluted  cere- 
bral surface  concerned  with  the  function  of  sight,  includes  the  entire 
occipital  lobe  both  on  its  mesotentorial  and  its  lateral  aspects;  also 
taking  in  adjoining  caudal  portions  of  the  parietal  and  temporal  lobes. 
This  cortical  visual  field  is  of  complex  composition.  It  includes  a  pri- 
mary or  lower  and  a  secondary  or  higher  cortical  visual  area.  The 
higher  area  is  extensively  subdivided.  The  primary  or  lower  cortical 
visual  area  which  corresponds  to  the  optic  perception  field  of  Wilbrand 
and  Saenger^,  is  situated  chiefly,  but  not  exclusively,  on  the  mesotento- 
rial surface  of  the  occipital  lobe ;  the  secondary  or  higher  cortical  visual 
area  corresponding  to  the  optic  memory  field  of  the  authorities  just  men- 
tioned, covers  a  large  extent  of  the  lateral  aspect  of  the  occipital  lobe, 
spreading,  to  some  extent,  over  the  temporal  and  tentorial  surfaces. 

In  order  to  understand  the  limits  and  subdivisions  of  the  visual  cor- 
tical areas,  in  accordance  with  the  broad  statements  just  made,  it  is 
necessary  to  have  some  idea  of  the  views  of  Flechsig.^ 

His  views  are  based  upon  his  studies  in  myelinization  as  especially 
observed  in  the  brain  of  the  foetus  and  of  the  infant  shortly  after  birth. 
He  believes  that  the  nerve  fibres  connected  with  special  cortical  areas 
become  medullated  at  certain  definite  periods,  these  myelogenetic  areas 

*  Wilbrand  and  Saenger,  Die  Neurologic  des  Auges,  vol.  iii.  Anatomie  und 
Physiologie  der  Optisehen  Bahnen  und  Centren,  Wiesbaden,  1904. 

'■'  Flechsig,  Gehirn  und  Seele,  ii.  Aufl.,  Leipz.,  1896,  Taf.  iv.  Flechsig's  views 
are  presented  in  a  condensed  form  in  an  article  over  his  own  signature  in  The 
Lancet,  vol.  ii.  1901,  p.  1027. 

The  references  somewhat  frequently  made  to  Wilbrand  and  Saenger,  unless 
otherwise  indicated,  refer  to  the  volume  above  noted.  Much  use  has  been  made  of 
this  valuable  book,  which  is  especially  rich  in  its  summary  of  the  work  of  German 
writers  and  investigators.  In  order  not  to  overload  the  chapter  by  a  repetition  of 
references,  I  might  here  call  attention  to  some  of  the  most  important  monographs 
or  treatises  used  in  its  preparation,  although  I  cannot  make  this  list  complete. 
These  include  Kussmaul's  Disturbances  of  Speech  in  Ziemssen's  Cyclopaedia  of  the 
Practice  of  Medicine,  Amer.  edition,  vol.  xiv..  New  York,  1877;  Ferrier's  Functions 
of  the  Brain,  1886,  and  Croonian  Lectures  on  Cerebral  Localization,  1890;  Gowers's 
Diseases  of  the  Nervous  System,  1893,  and  his  Clinical  Lectures  on  Subjective  Sen- 
sations of  Sight  and  Sound,  1904;  Wyllie's  Disorders  of  Speech,  1894;  and  v. 
Monakow's  Gehirnpathologie,  Wien,  1897.  Although  it  is  giving  scant  justice  to 
others,  space  will  only  permit  me  by  naming  them  to  acknowledge  my  great  in- 
debtedness to  Hitzig,  Munk,  Horsley,  Schiifer,  Beevor,  Saenger  Brown,  Dejerine, 
Henschen,  Pick,  Bateman,  de  Schweinitz,  Swanzey,  Oliver,  and  Elder,  among  many 
others.  Besides  this  general  acknowledgment,  references  are  made  in  various  parts 
of  the  chapter  to  particular  contributions. 


60 


THE  EYE  AND  NERVOUS   SYSTEM 


having  special  relation  to  the  evolution  of  particular  functions.  Some 
of  the  areas  appear  at  the  same  time,  others  at  a  time  separated  by  weeks 
or  months  from  the  areas  which  have  preceded  them.  Thirty-six  of 
these  areas  have  been  marked  out  by  Flechsig  on  the  cerebral  cortex. 


Fio.  1.— Flechsig's  primordial  and  association  areas  on  the  lateral  aspect  of  the  human  hemicerebrum ; 

the  primordial  areas  are  shaded. 

In  this  connection  our  chief  concern  is  with  Flechsig's  great  sub- 
divisions of  the  cortex  into:  (1)  primordial  zones,  and  (2)  association 
areas.  The  primordial  zones  correspond  to  the  so-called  projection 
areas,  the  regions  of  cortical  representation  of  the  special  senses,  com- 
mon sensibility  and  of  movements.    The  association  areas  occupy  a  large 


Fio.  2.— Flechsig's  primordial  and  association  areas  on  the  mesal  aspect  of  the  haman  hemicerebrum  ; 

the  primordial  areas  are  shaded. 

portion  of  the  cerebral  surface,  nearly  two-thirds  in  all.  They  are  situ- 
ated in  those  regions  of  the  brain  which  were  in  times  past  often  desig- 
nated as  latent  simply  because  we  did  not  know  their  functions.  Instead 
of  being  functiouless  they  are  the  seat  of  the  highest  and  most  special- 


THE  PSYCHOLOGY  OF  VISIO:^^ 


61 


ized  cerebral  attributes.  These  association  areas  were  divided  by  Flech- 
sig  into  two,  a  posterior  and  an  anterior.  The  posterior  association  area 
occupies  both  the  lateral  and  the  mesal  aspects  of  the  brain  and  includes 
portions  of  the  parietal,  temporal  and  occipital  lobes.  To  it  Flechsig 
also  gave  the  name  of  intermediary  area.  The  anterior  association  area 
which  is  situated  exclusively  in  the  prefrontal  region  has  also  been 
designated  by  Flechsig  as  the  terminal  zone.  These  primordial  and 
association  areas  are  represented  diagrammatically  in  Fig.  1  and  Fig.  2. 


CONCEPT 


Fio.  3. — Diagrammatic  sciieme  of  tlie  ptiysiological  areas  and  centres  of  ttie  lateral  asjiect  of  the  human 

hemicerebrum. 

I  have  suggested  that  the  term  concrete  concept  area  should  be 
applied  to  Flechsig's  posterior  or  intermediary  area.  It  includes  the 
optic  memory  field  of  Wilbrand  and  Saenger  and  much  more,  the  mem- 
ory field  directly  related  to  all  of  the  senses ;  indeed,  another  designation 
which  would  be  expressive  of  the  functions  of  this  great  area  would  be 
the  concrete  memory  field.  This  concrete  concept  area  or  concrete 
memory  field  is  indicated  in  the  diagrams,  Figs.  3  and  4.  This  area 
is  much  subdivided  according  to  the  evolution  of  function.  Tentatively 
its  most  important  separate  parts  or  centres  might  be  indicated  as  repre^ 
sented  in  the  diagram,  Fig.  3.  The  views  of  Campbell,  based  on  histolog- 
ical research  regarding  the  physiological  subdivisions  of  the  cortex,  are 
given  with  illustrations.  See  Figs.  11  and  12,  pages  73  and  74.  These 
views,  arrived  at  by  a  different  road,  are  similar  to  those  of  Flechsig  and 
the  writer. 


02 


THE   EYE   AXI)  NERVOUS   SYSTEAf 


Concrete  concepts  are  of  various  kinds  according  to  the  degree  of 
their  sinij)lieity  or  eonii)lexity.  One  may  have  a  simple  visual  con- 
cept of  a  leaf  or  flower,  made  up  purely  of  visual  sensations  and 
memories.  This  concept,  however,  becomes  more  complex,  and  at 
the  same  time  more  complete,  when  with  it  are  associated  the  memo- 
rial images  of  the  same  flower  or  plant  which  are  derived  through 
other  senses, — as  those  of  touch  and  smell.  This  higher  concrete  con- 
cept can  only  be  brought  about  by  associations  between  visual  and 
other  sensory  areas.  ^Moreover,  to  the  concrete  concept,  simple  or 
complex,  a  name  comes  to  be  given  through  a  series  of  cerebral  pro- 
cesses which   have  their  structural  substrata   in  the  higher  auditory 


Fig.  4.— Diagrammatic  scheme  of  the  physiological  areas  and  centres  of  the  mesal  aspect  of  the  human 

hemicerebrum. 


sphere;  and  to  connect  these  with  the  visual  areas,  other  associating 
tracts  must  be  present.  The  highest  psychical  area  of  the  brain,  that 
concerned  with  attention  and  volition,  with  abstract  concepts  of  every 
sort,  is  associated  with  the  visual  cortex,  as  it  is  with  all  other  parts  of 
the  great  concrete  memory  field ;  for  it  is  only  by  such  association  that 
reason,  judgment,  and  fancy  can  be  brought  to  bear  in  connection  with 
our  concrete  ideas. 

A  few  words  might  be  said  here  regarding  the  differences  between 
the  lower  and  the  higher  visual  areas  in  the  lower  animals  and  in  man, 
in  order  to  emphasize  the  manner  in  which  the  secondary  or  higher 
visual  centres  develop  as  evolution  proceeds. 


THE  PSYCHOLOGY  OF  VISIO:>^ 


63 


The  lower  or  primary  cortical  visual  area  is  largely  developed  in 
animals  much  below  man  which  use  their  peripheral  and  central  vision 
for  some  purposes  even  in  a  higher  degree  than  man.  They  see  acutely 
for  long  distances  and  have  a  large  peripheral  field.  The  optic  memory 
field  in  animals  below  man  and  in  savage  races  is  highly  developed  as 
regards  memories  concerned  with  the  recognition  of  those  natural  ob- 
jects, places  and  persons,  an  accurate  knowledge  of  which  is  necessary 
for  them  for  defense  and  subsistence.  The  visual  portion  of  the  concrete 
memory  field  in  the  highly  civilized  human  being  is  greatly  enlarged 
and  much  elaborated  in  accordance  with  his  acquirements. 

The  subdivisions  given  of  the  areas  for  concrete  memories  are  of 


Fig.  5.— Scheme  showing  the  extent  and  subdivisions  of  the  secondary  or  higher  visual  cortex  on  the 
lateral  aspect  of  the  cerebral  hemisphere. 

course  provisional,  and  will  doubtless  be  largely  added  to  when  our 
knowledge  of  the  regional  morphology,  physiology  and  anatomy  of  the 
brain  is  increased.  In  Figs.  5  and  6  are  sli0A\ni  the  subdivisions  of  the 
visual  areas  on  both  the  lateral  and  the  mesoventral  aspects  of  the  hemi- 
sphere according  to  the  writer's  latest  views. 

It  will  now  be  necessary  to  speak  of  the  connections  and  associa- 
tions of  the  visual  cortex.  Between  the  calcarine  and  cuneal  cortex  and 
the  pregeniculum  and  pulvinar  of  the  thalamus  runs  a  broad  band  of 
fibres  long  known  as  the  optic  radiations  of  Gratiolet,  while  the  optic 
tracts  connect  the  geniculate  and  thalamic  visual  centres  with  the  retina. 
The  fibres  from  the  pulvinar  run  in  the  dorsal,  those  from  the  pregen- 
iculum in  the  ventral  section  of  these  bundles.     It  is  not  improbable 


G4  THE    EYE    AND    NERVOUS    SYSTEM 

that  in  the  visual  radiations  are  fibres  of  at  least  two  different  origins, 
some  fibres  passing  from  the  basal  centres  to  the  cortex  and  others  from 
the  cortex  to  these  centres. 

We  shall  not  here  go  into  details  of  the  exact  manner  in  which  these 
connections  from  the  retina  and  the  occipital  cortex  are  made,  except  to 
say  that  it  is  after  such  fashion  that  similar  halves  of  the  retina,  of  both 
eyes,  are  related  to  the  occipital  cortex  on  one  side ;  the  right  halves  of 
the  retina  to  the  right  occipital  cortex,  and  the  left  halves  to  the 
left  occipital  cortex ;  while  a  small  central  macular  field  has  a  bundle  of 
its  own  which  also  makes  connection  with  the  cortex  of  the  brain  on  both 
sides.  Anotlier  connection  of  the  retina  through  the  optic  tracts  is  with 
the  anterior  coUiculus  of  tlie  quadrigeminum,  this  having  a  path  to  the 
occipital  lobe  and  others  to  the  nuclei  of  the  ocular  muscles.     The  reader 


Fig.  6. — Scheme  showing  the  position  of  the  primary  visual  centre  on  the  mesal  aspect  of  the  cere- 
bral hemisphere;  and  also  the  probable  jMsitions  of  the  centres  for  color  recognition,  for  elementary 
forma,  and  for  those  ocular  movements  which  are  immediately  responsive  to  visual  impressions. 

is  referred  to  Dr.  Spiller's  article  for  a  fuller  consideration  of  the  visual 
pathway.  The  entire  retino-intracranial  visual  apparatus  is  well  shown 
in  the  schematic  diagram  of  von  Monakow  Avhich  is  partly  based  on  the 
work  of  Ramon  y  Cajal.  The  illustration  is  reproduced  by  permission 
of  Professor  von  Monakow.  T  have  modified  it  so  as  to  introduce  connec- 
tions of  the  primary  optic  centres  with  the  higher  visual  field.  It  is 
ba.sed  upon  von  Monakow's  experimental  and  pathologico-anatomical 
investisrations.' 


*Von  Monakow,  Arch.  f.  Psycli.,  vol.  xx.  3,  and  Gehirnpatholc^ie,  1897. 


THE  PSYCHOLOGY  OF  VISION  65 

With  the  anatomy,  physiology  and  diseases  of  the  optic  radiations, 
the  basal  optic  centres,  the  optic  tracts  and  the  retina,  I  am  not  concerned 


BOfDB  AKD  OOHBS 


MULLEB  8  StJP- 
FOBTINO  CELLS 


EZTERKAIj  IJMITIMO  hkmhii  AMtt 
OUTER    NUCLEAR  LATER 

OUTER  UOLBOULAB    LATER 


OFTIO  NERVE 


fDDBTH    LATER 


OOLLBCnTNO   CEIX6 


HiaHER  VISUAL  OOBSXZ 
FBIUABT    OCCIPITAL  0ORTE< 


CORTIOAL    LATER 

OBEAX  FTBAMIDAL  OEIJ£ 


Fig.  7. — Scheme  by  von  Monakow  of  the  regions  of  the  optic  pathway  (opticus)  in  the  retina,  and 
its  further  termination  iu  the  cortex  of  the  occipital  lobes.  A,  cones ;  B,  rods ;  C,  nuclei  of  the  rods ; 
D,  bipolar  ganglion  cells  for  the  cones :  E,  bipolar  ganglion  cells  for  the  rods ;  F,  large  multipolar 
ganglion  cells  (cells  of  origin  of  the  optic  nerve);  G,  centrifugal  fibres  of  the  optic  tract  and  nerve 
which  originate  in  the  pregeminum  and  end  in  the  retina ;  H,  reticular  receiving  cells  of  the  pre- 
geniculum :  and  I,  the  optic  radiation  cells  of  the  pregeniculum.  The  course  of  the  light  waves 
towards  the  cortex  is  indicated  by  way  of  cones  and  rods ;  bipolar  ganglion  cells  for  the  cones  and 
rods  (optic  cells);  receiving  cells  in  the  pregeniculum,  optic  radiation  cells;  cortical  cells  (third  and 
fifth  layer).  In  order  to  complete  the  cortical  connections  of  the  optic  pathway  an  addition  has 
been  made  to  the  diagram  at  H  V,  which  indicates  the  higher  visual  cortex  in  the  angulo-occipital 
region  which  is  connected  with  the  lower  visual  cortex  by  the  lines  running  in  the  direction  of  the 
arrows.  It  must  be  remembered  in  studying  this  diagram  that  only  the  connections  to  one  side  of 
the  occipital  cortex  are  given.  The  semidecussation  of  the  optic  tracts  is  not  represented.  In  addi- 
tion, it  must  be  recalled  that  tracts  run  from  the  primary  visual  cortex  of  each  occipital  lobe  to  the 
higher  visual  cortex  of  the  opposite  side.  This  also  is  not  represented  in  the  diagram,  which  is 
intended  simply  to  give  a  clear  conception  of  the  connections  of  the  retina  with  the  basal  ganglia  and 
the  primary  visual  cortex  (primary  optic  centres). 

in  the  present  chapter.    It  is  only  necessary  to  recall  here  the  fact  that 
hemianopsia,  of  the  most  complete  type,  has  its  pathological  seat  in  a 
lesion  which  severs  the  optic  radiations  or  optic  tract. 
5 


66  THE    EYE    AND    NERVOUS    SYSTEM 

Si)ecial  sets  of  fibres  associate  the  primary  visual  centres  or  optic 
perception  fields  of  both  liemicerebrums  with  the  higher  cortical  centres 
of  each  side,  as  will  be  further  discussed  when  the  two  clinical  forms  of 
word-blindness  are  considered.  It  is  important  that  this  double  associa- 
tion should  be  borne  in  mind.  This  association  is  indicated  diagrammat- 
ically  in  Fig.  18,  in  which  O  1  and  0  2  represent  respectively  the  left 
and  the  right  occipital  lobe.  According  to  Dejerine,  it  is  brought  about 
by  the  fibres  from  the  cortex  of  the  calcarine  fissure  of  one  side  crossing 
through  the  forceps  major  in  the  splenium  of  the  callosum,  and  joining 
with  the  fibres  from  the  calcarine  cortex  of  the  other  side,  the  united 
bundle  then  passing  to  the  higher  visual  cortex. 

A  matter  of  considerable  interest  is  that  of  the  existence  or  non- 
existence in  the  carrefour  sensitif  of  Charcot,  or  in  juxtaposition  to  it, 
of  a  tract  concerned  with  the  transmission  of  visual  impressions.  Some 
of  the  older  records  of  cases  would  seem  to  bear  out  the  theory  of  the 
existence  of  such  visual  fibres  in  this  position,  but  the  weight  of  opinion 
is  against  this  view.  Most,  if  not  all,  of  the  cases  are  more  readily  ex- 
plained on  the  theory  of  implication  of  the  nearby  optic  radiations  in 
the  lesion  which  impairs  or  destroys  the  posterior  bundle  in  the  posterior 
limb  of  the  internal  capsule. 

Besides  the  tracts  which  associate  the  visual  perception  centres  of 
both  hemispheres  with  the  visual  memory  field  of  each  side,  other  asso- 
ciating tracts  must  be  taken  into  consideration  in  discussing  the  func- 
tions and  focal  diseases  of  the  visual  cortex.  These  associations  are,  in 
fact,  so  numerous  that  the  subject  would  become  befogged,  at  least  for 
the  purposes  of  the  physician,  if  an  attempt  was  made  to  discuss  them  in 
great  detail.  In  the  first  place,  the  various  subdivisions  of  the  higher 
cortical  visual  field  have  more  or  less  intimate  associations  with  each 
other,  in  accordance  with  the  degree  of  association  between  their  func- 
tions. The  centres  in  the  sphere  of  object  seeing  which  are  concerned 
with  the  recognition  of  persons,  places  and  natural  objects  are  associated 
with  the  centres  of  languaige  symbolization.  The  centres  of  the  higher 
visual  area  generally  are  connected  by  associating  tracts  with  similar 
concept  centres  in  other  portions  of  the  concrete  memory  field,  as,  for 
instance,  with  the  higher  centres  of  hearing,  touch,  taste  and  smell. 

HISTOLOGY  OF  THE  VISUAL  CORTEX,  ESPECIALLY 
WITH  REFERENCE  TO  VISUAL  LOCALIZATION. 

As  in  other  parts  of  the  cerebral  cortex,  histologv'  throws  some  light 
upon  localization.  What  is  known  as  the  layer  of  Gennari  occupies  a 
position  which  is  supposed  to  indicate  the  extent  and  limitation  of  the 


HISTOLOGY  OF  THE  VISUAL  CORTEX  67 

primary  visual  cortex  in  man  and  the  lower  animals.  The  layer  of 
Gennari  spreads  over  the  cortex  in  such  a  way  as  to  include  the  lingual 
lobule  from  the  junction  of  the  calcarine  and  parieto-occipital  fissures, 
caudad  to  the  occipital  pole,  including  the  calcarine  fissure,  the  entire 
cuneus,  and  the  first  occipital  convolution.  Fibres  from  the  optic 
radiations  have  been  traced  to  all  the  regions  in  which  this  layer  is 
situated. 

Space  cannot  be  given  to  a  full  consideration  of  the  minute  anatomy 
of  the  visual  cortex  and  its  bearings  upon  the  question  of  the  develop- 
ment and  the  localization  of  cortical  visual  centres. 

Meynert  believed  that  in  the  occipital  lobe  the  classic  arrangement 
of  the  cortex  into  five  layers,  as  especially  seen  in  the  motor  region,  was 
not  followed.  He  recognized  no  less  than  eight  layers.  The  present 
tendency  is,  following  Ramon  y  Cajal,  to  recognize  the  same  number  of 
layers  in  the  visual  as  in  other  portions  of  the  cortex.  Counting  from 
without  inwards,  Cajal  described  five  layers, — namely,  (1)  a  molecular 
layer ;  (2)  a  layer  of  small  fusiform  vertical  cells ;  (3)  a  layer  of  small 
pyramidal  cells ;  (4)  a  layer  of  large  pyramidal  cells ;  and  (5)  a  layer 
of  polymorphous  cells  (Fig.  8).  An  acceptation  of  the  views  of  Cajal 
brings  the  occipital  lobe  in  harmony  with  other  portions  of  the  cortex, 
with  primary  functions  like  hearing,  touch,  smell,  taste  and  the  repre- 
sentation of  movement.  The  studies  of  Cajal  were  made  upon  small 
mammals.  The  same  layers  modified  by  evolution  are,  however,  recog- 
nizable in  man  and  in  the  primates  below  man. 

In  Fig.  9  is  shown  a  crossed  section  through  the  normal  cortex  of 
the  left  calcarine  fissure  in  an  eleven-year-old  epileptic  studied  by 
von  Monakow.  The  right  hemisphere  was  the  seat  of  a  porencephalic 
defect. 

Von  Monakow's  method  of  considering  the  visual  cortex  is  exhibited 
in  this  and  in  other  illustrations  in  his  work.  Regarding  the  subject  in 
a  purely  topographical  sense,  the  following  layers  according  to  him  are 
recognizable  in  the  cortex  of  the  occipital  lobes :  ( 1 )  the  ependymal  and 
fusiform  layer  similar  to  the  layers  found  elsewhere  in  the  convolutions ; 
(2)  the  layer  of  the  small  pyramidal  cells,  although  even  here  there  are 
a  few  examples  of  the  large  pyramidal  cells;  (3)  the  layer  of  giant 
pyramidal  cells  between,  below,  and  above  which  are  found  accumu- 
lations of  nuclei;  (below  the  layer  of  the  small  pyramidal  cells  is 
the  upper  portion  of  the  Vicq  d'Azyr  stripe  and  below  this  a  collec- 
tion of  substantia  gelatinosa — the  lower  part  of  the  Vicq  d'Azyr  stripe 
extends  into  the  fourth  layer)  ;  (4)  the  fourth  layer  is  constructed 
chiefly  of  polymorphous  multipolar  cells  and  contains  many  myelinated 


68 


THE    EYE    AND    NERVOUS    SYSTEM 


fibres ;    it  is  rich  in  ground  substance ;     (5)  this  layer  is  constructed  of 
elements  similar  to  those  of  the  fourth  layer,  but  it  is  indicated  as  a  sep- 


iii    :/ 


IV 


VI 


Fv 


pPy 


Lvi~:2^:;rj 


gPy 


*>»:-S       > 


5. 


Fig.  9. — Croesed  section  through  the  normal 
cortex  of  the  left  calcarine  fissure  in  an  eleven- 
year-old  epileptic  with  porencephalic  defect  in 
the  right  parieto-occipital  lobes,  and  total  primary 
destruction  of  the  optic  radiations  on  the  right 
side,  magnification  300.  The  five  layers  of  the  cor- 
tex are  after  Meynert,— K,  nuclei ;  P,  giant  pyrami- 
dal cells;  m,  subcortical  white  matter,  (von 
Monakow.) 

arate  layer  because  of  the  less  number  of  horizontal  nerve  fibres  which 
it  contain.s. 


Fio.  8.— Scheme  representing  the  layers  of  the 
occipital  cortex  according  to  Cajal ;  I,  II,  III,  IV, 
V,  are  the  five  layers  of  Cajal ;  M,  the  molecular 
layer ;  Fv,  the  layer  of  fusiform  vertical  cellules ; 
pPy,  layer  of  the  small  pyramidal  cells ;  gPy,  layer 
of  large  pyramidal  cells ;  and  Pm,  layer  of  poly- 
morphous cells. 


HISTOLOGY  OF  THE  VISUAL  CORTEX  69 

A  comparative  histological  study  of  the  cortex  of  animals  having 
vision,  from  the  lowest  to  the  highest,  and  a  similar  comparative  study 
of  the  lower  and  higher  visual  cortical  areas  in  man,  bear  out  the  views 
as  to  function  based  on  other  methods  of  investigation,  as  those  of  physi- 
ology and  clinical  j^athology.  Much  work,  extending  over  many  years, 
has  been  done  in  this  field  of  research,  but  the  most  valuable  is  without 
doubt  that  recently  published  by  F.  W.  Mott  and  A.  W.  Campbell.  The 
correlation  of  structure  and  function  in  the  neuro-visual  apparatus  was 
demonstrated  by  Mott,^  as  exhibited  in  changing  cell  lamination  of  the 
visual  cortex  of  mammalia  from  insects  to  the  primates.  The  transition 
from  monocular  to  binocular  and  from  panoramic  to  stereoscopic  vision 
is  discussed,  and  the  relation  of  such  transition  to  full  decussation  and 
semi-decussation  of  the  optic  tracts  is  shown.  The  semi-decussation  is 
correlated  with  "progressive  development  in  the  layer  of  the  higher  asso- 
ciational  pyramidal  cells  lying  above  the  layer  of  the  granules  in  the 
cortex". 

Mott  illustrated  the  various  stages  of  evolution  of  the  visual  cortex 
by  actual  preparations.  He  begins  with  a  discussion  of  the  extremely 
simple  type  of  visual  cortex  shown  by  the  mole  and  the  shrew,  with  their 
limited  visual  powers.  In  these  animals  the  cell  structure  of  the  cortex 
is  of  the  most  rudimentary  character,  consisting  of  small  stellate  or 
quadrilateral  cells  resembling  granules,  below  which  is  a  thin  layer  of 
polymorphic  cells  with  a  few  large  cells.  These  three  types  of  cells, 
rudimentary  in  the  mole  and  the  shrew,  are  found  throughout  the  series 
of  animals  studied  by  Mott.  In  rodents  which  have  wide  panoramic 
vision,  but  unimportant  elaborations  and  associations,  the  visual  cortex 
becomes  considerably  more  complex. 

In  the  rabbit  is  a  higher  elaboration  of  the  visual  cortex  proportion- 
ate to  the  increased  visual  powers.  Here  are  seen  the  visuo-sensory  cells 
of  Raymon  y  Cajal,  a  thin  layer  of  large  stellate  cells,  and  closely  packed 
pyramidal  cells  just  above  the  granules.  L^p  to  this  point  in  Mott's 
series  the  vision  of  the  animal  studied  is  chiefly  panoramic  or  periscopic, 
being  characterized  but  little  by  comprehension  of  depth  and  special 
form.  Mott  traces  his  series  through  the  marsupials,  ungulates,  the 
canines  and  the  felidse,  each  mammalian  form  in  the  advancing  series 


*Mott,  The  Lancet,  vol.  ii.,  December  3,  1904,  p.  1555.  Mott's  results  were 
presented  in  the  Bowman  Lecture,  and  are  recorded  in  an  authorized  and  care- 
fully prepared  abstract  in  the  Lancet,  from  which  the  account  here  given  is 
taken.  I  have  reproduced,  by  permission  of  Dr.  Mott,  the  illustrations  given  in  Fig. 
10,  which  represent  two  histological  types  of  the  visual  cortex, — namely,  in  the 
mule-deer  and  macaeus-monkey. 


70 


THE    EYE    AND    NERVOUS    SYSTEM 


III 


IV 


*,  •,  %  ' 


>' 


A— Mule-Deeb. 


III  b 


IIIc 


■»,'»•••  'J»  ' 
t  *  *?.*  .*  .  ;  *•• 
'.  *        •  . ''      -  *     k  * 

■  »  4  ,     «  .       ♦  • 

.'«   '  .'    '*  ".  V .  ••''. 

,'■"•■. ',:'  •  ••■%  I, 

•  •        ,    '.'it" 


.  .  ,, 


IV 


m 


rv 


B — >U)NKKV. 


-J 


Fig.  10.— Reproduction  of  drawings  after  photoniicroprai>hs  of  sections  of  the  visual  cortex  in  the 
mule-deer,  and  of  the  visuo-sensory  and  viguo-psychic  cortex  in  the  monkey  (w«raci«i).  Magiiitieation 
IfiO.    The  percentage  of  thickness  of  the  different  layers  is  only  approximate. 

A,  Mule-deer,  the  visuo-sensory  area :  I,  20  per  cent.;  II,  20i)er  cent.;  scattered  pyramidal  and  large 
Stellate  cells ;  III,  11  per  cent,  granules ;  IV,  48  per  cent,  solitary  cells  of  Meynert;  polymorphic. 

B,  Monkey,  the  visuo-sensory  area:  1, 10  per  cent.;  II,  27  per  cent,  pyramidal;  III  a,  10  per  cent^ 
outer  layer  of  granules ;  III  b,  10  per  cent,  large  stellate  cells,  line  of  Gennari ;  III  c,  17  per  cent,  inner 
layer  of  granules;  IV,  11  per  cent,  inner  line  of  Baillarger,  with  solitary  cell  of  Meynert;  V,  15  per 
cent,  polymorphic. 

C,  Monkey,  the  visuo-psychic  area :  I,  17  per  cent.;  II,  43  per  cent,  pyramidal ;  large  pyramids ;  III, 
14  per  cent,  granules ;  IV,  10  per  cent,  inner  line  of  Baillarger,  with  solitarj-  cell  of  Meynert;  V,  16  per 
(Sent,  polymorphic.    (Mott.) 


HISTOLOGY  OF  THE  VISUAL  CORTEX  Yl 

showing  greater  extent  and  elaboration  of  the  visual  cortex  in  accordance 
with  the  addition  of  new  powers  of  vision,  binocular  and  stereoscopic 
I^erception  being  added  to  uniocular  and  panoramic  vision.  The  cat  is 
probably  the  first  animal  in  the  series  presented  by  Mott  which  is  capable 
of  convergent  stereoscopic  vision  by  movement  of  the  eyes  independent 
of  the  head.  Below  the  cat  complete  decussation  of  the  optic  tracts  is 
probably  present.  In  this  animal  an  imperfect  semi-decussation,  like 
that  which  is  later  seen  in  its  fuller  development  in  the  primates  and 
man,  takes  place.  The  cat  is  probably  the  first  species  in  the  series  of 
mammals  in  which  section  of  the  optic  tract  produces  hemianopsia.  The 
visual  cortex  is  developed  proportionately  in  each  species  according  to 
its  increased  powers.  In  the  felida^,  to  which  the  cat  belongs,  the  pyra- 
midal area  is  of  increased  depth,  the  solitary  cells  of  Meynert  very 
numerous,  and  the  polymorphic  layer  diminished  as  compared  with 
series  lower  in  the  scale. 

Reaching  the  primates  with  their  perfect  binocular  stereoscopic 
vision,  which  can  *be  added  to  by  movements  of  the  eyes  and  head,  a 
semi-decussation  of  the  optic  tracts,  a  distinct  macula  lutea,  and  a  defi- 
nite occipital  lobe  with  the  line  of  Gennari  visible  to  the  naked  eye,  are 
found.  For  the  first  time  the  visual  cortex  presents  two  structurally 
different  regions,  a  visuo-sensory  and  a  visuo-psychic.  The  visuo-sen- 
sory  cortex  attains  its  maximum  extent  and  complexity  in  the  ape  and  is 
characterized  by  increase  of  width  of  the  line  of  Gennari,  a  double  layer 
of  granules,  and  above  the  outer  of  these  a  double  layer  of  small  and 
medium-sized  pyramids.  The  visuo-psychic  or  associational  cortex  (not 
definite  as  a  distinct  zone  below  the  ape)  is  characterized  by  an  increased 
depth  of  the  pyramidal  layer  owing  to  the  presence  of  a  third  row  of 
large  associational  pyramids,  this  region  being  progressive  in  extent 
upwards  to  man,  in  whom  it  attains  its  maximum  development  at  a 
period  subsequent  to  birth.  The  visuo-sensory  and  visuo-psychic  cortical 
areas  correspond  to  the  primary  and  to  the  higher  cortical  visual  areas 
respectively.  The  results  of  the  researches  of  Campbell,  which  will  next 
be  referred  to,  are  practically  the  same  as  those  reached  by  Mott,  al- 
though the  investigations  were  in  the  main  along  different  lines. 

Campbell  ^  has  pursued  a  series  of  investigations  on  the  medullated 
structure  of  the  nerve  fibres  of  the  cerebral  cortex,  and  also  of  the  cor- 
tical cells,  with  the  result  that  these  investigations  have  ser\^ed  to  show 
that  such  studies  constitute  a  method  of  determining  the  functions  of 


^Campbell,  Proceedings  of  the  Royal  Society,  vol.  Ixxii.  Dec.  3,  190.3;  Journal  of 
Mental   Science,  October,   1904 


72  THE   EYE  AND   NERVOUS   SYSTEM 

different  regions  of  the  cerebral  surface  as  valuable  as  physiological 
investigations  on  the  lower  animals,  embryological  researches  like  those 
of  Flechsig  on  the  fetal  brain,  clinicopathological  records  of  well  ob- 
served cases,  and  morphological  and  anatomical  studies  of  fissural  and 
gyral  development  and  arrangement.  lie  has  shown  that  histological 
studies  of  the  fonn  and  arrangement  of  the  nerve  elements  in  the  normal 
adult,  the  microscopical  change  present  in  pathological  cases,  and  the 
relative  histological  findings  in  anthropoid  apes  and  in  man,  all  tend  to 
one  end — the  demonstration  and  verification  of  the  existence  of  specially 
arranged  separate  functional  areas  and  centres.  Among  the  clinico- 
pathological cases  those  examined  were  instances  of  amyotrophic  lateral 
sclerosis,  of  amputation  of  extremities,  and  of  deprivation  of  the  special 
senses.  The  results  obtained  are  of  the  greatest  interest,  and  in  the  main 
are  confirmative  of  the  views  of  Flechsig,  of  the  morphological  and  ana- 
tomical studies  of  E.  A.  Spitzka  and  the  writer,  and  of  the  clinicopatho- 
logical facts  accunmlated  during  recent  years  by  a  large  number  of  con- 
tributors to  neurology.  ' 

"  Viewed  collectively,"  Campbell  says,  "  the  human  brain  harbors 
two  varieties  of  centres,  controlling  what  we  may  call  '  primary'  and 
^  higher  evolutionary'  functions  respectively ;  the  former  are  those  com- 
mon to  all  animals  and  essetitial  to  survival, — viz.,  centres  for  movement 
and  common  and  special  sensation ;  the  latter  are  those  complex  psychic 
functions  in  the  possession  of  which  man  rises  superior  to  all  other 
beings." 

Among  Campbell's  conclusions  are  (1)  the  motor  region  is  ceph- 
alad  of  the  central  fissure;  (2)  an  area  for  higher  skilled  move- 
ments, which  Campbell  calls  the  "  intermediary  precentral"  area,  just 
in  advance  of  the  motor  area,  corresponds  practically  to  the  so-called 
motor  centres  for  speech  and  writing;  (3)  the  prefrontal  lobe,  rela- 
tively weak  in  nen'e  cells  and  fibres,  is  the  last  pallium  of  the  human 
brain,  its  functions  as  yet  being  uncertain;  (4)  the  post-central  con- 
volution is  the  main  terminus  for  common  sensory  impressions;  (5) 
the  post-central  g^'re  is  probably  a  primary  area,  one  serving  for  the 
recognition  of  the  simplest  components  in  common  sensation,  as  impres- 
sions of  heat  and  pain;  (6)  just  behind  the  post-central  area  is  an 
intermediate  post-sensory  area  concerned  with  the  elaborations  of  sensa- 
tions like  nmscular  sensibility  and  stereognostic  perception. 

With  regard  to  the  visual  cortex,  with  which  I  am  here  particularly 
concerned,  the  views  of  Campbell  are  in  keeping  with  what  has  been 
indicated  as  his  opinions  regarding  other  portions  of  the  cerebral  sur- 
face ;    moreover,  these  views  are  in  accord  with  those  of  Flechsig,  the 


HISTOLOGY  OF  THE  VISUAL  CORTEX 


73 


writer  and  others,  who  have  endeavored  to  show  that  a  large  part  of  the 
parieto-temporo-occipital  cortex  should  be  regarded  as  a  great  concrete 
memory  field,  a  region  higher  than  those  concerned  with  the  mere  recep- 
tion of  sensory  impressions  and  the  projection  of  impulses  concerned 
with  movements.  Campbell  believes,  as  the  result  of  his  studies,  that 
the  visual  area  is  histologically  divisible  into  two  areas.  The  first  of 
these,  according  to  him,  closely  follows  the  calcarine  fissure,  extending 
only  a  short  distance  from  it  on  each  side.  It  is,  he  believes,  intended 
for  the  reception  of  primary  visual  impulses.  To  it  he  gives  the  name 
visuo-sensory  area.  The  second  area  skirts  and  invests  the  first,  and  is 
termed  visuo-psychic,  its  purpose  being  the  further  elaboration  of  visual 


Precentral    Postcentral   Intermediate  postcentral 


Intermediate  precentral 


arietal 


Frontal 


Visuo-psychic 


Prefrontal 


-Common  temporal 
Audito-sensory 


Audito-psychie 

Fig.  H. — Scheme  of  the  lateral  aspect  of  the  left  hemicerebrum  showing  the  position  of  the  primary 
Intermediate  cortical  functional  areas.  The  primary  or  lower  areas  in  the  case  of  the  special  senses  are 
designated,  respectively,  audito-sensory  and  audito-psychie,  and  visuo-sensory  and  visuo-psychic.  The 
visuo-sensory  area  is  present  over  a  very  limited  field  about  the  occipital  pole,  while  the  visuo-psychic 
spreads  over  a  much  larger  area  of  the  lateral  occipital  lobe.    (A.  W.  Campbell.) 

sensory  impressions.  It  is  characterized  by  a  remarkable  wealth  of 
nerve  fibres,  coupled  with  the  presence  of  curious  large  pyramidal  cells. 
The  various  affections  included  under  the  general  designation  of  psychic 
blindness  are  to  be  attributed  to  the  destruction  of  this  visuo-psychic 
area. 

A  study  of  the  diagrams  of  Campbell  (Figs.  11  and  12)  shows  that 
his  visuo-sensory  and  visuo-psychic  areas  correspond  in  a  general  way 
to  the  subdivision  of  the  visual  cortex  as  given  by  me  in  this  article. 
They  differ  somewhat,  however,  as  regards  the  limitations  and  exten- 
sions of  each  of  the  areas.  In  accord  with  Henschen,  he  relates  his 
primary  or  visuo-sensory  area  quite  closely  to  the  calcarine  fissure.     I 


74 


THE    EYE    AND    NERVOUS    SYSTEM 


believe  it  should  be  extended  so  as  to  include  the  whole  of  the  cuneus, 
and  possibly  a  narrow  fringe  of  cortex  on  the  lateral  aspect  of  the  occip- 
ital lobe.  He  restricts  his  visuo-psychic  area  to  the  occipital  convolu- 
tions both  on  the  nieso tentorial  and  lateral  aspects  of  the  hemisphere^ 
while  I  would  extend  it  forward  on  the  latter,  so  as  to  include  the  caudal 
extremities  of  the  parietal  and  temporal  convolutions.  The  principle^ 
however,  is  the  same. 

In  a  general  way  the  histological  investigations  of  Campbell  show 
that  the  posterior  association  area  of  Flechsig,  the  concrete  concept  area 


Postcentral 
Intenne<liate  postcentral       *' 


Precentral 


Limbic 
Variatioas  at  R  and  < 


Parietal 


Intermediate  precentral 


Frontal 


Prefrontal 


VIsuo- psychic 


Visuo-sensory 


Visuo-psychic 


Common  temporal 


Pyriform 


Fia.  12.— Scheme  of  the  mesal  and  mesotentorial  aspect  of  tlie  left  hemicerebnim.  The  visuo-sen- 
sory  area  aa  here  indicated  follows  closely  the  calcarine  fissure,  while  the  visuo-psychic  extends  beyond 
the  calcarine  cortex  in  both  directions.  The  various  cortical  subdivisions  are  Indicated  with  clearness, 
both  graphically  and  descriptively.    (A.  W.  Campbell.) 


of  the  writer,  is  subdivided  into  sub-areas  for  the  representation  of  con- 
crete memories,  those  memories  which  are  derived  primarily  by  way  of 
common  sensibility  and  special  sensation ;  they  are,  in  other  words, 
higher  areas  of  vision,  audition,  common  sensibility,  olfaction  and  gus- 
tation. 

One  point  stands  out  prominently  in  Mott's  investigations, — namely,, 
that  as  vision  advances  increase  in  the  development  of  the  pyramidal 
cells  takes  place.  Both  in  the  race  and  in  the  individual  development 
of  the  pyramidal  cells,  in  number  and  in  size,  increases  step  by  step 
with  the  evolution  of  power.  Mott  refers  to  the  well-known  myelination 
views  of  Flechsig,  especially  with  reference  to  the  optic  radiations  and 
the  development  of  the  different  layers  of  the  visual  cortex.     The  inter- 


CORTICAL  VISUAL  AREAS  75 

mediate  zones  of  Campbell  are  but  other  uames  for  the  association  areas 
of  Flechsig  or  for  the  concrete  concept  or  concrete  memory  field  of  the 
writer.  In  the  visual  cortex  the  intermediate  visuo-psychic  area  is  the 
optic  memory  field  of  Wilbrand  and  Saenger,  the  region  already  dis- 
cussed as  the  seat  of  those  higher  visual  powers  such  as  the  recognition 
of  persons,  places,  objects,  designs,  and  the  symbols  of  language. 

In  his  discussion  of  the  development,  structure,  and  function  of  the 
visual  cortex,  Mott  refers  to  the  morphological  and  anatomical  studies 
on  the  brains  of  individuals  of  low  and  high  development,  and  of  those 
of  different  races,  as  of  the  Soudanese  and  Fellaheen,  also  of  the  Chinese, 
Goanese,  Congo  negro  and  the  Egyptian.  The  work  of  E.  A.  Spitzka 
on  the  brains  of  men  distinguished  for  their  intellectual  powers,  and  that 
of  the  writer  on  the  brains  of  paranoiacs,  imbeciles,  negroes  and  crim- 
inals bear  out  the  views  of  Mott. 

POSITION  AND  THE  SUBDIVISIONS  OF  THE 
PRIMARY  CORTICAL  VISUAL  AREA. 

The  subdivision  of  the  visual  cortex  into  a  primary  or  lower  and  a 
secondary  or  higher  visual  region  having  been  considered,  and  such  sub- 
division having  been  seen  to  be  in  accord  with  the  views  of  Flechsig  and 
the  similar,  although  otherwise  expressed,  views  of  Mott  and  Campbell 
as  to  primordial  and  association  areas,  in  the  further  discussion  of.  our 
subject  it  is  next  necessary  to  glance  at  the  question  of  the  exact  position, 
and  the  extent  and  subdivision  of  the  primary  cortical  visual  area. 
While  all  authorities  are  in  practical  accord  in  placing  the  primary  or 
lower  visual  area,  in  whole  or  in  very  large  part,  on  the  mesotentorial 
surface  of  the  hemicerebrum,  they  differ  considerably  with  regard  to 
the  exact  limitations  and  extensions  of  this  physiological  region.^ 

In  studying  the  literature  of  the  subject  one  is  first  struck  with  the 
great  differences  between  authorities  with  regard  to  the  extent  of  the 
primary  cortical  area.  The  extreme  on  one  side  is  represented  by  the 
view  of  Henschen,^  that  the  entire  optic  perception  field  is  confined  to 


'Dr.  Wm.  G.  Spiller,  in  his  chapter  in  this  work  on  the  Intracranial  Portion 
of  the  Encephalic  Nerves,  Especially  of  Those  Related  to  the  Eye,  in  the  section 
on  Primary  Optic  Centres,  goes  at  some  length  into  the  question  of  their  position, 
and  the  reader  is  referred  to  this  portion  of  his  article  in  connection  with  what 
is  here  said.  It  is  necessary,  however,  to  completeness,  and  in  order  that  the  exact 
views  of  the  writer  with  regard  to  cortical  physiology  of  vision  may  be  understood, 
that  he  should  here  give  some  consideration  to  the  location  and  extent  of  the 
primary  cortical  centres  for  sight. 

'  Henschen,  Klin.  u.  anat.  Beitr.  z.  Pathol,  des  Gehirns,  Upsala,  1890-1903. 


76  THE    EYE    AND    NERVOUS    SYSTEM 

the  calcarine  fissure,  and  the  hypothesis  is  based  on  the  fact  that  in  one 
of  his  own  cases  of  hemianopsia  the  lesion  was  really  or  apparently 
restricted  to  this  fissure ;  and  on  the  other  hand  by  the  view  of  Vialet,* 
that  the  primary  visual  area  of  the  cortex  includes  the  entire  mesoten- 
torial  surface  of  the  occipital  lobe.  Seguin  ®  confined  the  primary  cen- 
tres for  vision  to  the  cuneus.  Not  a  few  of  the  physiologists  and  clinico- 
pathologists  who  have  written  on  the  subject  have  included  not  only  the 
entire  mesotentorial  occipital  cortex,  but  also  a  large  part  of  the  lateral 
occipital  lobe,  and  some  like  Ferrier  have  even  extended  the  region 
into  the  temporal  and  parietal  lobes.  It  must  be  borne  in  mind,  how- 
ever, that  some  of  these  writers  have  not  made  the  distinction  which  is 
drawn  here  and  should  always  be  made  between  the  primary  and  the 
secondary  visual  regions  of  the  cortex.  Not  a  little  confusion  has  arisen, 
especially  in  the  earlier  consideration  of  the  subject,  by  a  non-recogni- 
tion of  this  distinction.  The  only  truly  primary  or  primitive  visual 
function  is  the  recognition  of  luminosity,  or  perhaps  it  would  be  better 
to  say  the  recognition  of  lightness  or  darkness.  Color  recognition, 
and  that  of  fonn,  come  later  in  evolution.  In  a  strict  sense,  therefore, 
the  most  primitive  centres  for  vision  are  those  concerned  only  with  the 
appreciation  of  lightness  and  darkness.  I  believe  that  the  centres  for 
luminosity,  color  and  form  are  distinct  in  the  cerebral  cortex.  The 
colof  centres  and  the  centres  for  form  have  developed  out  of  the  centres 
for  light,  just  as  the  ability  to  recognize  color  and  form  has  developed 
upon  the  basis  of  luminous  recognition.  This  subject  will  be  more  fully 
treated  when  the  cortical  centres  for  color  are  discussed.  It  will  only 
be  necessary  for  clearness  to  state  here,  that  it  is  view  of  the  writer 
that  the  centres  for  color  are  contiguous  to  the  primitive  centres  for 
sight,  and  that  it  is  probable  that  they  are  located  in  the  lingual  and 
fusiform  lobules  below  the  calcarine  fissure,  while  the  centres  for  form, 
beginning  at  the  mesal  edge  of  the  occipital  portion  of  the  hemisphere, 
are  spread  out  in  more  and  more  complex  development  on  the  lateral 
aspect  of  the  hemicerebrum  chiefly  in  the  occipital  convexity.  The  pri- 
mary centre  for  vision  as  here  defined  is,  I  believe,  situated  in  the 
cuneus,  the  calcarine  fissure  and  the  cuneal  slope  of  the  parieto-occipital 
fissure. 

This  primitive  cortical  area  for  sight,  located  in  the  cuneus  and  the 
calcarine  and  parieto-occipital  fissures,  has  in  the  first  place,  two  sub- 
divisions, a  peripheral  and  a  central.  The  central  subdivision  is  for  the 
cortical  representation  of  the  macula  and  the  peripheral  for  the  rest  of 

■  Vialet,  Les  centres  cerebraux  de  la  vision,  etc.,  Paris,  1893. 
•  Seguin,  Jour.  Nerv.  and  Ment.  Dis.,  vol.  xiv.,  1887. 


CORTICAL  VISUAL  AREAS  77 

the  retina.  In  the  affection  known  as  hemianopsia  frequently  the  blot- 
ting out  of  the  visual  field  is  not,  strictly  speaking,  as  the  name  indi- 
cates, for  exactly  half  of  this  field  but  for  one-half  of  it  less  a  small 
semicircular  area  which  is  inserted  notch-like  into  the  hemianopsic  area 
at  its  internal  central  part.  As  Swanzey  ^^  expresses  it  this  small  area 
of  retained  vision  is  bitten  out  of  the  blind  field.  A  patient  may  have 
double  hemianopsia  as  the  result  of  successive  attacks  involving  both 
cerebral  hemispheres  in  the  occipital  region,  and  circumscribed  central 
vision  remain. 

These  and  other  facts  would  seem  clearly  to  point  to  separate  regions 
of  representation  for  the  central  and  for  peripheral  portions  of  the 
retinal  field  respectively.  As  will  more  fully  appear  later,  I  hold  that 
both  in  the  lower  and  in  the  higher  visual  fields,  the  macula  and  the 
peripheral  retina  are  separately  represented,  but  first  let  me  speak  of 
the  probable  position  of  the  separate  centres  for  the  macula  and  periph- 
ery in  the  primary  cortical  visual  area.  For  the  present  I  believe  it 
is  best  to  accept  the  view  of  Henschen  which  has  his  own  well  recorded 
case  in  its  support,  and  a  few  other  facts,  that  the  centre  for  the  macula 
in  the  primary  visual  cortex  is  situated  in  the  more  anterior  portidn  of 
the  calcarine  fissure,  that  is  towards  the  apex  of  the  cuneus. 

Tentatively  it  may  also  be  assumed  that  a  horizontal  zonular  strip 
of  the  retina  is  represented  in  the  posterior  part  of  the  calcarine  fissure. 
These  two  localizations  are  based  upon  cases  of  hemianopsia  with  lesion 
of  the  middle  portion  of  the  calcarine  fissure  and  adjoining  cuneus, 
while  the  frontal  and  posterior  parts  have  escaped. 

Cases  of  cortical  disease,  quadrant  and  sector  hemianopsia,  and  cases 
with  ring-like  or  other  scotomata  in  the  visual  field,  are  on  record.  Such 
cases  are  only  to  be  explained  on  the  supposition  of  a  subdivision  of 
retinal  representation  in  the  visual  half  centres.  Much  difference  of 
opinion  has  arisen  as  to  how  this  subdivision  takes  place,  and  the  cases 
reported  in  proof  of  various  positions  are  somewhat  confusing  and  con- 
tradictory. A  case  of  Henschen,  for  example,  would  seem  to  show  that 
the  upper  part  of  the  retina  is  represented  in  the  sujjerior  lip  of  the  cal- 
carine fissure  and  the  lower  part  in  the  inferior  lip,  and  that  a  horizontal 
strip  between  the  upper  and  lower  parts  of  the  retina  is  represented  at 
the  posterior  extremity  of  this  fissure;  but  the  weight  of  facts  is  cer- 
tainly against  the  view  that  the  entire  half  vision  centre  is  situated  in 
the  depth  of  the  calcarine  fissure.     If  this  were  true,  in  a  case  recorded 

^  Swanzey,  Eye  Disease  and  Eye  Symptoms  in  Their  Relation  to  Organic  Dis- 
eases of  the  Brain  and  Spinal  Cord,  in  System  of  Diseases  of  the  Eye,  edited  by 
Xorris  and  Oliver,  Philadelphia,   1900. 


78  THE    EYE    AXD    NERVOUS    SYSTEM 

by  Beevor  and  Collier,"  left  lieiniauo[)sia  should  have  been  present,  or 
at  least  left  hemianopsia  with  the  exception  of  a  small  area  around  the 
fixation  point.  The  calearine  fissure,  the  inferior  portion  of  the  cuneus 
dorsal  to  it  and  the  lingual  lobule  on  its  ventral  side,  were  all  destroyed, 
yet  quadrantie  hemianopsia  alone,  the  fixation  point  escaping,  was  pres- 
ent. The  only  explanations  whieli  suggest  themselves  for  Ilenschen's 
case  are  that  in  some  persons  retinal  half  representation  is  more  concen- 
trated cortically  than  in  others,  or  as  suggested  by  Beevor  and  Collier, 
the  hemian()j)siH  was  due  to  involvement  of  the  optic  radiations  in  addi- 
tion to  the  calearine  cortex.  The  case  of  Beevor  and  Collier  is  referred 
to  in  some  detail  by  Spiller,  see  page  i),  and  will  again  be  recalled 
when  I  consider  cortical  anopsias  and  hemianopsias.  I  shall  only  say 
here  that  it  seemed  to  demonstrate  that  the  lower  quadrant  of  the  visual 
field  is  re])rcsented  by  the  upper  or  dorsal  portion  of  the  cuneus,  as  in 
their  case  the  patient  presented  blindness  of  the  left  upper  quadrant  of 
the  visual  field,  the  fixation  point  escaping. 

The  case  of  llun  '-  has  long  held  an  important  place  with  regard  to 
this  question  of  the  subdivision  of  retinal  representation.  In  this  case 
the  defect  of  vision  was  in  the  lower  left  quadrant  of  each  eye,  and  the 
lesion  as  recorded  by  Hun  was  atrophy  of  the  lower  half  of  the  right 
cuneus.  Hun  argued  from  this  case  that  the  representation  of  the  lower 
quadrant  of  the  visual  field  was  in  the  lower  half  of  the  cuneus.  I  have 
re-examined  Hun's  original  report  of  this  case,  and  am  inclined  to  agree 
with  Beevor  and  Collier  and  others  in  the  view  that  the  case  does  not 
fully  support  the  contention  that  the  lower  half  of  the  cuneus  is  the 
area  of  representation  for  the  lower  quadrants  of  the  retina  of  the  same 
side.  It  is  notable  in  the  record  of  the  case  that  the  necropsy  was  made 
thirteen  days  after  death  and  after  the  body  had  been  embalmed,  and 
also  that  Hun  reports  that  the  sulK'ortical  white  matter  was  involved  in 
the  lesion  to  the  depth  of  at  least  onothird  of  an  inch.  Probably  as  no 
microscopical  examination  was  made,  and  as  a  brain  lesion  usually  ex- 
tends beyond  the  point  where  it  can  Ihj  traced  by  the  naked  eye,  the 
degree  of  involvement  of  the  subcortex  and  therefore  of  the  optic  radia- 
tions, was  greater  even  than  would  be  understood  by  the  statement  that 
the  destruction  extended  to  the  dejjtli  of  one-third  of  an  inch.  The  case 
therefore  has  less  value  in  the  study  of  the  subdivisions  of  retinal  half 
representation  than  that  of  Beevor  and  Collier.  In  their  case  the  lesion 
was  studied  with  extreme  care  lx)th  macroscopically  and  raicroscopi- 
callv,  and  showed  that  the  cortex  alone  was  involved. 


"  Beevor  and  Collier,  Brain,  part  2.  1904,  p.  153. 

"  Hun.  Amer.  .lour,  of  the  Med.  Sci.,  vol.  xeiii.  .fan-April,  1887,  p.  140. 


CORTICAL  CENTRES  79 

My  conclusions  with  regard  to  the  subdivisions  of  retinal  representa- 
tion in  the  primary  cortical  visual  centres  are :  ( 1 )  that  a  lower  quad- 
rant of  the  field,  or  a  little  less  than  a  quadrant,  is  represented  in  the 
upper  half  or  two-thirds  of  the  cuneus;  (2)  that  the  upper  quadrant 
or  a  little  less  than  a  quadrant,  is  represented  in  the  lower  half  of  the 
cuneus,  including  the  middle  portion  of  the  calcarine  fissure;  (3)  that 
the  macula,  or  at  least  the  fovea,  is  represented  in  the  anterior  ex- 
tremity of  the  calcarine  fissure  and  its  cuneal  border;  and  (-i)  that  a 
zone  or  strip  of  the  peripheral  retina  is  represented  in  the  posterior 
extremity  of  the  calcarine  fissure  and  its  cuneal  border.  ^Vllen  I 
speak  of  a  little  less  than  a  quadrant  being  represented,  it  is  because  the 
strip  supposed  to  be  represented  in  the  posterior  limits  of  the  cal- 
carine fissure  passes  horizontally  from  about  the  line  of  the  fixation 
point  through  the  peripheral  retinal  field.  It  will  be  seen  that  in  com- 
ing to  these  conclusions  I  have  adopted  in  part  the  views  of  Henschen 
and  in  part  those  of  Beevor  and  Collier.  Doubtless  the  differentiation 
of  retinal  representation  in  the  primary  visual  cortex  is  much  greater 
than  is  here  indicated,  but  the  facts  at  command  only  permit  at  present 
this  degTee  of  subdivision. 

FOCAL  DISEASES  OF  THE  VISUAL  CORTEX. 

The  focal  diseases  of  the  visual  cortex  are  those  disorders  of  sight 
and  intelligence  which  result  from  lesion  or  disturbance  of  any  part  of 
the  lower  or  higher  visual  zones.  The  most  important  of  these  affec- 
tions and  those  which  will  be  considered  in  the  present  chapter  are: 
(1)  cortical  anopsias  and  hemianopsias;  (2)  hemiachromatopsia  and 
other  forms  of  color-blindness  of  cortical  origin;  (3)  central  ambly- 
opias crossed  and  uncrossed;  (4)  mind-blindness,  total  or  partial,  and 
transient  or  permanent ;  ( 5 )  word-blindness,  letter-blindness  and  num- 
ber-blindness;  and  (6)  optic  aphasia.  Various  disorders  dependent 
upon  visual  cortical  disease  or  related  to  it,  such  as  alexia,  paralexia, 
dyslexia,  visual  agraphia,  paragraphia,  and  dysgraphia,  will  receive 
consideration,  as  will  also  the  cortical  and  transcortical  mechanism  of 
visual  spectra  and  aurse. 

HEMIANOPSIAS    AND    OTHER    DEFECTS    IN    THE    VISUAL 
FIELDS  DUE  TO  CORTICAL  LESION. 

It  will  not  be  necessary  to  go  at  length  into  a  consideration  of 
the  first  of  the  classes  of  focal  disease  due  to  lesions  of  the  visual  cortex, 
the  hemianopsias  and  the  other  more  restricted  defects.     The  subject 


80  THE    EYE    AND    NERVOUS    SYSTEM 

has  already  necessarily  received  some  cousideration  when  discussing  the 
position,  limitations  and  subdivisions  of  the  primary  cortical  visual 
area,  and  it  has  also  claimed  the  attention  of  Dr.  Spiller  in  his  discus- 
sion of  the  primary  optic  centres.  The  unusually  interesting  cases  of 
Ilun  and  of  Beevor  and  Collier  have  been  given,  and  some  reference 
has  been  made  to  other  cases. 

A  word  or  two  concerning  the  terms  most  commonly  used  in  de- 
scribing the  diseases  of  the  primary  cortical  areas  may  serve  to  keep 
tlie  subject  well  in  hand.  In  the  first  place  it  should  be  remembered 
that  hemianopsia,  of  whatever  type,  may  be  partial  or  complete.  It 
may  be  partial  as  regards  the  amount  of  the  visual  field  obscured  or 
with  reference  to  the  degree  of  this  obscuration.  An  absolutely  total 
hemianopsia  is  one  in  which  the  dividing  line  between  the  blind  and 
the  unimpaired  field  passes  through  the  fixation  point.  In  practice, 
however,  it  is  customary  to  describe  those  cases  as  hemianopsic  in 
which  there  is  a  small  central  area  of  retained  vision,  a  fact  which  has 
been  referred  to  several  times.  In  quadrant  anopsia  only  one-half  of 
the  half-field  is  obscured.  A  few  almost  typical  cases  of  this  form 
of  impairment  of  the  visual  field  have  been  recorded.  In  some  cases, 
however,  a  little  less  than  a  quadrant  of  the  entire  visual  field  is  lost, 
a  ribbon-like  area  of  retained  vision  being  left  at  about  the  position 
of  the  horizontal  meridian.  In  double  hemianopsia,  as  the  result  of 
simultaneous  or  successive  attacks  of  homonymous  lateral  hemianopsia, 
the  entire  field  of  vision  usually,  with  the  exception  of  a  small  central 
area,  is  obscured.  By  a  relative  anopsia  or  hemianopsia  is  meant  a 
partial  impairment  of  the  power  of  visual  recognition  in  a  portion  or 
in  one-half  of  the  visual  field.  This  relative  hemianopsia  may  take 
several  forms.  The  patient  may,  for  example,  in  the  impaired  field 
recognize  the  color,  form,  and  luminosity  of  an  object,  but  not  with 
the  same  clearness  and  intensity  as  in  the  unimpaired  field.  Ilemia- 
chromatopsia  is,  in  a  sense,  a  relative  hemianopsia;  the  patient,  while 
not  recognizing  colors  in  the  affected  field,  is  able  to  recognize  form 
and  light.    (See  Chap,  iv,  p.  107.) 

Everyone  familiar  with  the  gross  and  microscopic  pathological  anat- 
omy of  the  brain  knows  that  it  is  extremely  rare  to  find  disease 
strictly  limited  to  the  cortical  cinerea.  Hemorrhagic  lesions  almost  in- 
variably involve  both  cortex  and  subcortex,  and  often  the  inner  mem- 
branes as  well.  The  same  remark  applies  with  increased  force  to  ab- 
scesses of  the  brain ;  and  tumors  are  almost  never  limited  to  the  cor- 
tex, although  growing,  as  they  frequently  do,  from  the  membranes, 
they  may  for  a  time  simply  erode  or  displace  it,  later  causing  de- 


CORTICAL  CENTRES  81 

striiction  of  both  cortical  and  subcortical  substance.  Softening  due  to 
either  embolic  or  thrombotic  occlusion,  while  it  often  includes  a  large 
cortico-subcortical  area,  may  be  limited  to  either  the  subcortex  or  the 
cortex.  Wliat  closure  of  a  vessel  will  cause  is,  of  course,  conditioned 
by  the  parts  supplied  bj  it.  In  rare  cases  a  cerebral  hemorrhage  in- 
volves so  little  of  the  subcortex  as  to  make  it  practically  a  cortical  lesion. 
From  the  above  statements  it  will  be  seen  that  the  classes  of  cases  which 
throw  most  light  upon  the  exact  functions  of  cortical  visual  areas,  as 
of  other  regions  of  the  brain  surface,  are  tumors  growing  from  the 
membranes  before  they  have  invaded  deeply  the  cerebral  substance,  and 
a  few  cases  of  necrosis  restricted  to  the  cortex.  The  cases  of  Hun  and 
of  Beevor  and  Collier  were  of  the  latter  class. 

Cases  of  purely  cortical  or  of  nearly  purely  cortical  hemianopsias 
or  anopsias  are  extremely  rare.  A  few  have  been  recorded  and  are  to 
be  found  in  the  most  recent  works  on  ophthalmology^  and  neurologj'. 
Wilbrand  and  Saenger  note  the  most  important  of  these.  In  one  case 
left-sided  hemianopsia  was  present,  a  small  portion  of  the  visual  field 
just  beyond  the  fovea,  three  to  six  degrees,  not  being  obscured.  'Ke- 
cropsy  and  microscopical  examination  showed  an  exclusively  cortical 
softening  in  the  depths  of  the  calcarine  fissure.  (Holm.)  In  another 
case  of  complete  right-sided  homonymous  hemianopsia,  the  necropsy 
revealed  calcarine  softening.  (Hilden.)  Another  case  of  permanent 
hemianopsia  with  insular  formed  scotomata  in  the  neighborhood  of 
the  horizontal  meridian  in  which  a  cortical  focus  of  softening  was 
found  in  the  floor  of  the  calcarine  fissure  has  been  recorded.  (Eggers.) 
In  addition  to  these  cases  of  what  appears  to  have  been  strictly  cortical 
lesion,  a  few  others  have  been  reported  in  which  the  disease  extended 
very  slightly  into  the  subcortex. 

It  sometimes  happens  that  a  lesion  of  the  cortex  of  the  lateral 
occipital  lobe  apparently  causes  a  true  hemianopsia,  but  in  these  cases 
the  local  pathological  etiology-  is  only  apparent.  A  closer  examination 
always  shows  that  the  lesion  involves  the  optic  radiations  somewhere 
in  their  course  from  the  pregeniculum  to  the  calcarine  fissure  and 
cuneus.  This  is  the  explanation  of  a  number  of  cases  of  hemianopsia 
which  have  been  recorded  as  occurring  in  connection  with  lesions  of 
the  angular  gyre  and  other  parts  of  the  lateral  temporo-occipital  region. 
Von  Monakow  gives  an  illustration  of  a  case  of  this  kind,  the  surface 
appearance  being  shown  in  Fig:  13.  In  this  case,  by  making  frontal 
sections  through  and  cephalad  of  the  lesion  so  as  to  expose  the  cortex, 
corona  radiata  and  other  related  parts,  it  was  found  that  the  original 
lesion  involved  to  some  extent  the  optic  radiations,  in  which  also  sec- 
6 


82 


THE    EYE    A:N^D    NERVOUS    SYSTEM 


ondary  degeneration  had  occurred.     Von  Monakow  gives  illustration8 
of  the  hemianopsic  fields. 

Double  hemianopsia  is  of  comparatively  infrequent  occurrence,  but 
a  sufficient  number  of  cases  have  been  recorded,  both  with  and  without 
necropsy,  to  shed  some  light  upon  its  pathology  in  different  cases.  In 
most  of  the  instances  of  this  affection  with  necropsy  both  cortex  and 
subcortex  have  been  implicated  in  the  lesion.  It  is  indeed  the  rule 
in  any  case  of  hemianopsia  when  the  cortex  is  involved  for  the  sub- 
cortex also  to  take  part  in  the  disease,  but  the  reverse  is  not  so  com- 
mon. In  other  words,  lesions  of  the  optic  radiations,  the  cortex  remain- 
ing free,    are   relatively   frequent   and  of  course,   in   those   cases   in 


Fig.  13.— Lateral  aspect  of  the  left  cerebral  hemisphere  in  a  case  of  apparent  cortical  hemianopeia. 
U,  lesion  in  the  interparietal  fissure,  and  also  of  the  superior  parietal  convolution  (P,  i),  which  is  in 
large  part  destroyed.  The  line  1-1  represents  a  transsection  made  through  the  hemisphere  and  the 
posterior  portion  of  the  lesion.  The  line  2-2  indicates  the  position  at  which  a  transsection  was  made  a 
short  distance  in  front  of  the  lesion.  It  is  so  placed  as  to  about  iiass  through  the  angular  gyre.  These 
sections  were  made  in  order  to  study  the  relation  of  the  lesion  to  the  cortex  and  subcortex.  The  optic 
radiations  were  involved  in  the  lesion  in  which  also  secondary  degeneration  had  occurred.  (Von 
Monakow. ) 


which  the  hemianoi)sia  is  due  to  lesion  of  the  optic  nerves,  tracts,  or 
pregenicula^,  the  question  of  cortical  implication  does  not  arise. 

In  discussing  double  hemianopsias  the  fact  already  alluded  to 
must  be  emphasized,  that  in  lateral  homonymous  hemianopsia  the  loss 
sometimes  extends  over  the  entire  visual  half-field  to  a  dividing  line 
which  passes  through  the  fixation  point,  while  in  other  cases  a  small 
central  area  of  retained  vision  near  the  fixation  point  is  left.  In  double 
hemianopsia  two  similar  sets  of  cases  are  likewise  observed;  both 
halves  of  the  field  may  be  blotted  out  to  the  dividing  line,  causing,  of 
course,  complete  blindness,  or  a  small  central  area  on  each  side  of  the 
fixation  point  may  be  left,  leaving  a  form  of  tubal  or  barrel  vision.     In 


CORTICAL  CENTRES  83 

most  cases  of  double  hemianopsia  one-half  of  the  visual  field  is  ob- 
scured, and  later  the  other.  In  other  words,  a  lateral  homonymous 
hemianopsia  becomes,  as  the  result  of  successive  attacks,  converted  into 
a  double  hemianopsia,  either  complete  or  with  a  small  area  of  vision 
representing  the  fovea  and  its  immediate  environment.  As .  already 
intimated,  the  cases  of  hemianopsia,  single  or  double,  with  a  retained 
area  of  central  vision,  are  instructive  in  connection  with  the  question 
of  the  existence  of  a  separate  macular  centre  in  the  lower  cortical 
visual  zone.  The  cases  can  scarcely  be  explained  except  on  this  sup- 
position. 

With  regard  to  double  hemianopsia  of  cortical  origin,  with  which 
I  am  particularly  concerned,  cases  with  lesions  strictly  limited  to  the 
cortex  can  scarcely  be  found  in  literature,  although  in  some  of  the 
few  cases  recorded  the  lesions  were  largely  cortical.  The  case  recorded 
by  Dejerine  and  Vialet  ^^  had  several  points  of  particular  interest.  The 
double  hemianopsia,  or  rather  the  complete  loss  of  vision,  took  place  at 
once  and  without  any  recognizable  apoplectiform  attack.  Peripheral 
and  basal  disease  were  eliminated  and  the  diagnosis  of  cortical  blindness 
was  made.  The  patient  died  after  many  years  and  necropsy  showed 
bilateral  lesions.  On  the  right  the  lesion  involved  the  cuneus  and  the 
lingual  and  fusiform  lobules;  in  fact,  the  entire  inferior  temporo- 
occipital  lobe ;  on  the  left  the  cuneus  escaped,  but  the  lingual  lobule 
and  other  portions  of  the  temporo-occipital  lobe  corresponding  to  the 
seat  of  disease  on  the  right  were  included  in  the  lesion.  It  is  probable 
that  the  calcarine  fissure  was  implicated.  In  all  cases  of  double 
hemianopsia  in  which  cortical  lesions  have  been  present,  these  have 
been  situated  on  the  mesoventral  surface  of  the  occipital  lobe,  and,  as 
far  as  I  know,  the  calcarine  fissure  has  not  escaped  in  any.  The  cases 
recorded  with  necropsy  besides  that  of  Dejerine  and  Vialet,  just  re- 
ferred to,  are  those  of  Schmidt-Rimpler  ^ ^  and  of  Bouveret.^^ 

I  was  consulted  with  regard  to  the  interesting  case  which  has  been 
recorded  by  Dunn  ^^  and  Avhich  is  now  part  of  the  literature  of  the' 
subject  of  double  hemianopsia.  In  this  case  the  patient  had  loss  of 
power  of  locating  or  orientating  himself.  He  could  not  form  a  con- 
ception of  the  geography  of  his  house  nor  of  any  other  place  to  which 
he  had  ever  been.  He  could  remember  a  place  by  its  name,  but  could 
not  call  it  up  and  relate  it  with  its  environment.     He  retained,  how- 


"  Dejerine  and  Vialet,  Centralblatt  fiir  Augenheilkunde,  February,  1894,  S.  64. 
"  Schmidt-Rimpler,  Archiv  fiir  Ophthalmologie,  November,  1887. 
"Bouveret,  Revue  generale  d'Ophtalniologie,  November,  1887. 
"Dunn,  University  Medical  Magazine,  Philadelphia,  May,  1895. 


84  THE    EYE    AND    XERVOUS    SYSTEM 

ever,  the  ability  to  recall  and  to  describe  individuals,  A  small  central 
area  of  retained  vision  remained.  From  a  study  of  his  case,  Dunn 
suggested  the  existence  in  the  brain  of  a  geographical  centre,  where 
are  recorded  the  optical  images  of  locality.  It  will  be  noted  that  in  one 
of  my  schemes  of  cerebral  zones  and  centres  I  have  placed^  a  centre  for 
orientation  along  the  anterior  border  of  the  inferior  portion  of  the  visual 
field. 

Cases  like  that  of  Dunn  and  a  few  others  which  have  been  reported 
appear  to  indicate  that,  in  order  that  loss  of  orientation  shall  occur, 
the  lesions  must  be  bilateral,  that  both  the  right  and  the  left  occipital 
or  temporo-occipital  regions  must  be  the  seat  of  destructive  lesions. 
This  is  not,  however,  strictly  correct.  Cases  are  on  record  in  which  a 
destructive  lesion  of  one  hemicerebrum  caused  loss  or  impairment  of 
orientating  power.  Such  a  case  has  been  put  on  record  by  Peters.^^ 
The  lesion  was  right-sided,  the  patient  having  left  lateral  homonymous 
hemianopsia.  A  lesion  situated  on  either  the  right  or  the  left  side  is 
apparently  competent  to  cause  impairment  or  loss  of  the  power  of 
orientation,  as  in  another  case,  that  of  Groenouw,^^  the  hemianopsia 
was  for  the  right  fields.  It  would  seem  to  be  much  the  same  with  dis- 
order of  orientation  as  with  mind-blindness,  of  which,  perhaps,  it  is 
an  expression, — namely,  that  in  the  most  usual  case  and  most  decided 
form  it  is  the  result  of  bilateral  lesions,  but  may  occur  in  special  in- 
stances, either  transiently  or  permanently,  from  a  lesion  of  one  hemi- 
sphere. 

Relative  cortical  hemianopsia  is  a  comparatively  rare  affection.  If 
the  theory  of  Wilbrand  were  true  that  light  and  color  are  represented 
in  superimposed  strata,  some  forms  of  it  would  be  explicable  on  the 
view  that  the  most  superficial  layers  of  the  cortex  were  destroyed. 
Such  destruction  might,  for  instance,  cause  a  hemiachromatopsia,  leav- 
ing light  perception  unaffected,  but  it  is  fanciful,  if  not  absurd,  to 
reason  that  any  such  neatly  demarcated  destruction  of  superimposed 
cortical  layers  takes  place  as  the  result  of  disease,  and,  so  far  as  I 
know,  such  a  case  has  never  been  reported.  The  manner  of  blood 
supply  to  the  cortex  and  subcortex  would  also  make  such  a  lesion  un- 
likely. On  the  theoretical  grounds  advanced  by  Wilbrand,  that  form 
of  relative  hemianopsia  which  is  known  as  hemiachromatopsia  might 
be  explained,  but  the  explanation  is  not  tenable.  In  the  next  section 
the  probable  lesions  causing  hemiachromatopsia  will  be  considered. 

"  Peters,  Archiv  fiir  Augenheilkunde,  xxxii.  S.  175. 

"  Groenouw,  Archiv  fUr  Psychiatrie  und  Xervenkrankheiten,  xxiii.  339. 


COETICAL  CENTRES  85 

Relative  hemianopsia  sometimes  takes  the  form  of  a  diminution 
of  the  power  of  recognizing  light,  form,  and  color  in  the  affected  field, 
as  in  a  recent  case  studied  by  me  in  which  the  patient  had  the  general 
symptoms  of  brain  tumor,  such  as  headache,  -double  optic  neuritis,  and 
vertigo,  with  other  focal  symptoms,  besides  lateral  relative  hemianop- 
sia pointing  to  the  left  occipital  lobe  and  especially  its  mesoventral 
surface.  In  this  case  the  relative  hemianopsia  eventually  became  com- 
plete. A  relative  quadrantic  anopsia  may  be  present,  and  what  is  still 
more  interesting,  the  case  may  show  in  one  part  of  the  obscured  field 
complete  loss  of  the  powers  of  recognizing  light,  form,  and  color,  while 
in  another  the  loss  may  be  only  for  color  or  form.  Of  course,  if  the 
power  of  recognizing  lightness  and  darkness  is  lost,  color  and  form 
recognition  go  with  it,  but  form  or  color  appreciation,  or  both  of  these, 
may  be  abolished  and  light  and  dark  recognition  remain.  It  may  be 
that  eventually  some  clearer  insight  into  the  exact  position  of  separate 
centres  for  light,  form,  and  color  will  be  obtained  by  a  study  of  cases 
in  which  there  is  loss  of  the  three  senses  in  one  part  of  the  field,  and 
of  only  one  or  two  in  the  other.  Some  curious  forms  of  relative 
achromatopsia  will  be  referred  to,  in  the  section  on  the  color  sense  and 
cortical  color  centres. 

In  the  differentiation  of  cortical  anopsias  and  hemianopsias,  as 
well  as  hemianopsias  due  to  lesions  of  the  optic  radiations  of  Gratiolet, 
the  importance  of  the  Wernicke  symptom,  inaction  of  the  pupils  on 
stimulation  of  the  blind  half  of  the  retina  must  be  remembered.  It 
is  only  necessary  to  recall  that  in  cortical,  as  in  optic  radiation  cases, 
the  pupillary  response  remains  for  the  entire  retina,  while  in  cases 
of  disease  of  the  optic  nerves  and  tracts  and  of  those  portions  of  the 
basal  ganglia  which  are  concerned  with  vision,  the  pupillary  inaction 
can  be  demonstrated.  Some  apparently  conflicting  cases  have  been  re- 
corded, but  these  are  probably  due  to  imperfect  observation.  It  is 
well  known  to  experienced  ophthalmologists  and  neurologists  that  it 
is  one  of  the  most  difficult  of  undertakings  to  demonstrate  with  cer- 
tainty Wernicke's  hemianopic  pupillary  inaction,  but  nevertheless  it 
can  be  done  if  sufficient  care  is  taken.  In  two  cases  in  which  Ferrier 
accidentally  cut  the  optic  tract  in  the  course  of  his  experiments  on 
monkeys,  the  pupil  failed  entirely  to  respond  to  a  pencil  of  electric 
light  carefully  focused  upon  the  blind  half  of  the  retina,  while  in  a 
number  of  cases  of  cerebral  extirpation  experiments  the  pupil  never 
failed  to  respond. 

The  Wernicke  inaction  symptom,  however,  only  serves  to  place  the 
lesion  in  the  cortex  or  the  optic  radiations.     In  determining  whether 


86  THE    EYE    AND    NERVOUS    SYSTEM 

the  lesion  is  iu  the  optic  radiations  or  in  the  calcarine  cortex  or  cuneus, 
the  diagnosis  must  be  reached  by  a  study  of  the  associated  symptoms 
and  of  some  special  features  of  the  hemianopsia.  Hemianopsia  due  to 
lesion  of  the  optic  radiations  is  only  in  extremely  rare  cases  an  isolated 
symptom.  Its  associated  manifestations  may  be  few  or  comparatively 
many.  It  may,  for  instance,  be  part  of  a  symptom  complex  which  in- 
cludes word-blindness,  mind-blindness,  and  word-deafness,  with  other 
symptoms  so  often  combined  with  these  foi-ms  of  sensory  aphasia,  such 
as  alexia,  dyslexia,  and  paraphasia.  If  the  lesion  is  deeply  placed  so 
as  to  implicate  the  posterior  portions  of  the  posterior  half  of  the  in- 
ternal capsule,  hemianesthesia  may  be  present,  as  may  also  impair- 
ment or  loss  of  the  muscular  sense  and  stereognostic  perception.  When 
hemiplegia  or  hemiparesis  occurs  with  hemianopsia,  which  can  be  re- 
ferred to  the  optic  radiations,  the  lesion  is  usually  an  extensive  one, 
although  so-called  distant  symptoms  may  be  exhibited. 

The  foregoing  are  some  of  the  associations  by  means  of  which  one 
is  enabled,  with  some  approach  to  certainty,  to  conclude  that  the  lesion 
causing  the  hemianopsia  is  subcortical,  and  probably  situated  beneath 
and  in  particular  relation  with  special  portions  of  the  lateral  aspects 
of  the  postparietal  and  temporo-occipital  regions.  Hemianopsias,  cor- 
tical, or  largely  so,  such  as  were  illustrated  by  the  cases  of  Henschen, 
Ilolm,  Ilun,  Beevor  and  Collier,  are  usually  distinguished  by  the 
absence  of  such  symptoms  as  hemianaesthesia  and  the  various  symp- 
toms classed  under  the  general  head  of  visual  and  auditory  aphasias, 
but  if  both  cortex  and  subcortex  are  involved,  the  associated  clinical 
phenomena  will  depend  upon  the  depth  and  extent  of  the  lesion.  Quad- 
rant anopsia  suggests  a  cortical  origin,  although  not  necessarily,  as  it  is 
altogether  probable  that  dorsal,  ventral,  and  medial  portions  of  the 
retinal  half-field  are  represented  by  separate  bundles  in  the  optic  radia- 
tions, as  they  are  by  subdivision  of  the  cortex  in  the  cuneus  and  cal- 
carine fissure. 

Dufour'^  has  suggested  a  special  means  of  separating  cortical 
hemianopsia  from  the  same  symptom  dependent  upon  disease  of  the 
optic  radiations.  If  the  case  is  purely  cortical,  he  holds  that  the  pa- 
tient will  suffer  from  "  vision  nulle,"  that  is,  from  the  absence  or 
negation  of  vision.  The  patient  is  not  conscious  that  he  cannot  see 
in  the  blind  field  until  his  attention  is  called  to  the  fact  by  perimetric 
or  other  investigation,  or  by  some  accidental  circumstance.  If  the 
hemianopsia  is  due  to  lesion  of  the  optic  radiations,  on  the  other  hand, 

"Dufour,  Revue  m^dicale  de  la  Suisse  Romande,  August  20,  1889. 


CORTICAL  CENTRES  87 

he  has  a  subjective  sense  of  darkness.  It  is  doubtful  whether  this  is  a 
reliable  test  of  cortical  anopsia  and  hemianopsia,  as  a  few  cases  have 
been  recorded  which  seem  to  be  in  contradiction  to  Dufour's  views 
with  regard  to  negative  vision;  in  other  words,  in  cases  in  which  the 
necropsy  demonstrated  the  existence  of  a  lesion  of  the  optic  radiations, 
Dufour's  negative  vision  was  observed. 

Before  taking  up  the  subject  of  cortical  color  representation  and 
chromatic  disorders  due  to  disturbance  or  disease  of  color  centres,  I 
shall  here  make  a  few  suggestions  as  to  terminology  which  seem  to 
me  to  have  some  practical  value.  Hitherto  in  the  discussion  of  the  dis- 
eases of  the  cortical  half-vision  centres  I  have  made  use  of  the  term 
hemianopsia  or  anopsia.  It  might  be  better,  recognizing  that  the  cor- 
tical representation  of  light,  form,  and  color  is  separate,  to  give  dis- 
tinct names  to  the  three  forms  of  hemianopsia, — the  visual  half  loss 
for  light,  for  form,  and  for  color.  The  terms  achromatopsia  and  hemia- 
chromatopsia  are  well-grounded  in  medical  literature.  To  express  the 
loss  of  the  light  sense  or  the  sense  of  whiteness  and  blackness,  I  would 
suggest  the  word  aleucopsia  from  the  Greek,  a,  priv. ;  ^ewxo?,  white, 
and  hemialeucopsia  for  the  loss  of  this  sense  in  the  visual  half  fields; 
for  the  loss  of  the  sense  of  form  the  term  amorphopsia,  from  the  Greek 
«,  priv.;  fiopfTJ ,  form,  and  for  this  loss  in  the  corresponding  half 
fields,  hemiamorphopsia.  These  terms  may  be  found  convenient  in 
description.  It  is  true  that  when  the  loss  of  the  light  sense  is  complete 
that  of  form  and  that  of  color  necessarily  go  with  it,  but  as  was  seen 
when  discussing  relative  hemianopsias,  we  may  have  incomplete  forms 
of  hemialeucopsia. 

Hemialeucopsia  has  received  the  consideration  required  in  this 
chapter  in  the  discussion  of  hemianopsia  in  its  cerebral  and  especially 
its  cortical  relations,  and  the  achromatopsias  will  be  presently  con- 
sidered, A  few  words  might  be  said  rather  in  the  way  of  suggestion 
than  of  detailed  consideration  about  the  study  of  amorphopsia,  espe- 
cially with  reference  to  its  bearings  upon  questions  of  cerebral  localiza- 
tion. Usually  the  methods  of  examining  the  fields  for  form  are  of  the 
simplest  character,  although  sufficient  for  the  ordinary  purposes  of  the 
ophthalmologist.  The  reader  unacquainted  with  these  methods  is  re- 
ferred elsewhere  and  can  find  them  in  the  works  on  ophthalmology. 
An  adaptation  of  the  eye  to  different  degrees  of  illumination  is  re- 
quired for  a  better  study  of  the^  fields,  and  particular  methods  of  get- 
ting the  best  results  have  been  described  by  some  writers. 

I  have  already  expressed  the  view  that  the  only  primitive  cortical 
•centre  of  vision  is  that  for  light  or  for  the  recognition  of  whiteness 


88  THE    EYE    AND    XEllVOUS    SYSTEM 

and  blackness,  and  that  color  recognition  develops  ventrally  in  the 
expansion  of  the  cerebral  surface,  the  recognition  of  form  probably 
developing  dorsally.  In  the  diagrams,  Figs.  5  and  6,  the  most  ele- 
mentary centres  for  form  are  represented  as  probably  situated  along 
the  edge  of  the  heinis])here,  both  on  its  mesal  and  lateral  aspect;  in 
other  words,  in  the  most  dorsal  part  of  the  cuneus  and  in  the  first 
occipital  convolution.  Presumably  elementary  forms  are  represented 
cortically  in  closest  relation  with  the  primitive  centre  for  light,  the 
representations  for  more  and  more  complex  forms  receding  with  cor- 
tical development  from  the  primitive  area  of  representation. 

Little  is  known  about  form  fields  through  studies  of  their  extent  by 
means  of  objects  of  different  shaj>e  and  character,  and  it  is  possible  that 
something  of  value  with  regard  to  cortical  localization  might  be  learned 
by  perimetric  observations  of  the  fields  for  form  as  determined  with 
articles  of  particular  shape  or  design,  instead  of  confining  these  in- 
vestigations to  the  use  of  a  limited  number  of  geometric  figures  or  to 
letters  in  one  or  two  languages.  These  studies  might  prove  of  par- 
ticular value  in  cases  of  })artial  mind-blindness  or  of  word-  or  letter- 
blindness  \vith  large  retention  of  vision  for  other  purposes. 

CEREBRAL  CHROMATIC  PHENOMENA  AND  THE  COR- 
TICAL CENTRES  FOR  COLOR  RECOGNITION. 

Color-blindness,  or  achromatopsia,  is  the  inability  to  recognize  col- 
ors. It  may  be  total,  although  this  form  of  achromatopsia  is  rare,  and 
even  when  it  is  present  the  individual  may  distinguish  as  differences 
in  lightness  or  darkness  what  others  recognize  as  differences  in  color. 
Most  frequently  the  color-blindness  is  partial,  and  this  partial  color- 
blindness may  be  of  several  forms.  Hemiachromatopsia  is  the  loss 
of  the  ability  to  recognize  colors  in  one  half-field.  Central  achroma- 
topsia is  an  affection  in  which  the  half  visual  fields  retain  the  power 
of  color  recognition,  while  this  is  lost  in  the  central  or  macular  field. 
Dyschromatopsia  is  difficulty  in  distinguishing  colors,  and  parachro- 
matopsia  is  false  or  incorrect  perception  of  colors.  Partial  color- 
blindness may  be  regarded  from  another  point  of  view,  as  to  the  color 
or  colors  which  are  lost  or  disturbed ;  this  is  illustrated  by  such  affec- 
tions as  red-blindness,  green-blindness,  violet-blindness,  or  red-green 
blindness.  Color  amnesia,  or  color  aphasia,  sometimes  incorrectly 
spoken  of  as  amnesic  color-blindness,  is  an  affection  in  which  the 
patient,  while  able  to  recognize  colors  by  the  different  tests,  is  unable 
to  name  the  colors  recognized. 

For  the  sake  of  a  clear  understanding  of  cerebral  color  affections. 


CORTICAL  CE:NtTRES  89 

it  may  be  worth  while  to  call  attention  here  to  a  few  well-known  facts 
regarding  chromatic  phenomena.  The  sensations  of  color  result  from 
dissolving  white  light  into  its  spectral  colors.  The  so-called  primary 
colors  of  the  spectrum,  as  green,  red,  yellow,  and  blue,  each  includes 
somewhat  differing  sensations  of  color.  These  correspond  roughly  to 
what  are  known  as  shades  of  color.  The  power  of  recognizing  these 
differences  varies  greatly  in  different  individuals,  and  even  in  the  same 
individual  at  different  stages  in  the  course  of  his  education  or  train- 
ing. The  mixing  of  certain  colors  together  and  the  mixing  of  certain 
colors  with  Avhite  light  produces  new  sensations  of  color.  A  word  or 
two  might  be  said  about  a  few  of  the  terms  frequently  used  in  the 
discussion  of  color  phenomena.  Hue  depends  on  the  wave  lengths 
of  the  rays;  the  intensity  on  the  amount  of  light  falling  on  a  body 
in  the  given  time,  and  the  saturation  on  the  amount  of  white  light  fall- 
ing on  the  area  in  the  same  time.  Mixing  of  the  colors  of  the  spectrum 
produces  white  light,  or  the  same  can  result  from  the  mixture  of  com- 
plementary colors,  although  in  this  case  the  light  will  differ  in  intensity 
from  the  white  light  which  is  the  effect  of  mixing  all  the  colors  of  the 
spectrum.  % 

By  hue  we  mean  color  in  the  ordinary  sense  in  which  the  word  is 
used;  it  is  hue  which  gives  red,  green,  blue,  etc.  The  hue,  however, 
is  not  always  simple  and  therefore  cannot  always  be  defined  by  making 
it  synonymous  with  wave  lengths.  It  may  be  the  result  of  an  admix- 
ture with  various  spectral  colors,  or  of  white  with  some  colors,  and  then 
it  can  only  be  expressed  by  averaging  or  approximating  the  wave 
lengths  of  the  spectral  colors.  Sometimes  the  three  constants  of  color 
are  defined  as  hue,  purity  or  tint  and  brightness.  Purity  or  tint  does 
not  depend  upon  the  wave  length,  but  upon  the  amount  of  white  light 
that  enters  into  the  colors;  the  less  there  is  of  white  light  the  purer 
is  the  color. 

Brightness  depends  upon  the  energy  of  the  ethereal  movement  and 
the  sensitiveness  of  the  retina  for  the  particular  color.  Explained  in 
another  way,  the  brightness  is  in  inverse  proportion  to  the  amount  of 
blackness  mixed  with  the  color.  In  the  present  connection  we  are 
only  concerned  with  these  general  phenomena  of  color  in  so  far  as 
they  have  some  bearing  upon  the  question  of  cerebral  activity  with 
regard  to  color  sensations.  Hue,  intensity,  tint,  and  brightness  as  sub- 
jective phenomena  all  have  their  cerebral  correlatives,  but  the  only 
constant  material  substratum  is  for  hue  as  expressed  in  fundamental 
colors. 

It  has  been  discovered  that  in  the  retina  exists  one  and  perhaps 


90  THE    EYE    AND    NERVOUS    SYSTEM 

several  photo-chemical  color  substances.  The  best  known  of  these  is 
the  visual  purple.  The  existence  of  this  substance  has  been  demon- 
strated in  various  ways,  as  by  removing  the  eyes  of  a  frog  after  having 
kept  the  animal  some  time  in  the  dark,  when  the  retina  is  found  to  be 
of  a  red  or  purplish  color,  which  soon  fades,  leaving  it  an  opaque  white. 
Investigation  has  shown  that  this  visual  purple  is  confined  to  the  rods 
and  even  to  their  outer  limbs.  It  is  absent  from  the  fovea  centralis. 
It  is  not  present  in  the  eyes  of  some  animals,  as,  for  instance,  in  the 
eyes  of  snakes,  whose  retinae  are  composed  almost  entirely  of  cones. 
It  is  believed,  but  not  fully  demonstrated,  as  in  the  case  of  visual  pur- 
ple, that  in  the  retina  are  present  visual  substances  for  each  of  the 
fundamental  colors. 

As  is  well  known,  views  differ  as  to  which  of  the  colors  of  the 
spectrum  are  fundamental.  Helmholtz  believed  that  they  were  three 
in  number, — namely,  red,  green,  and  violet;  while,  according  to 
Bonders,  red,  yellow,  green,  and  blue  were  primary  or  simple  colors. 
Ilering  regarded  as  distinct  the  recognition  of  white,  black,  red,  yellow, 
green,  and  blue.  Other  views  as  to  the  fundamental  colors  have  been 
held,  but  these  are  usually  represented  as  three  or  four  in  number. 
The  hypothesis  of  three  fundamental  colors  was  first  put  forward  by 
Young.  Foster  and  many  physiologists  accept  the  trichromatic  view; 
all  agree  that  while  there  may  be  more,  it  is  necessary  to  recognize  at 
least  three  primary  colors.  Each  of  these  probably  has  its  own  visual 
substance,  so  that  in  the  retina,  besides  the  visual  purple,  there  is 
probably  a  visual  red,  visual  green,  visual  blue,  and  possibly  a  visual 
yellow  substance. 

The  theory  is  that  the  decomposition  of  each  visual  substance  is 
accompanied  by  molecular  vibrations  which  are  taken  up  by  the  retinal 
structures  and  transmitted  through  the  various  layers  of  the  retina 
to  the  optic  nerve,  and  thence  by  the  optic  tract,  basal  centres  and 
optic  radiations  to  the  visual  cortex.  The  colors  are  sifted  at  the 
periphery  of  the  visual  apparatus  and  each  sifted  color  has  its  place 
of  representation  in  the  cerebral  cortex. 

Young  held  that  the  three  primary  color  sensations  were  repre- 
sented by  three  distinct  nerve  arrangements  in  the  retina  and  visual 
apparatus.  ^Vllatever  view  is  taken  as  to  the  number  and  character 
of  the  fundamental  colors,  I  believe  with  Young  and  Helmholtz  that  in 
the  peripheral  end  organs  are  separate  structures  for  their  sifting.  It  is 
with  colors  as  it  is  with  other  forms  of  sensation.  All  cutaneous  sensa- 
tions, as,  for  instance,  those  for  touch,  pain,  and  temperature,  undergo 
a  receiving  and   sifting  or  differentiating  process  at  the  body's  pe- 


CORTICAL  CENTRES  91 

riphery.  These  sifted  sensations  are  carried  to  the  dorsal  ganglia,  to 
the  spinal  cord,  to  the  oblongata,  the  basal  ganglia,  and  the  brain  cor- 
tex by  separate  tracts,  and  it  is  believed  that  they  have  separate  centres 
of  representation  in  the  cortex.  The  phenomena  of  dissociated  cu- 
taneous sensations  are  best  explained  on  this  supposition,  and  color  sen- 
sations are  dependent  upon  analogous  structures  and  processes. 

Colors  are  recognized  by  means  of  a  specially  developed  apparatus, 
which,  beginning  with  certain  photo-chemical  substances  in  the  retina, 
ends  with  cell  groups  located  in  a  part  of  the  cerebral  cortex  set  apart 
for  color  recognition.  In  the  retina  are  differentiated  structures  for 
the  sifting  of  different  colors,  and  midway  between  these  and  the  ulti- 
mate cortical  centres  for  colors  is  a  basal  relaying  station,  the  pregenic- 
ulum,  in  which  colors  receive  their  first  central  differentiation.  Trans- 
mitting structures  for  color  sensations,  probably  also  differentiated,  are 
contained  in  the  optic  tract  and  the  optic  radiations.  It  is  through  the 
workings  of  this  barely  sketched  complex  mechanism  that  colors  are 
appreciated  as  separate  sensations.  The  first  sifting  takes  place  in  the 
retina,  the  last  in  the  cortex,  and  blendings  as  well  as  siftings  occur 
in  both.  These  blendings  give  the  different  hues  and  shades  of  color, 
those  in  the  cortex  being  interpreted  through  cellular  associations. 

Before  taking  up  the  consideration  of  cortical  disease  causing  color 
impairment  or  loss,  it  will  be  useful  to  glance  at  the  various  focal  affec- 
tions of  the  entire  visuo-chromatic  apparatus  capable  of  producing  these 
affections.  Beginning  at  the  extreme  periphery,  in  the  first  place,  one 
or  more  of  the  photo-chemical  substances  in  the  retina  may  be  congeni- 
tally  absent  or  deficient,  or  these  may  be  injured  or  destroyed.  Our 
knowledge  is,  however,  quite  limited  as  to  achromatopsias,  which  have 
their  cause  in  abnormalities  of  the  color  substances;  in  the  second 
place,  disease  involving  the  peripheral  nerve  apparatus,  which  takes 
part  in  the  early  stages  of  color  sifting,  that  is,  of  the  rods  and  cones 
and  of  the  ganglia-cells  or  other  cells  of  the  retina,  may  cause  color- 
blindness or  some  form  of  disturbance  of  color  perception.  This  has 
been  observed,  for  instance,  in  some  of  the  forms  of  retino-chorioiditis 
involving  either  the  macula  or  the  retinal  periphery. 

Color  sensation  may  be  interfered  with  or  lost  by  any  form  of 
disease  or  disturbance  which  impairs  or  destroys  the  conducting  fibres 
concerned  with  the  transmission  of  those  retinal  stimuli  which  give 
rise  in  consciousness  to  ideas  of  color.  The  optic  nerve  and  tract  are 
subject  to  such  affections  as  retrobulbar  neuritis  in  toxic  amblyopias, 
and  neuritic  atrophy  in  tabes  dorsalis.  They  and  the  optic  radiations 
may  be  the  seat  of  embolic  or  thrombotic  lesions  causing  softening,  or 


92  THE    EYE    AND    NERVOUS    SYSTEM 

somewhere  along  these  transmitting  paths  may  be  located  hemorrhages, 
abscesses  or  tumors.  In  so  far  as  such  lesions  interfere  with  the  fibres 
or  fibrils,  whose  business  it  is  to  transmit  vibrations  concerned  with 
color  perception,  impairment  or  loss  of  the  power  of  recognizing  colors 
will  result.  Gross  or  microscopic  disease  of  the  pregeniculum,  the  great 
relaying  station  in  the  visual  pathway,  will  give  various  affections  of 
the  color  sense,  according  to  its  nature,  exact  position  and  extent. 

In  the  occipital  cortex  are  situated  separate  centres  for  the  recogni- 
tion of  the  fundamental  colors.  Authorities  equally  high  differ  with 
regard  to  this  subject:  some,  like  Holden  ^"  in  a  recent  paper,  arguing 
that  it  is  not  necessary  to  predicate  the  existence  of  cortical  color  cen- 
tres in  order  to  explain  the  normal  chromatic  phenomena  or  the  dis- 
turbances in  color  recognition  which  result  from  disease  or  injury  of 
the  brain ;  others,  like  Landolt,  Verrey,^^  and  Vialet,  with  whom  I 
agree,  believing  that  distinct  centres  for  luminosity,  form,  and  color 
are  differentiated  in  contiguous  regions  of  the  cerebral  surface.  Pro- 
visionally I  would  place  these  centres  of  chromatic  representation  ven- 
trally  to  the  primitive  centres  for  light,  either  in  the  lingual  lobule  if 
the  representation  of  light,  that  is,  of  whiteness  and  blackness,  reaches 
only  to  the  calcarine  fissure,  or  in  the  fusiform  lobule  (fourth  temporo- 
occipital  convolution)  if  the  primary  centre  includes,  as  some  sup- 
pose, the  lingual  lobule   (fifth  temporo-occipital  convolution). 

It  would,  of  course,  be  absurd  to  contend  that  the  almost  innumera- 
ble shades  and  tones  of  color  have  separate  representation  in  cerebral 
cortical  cell  groups.  The  representation  as  indicated  is  probably  only 
for  the  fundamental  colors.  Wliat  is  known  as  to  complementary  colors 
and  as  to  the  results  of  the  admixture  of  different  colors  in  the  produc- 
tion of  white  or  gray,  and  also  as  to  the  blending  of  white  or  black  with 
the  various  fundamental  colors,  suggests  the  probable  explanation  of 
the  cerebral  processes  related  to  the  recognition  of  the  numerous  shades 
and  tints  of  color.  Both  in  the  eye  and  in  the  brain  fine  color  dis- 
crimination is  brought  about  through  processes  of  blending  or  associa- 
tion ;  at  the  periphery  through  photo-chemical  blendings  and  end-organ 
selection,  in  the  cortical  centres  through  neuronal  and  neuro-fibrillary 
associations. 

Wilbrand's  --  hypothesis  regarding  the  centres  for  color  and  for 
light  and  darkness,  already  alluded  to,  is  well  known  and  of  much 


*"  Holden,  Archives  of  Ophthalmolo":}',  vol.  xxiv.  p.  447. 
"  Verrpy,  Arch.  d'Ophthal.,  par.  v.  8.  .July  and  August,  1888. 
^  Wilbrand,  Ophthalmiatrische  Beitrage  zur  Diagnostik  der  Gehirnkrankheiten, 
1883. 


CORTICAL  CENTRES  93 

interest  and  needs  fuller  consideration  here.  He  would  have  us  be- 
lieve that  the  cell  groups  representing  color  and  light  are  placed  in 
layers  or  strata,  one  above  the  other,  in  the  cerebral  cortex  of  the 
calcarine  fissure.  Stratification  localization  has  been  favored  by  not  a 
few  neurophjsiologists.  The  representation  of  the  tactile  sense,  the 
muscular  sense,  and  of  movements  have,  for  instance,  been  regarded  as 
in  superimposed  layers  in  the  recognized  motor  cortex.  The  methods 
of  the  evolution  of  cerebral  structure  are  opposed  to  views  of  this  kind. 
The  cells  and  cell  groups  which  minister  to  special  functions  and  to 
association  are  arranged  with  considerable  uniformity  in  a  similar 
way  in  layers  and  strata  in  all  parts  of  the  brain.  As  function  is 
added  to  function  and  as  function  is  modified  in  the  course  of  phylo- 
genetic  and  ontogenetic  evolution,  the  unvarying  tendency  in  anatom- 
ical development  is  towards  surface  expansion.  To  accommodate 
evolving  function  new  regions  of  the  cerebral  cortex  are  added.  In 
this  way  primary,  secondary,  and  tertiary  fissures  and  gyres  have  de- 
veloped. The  organ  of  vision,  peripheral  and  cerebral,  at  first  only 
capable  of  appreciating  light  or  lightness  and  darkness,  as  evolution 
advances,  acquires  the  power  of  recognizing  color  and  form. 

According  to  Wilbrand,  the  light-dark  or  white-black  centres  are 
located  in  the  deepest  layers  of  the  primary  visual  cortex  near  the 
centrum  ovale,  while  the  cells  representing  the  different  colors  are 
arranged  in  superimposed  strata  nearer  the  cortical  surface.  In  order 
that  sensations  of  color  shall  be  realized,  it  is  necessary,  according  to 
him,  that  sensations  of  lightness  and  darkness  shall  be  first  appreciated, 
and  for  this  reason  these  perceptive  centres  are  most  deeply  placed  in 
the  cortical  cinerea.  A  visual  sensation  of  light  having  occurred,  this 
is  transferred  to  superimposed  layers.  Wilbrand  applies  Hering's 
valence  theory  as  to  the  manner  in  which  atoms  combine,  to  explain 
what  occurs  in  the  process  of  the  recognition  of  colors.  The  percep- 
tion of  color  is  in  accordance  with  the  valence  relations  which  the 
colors  bear  to  lightness  and  darkness.  Red,  green,  blue,  yellow,  each 
of  the  fundamental  colors,  has  in  it  its  own  color  valence  and  besides 
a  certain  valence  of  white  light,  and  in  accordance  with  the  destruction 
or  disturbance  of  this  valence  will  be  the  degree  of  impairment  of 
color  vision.  The  perceptive  centres  for  light  and  colors  in  the  cor- 
tex have  a  power  of  valence  differentiation  which  corresponds  with 
that  possessed  by  the  photo-chemical  substances  and  peripheral  nerve 
structures  at  the  other  extremity  of  the  visuo-chromatic  cerebral 
apparatus. 

I  shall  next  take  up  the  question  of  the  position  of  the  centres  of 


94  THE    EYE    AI^D    NERVOUS    SYSTEM 

color  representation  from  the  standpoint  of  clinicopatliological  evidence. 
The  cases  put  on  record  are  chiefly  illustrations  of  loss  of  color  recog- 
nition in  the  half-fields;  in  other  words,  of  hemiachromatopsia.  Cen- 
tral or  paracentral  achromatic  scotomata  are  sometimes  observed,  and 
doubtless  with  closer  observation  we  shall  in  the  future  be  able  to 
determine  central  as  well  as  peripheral  fields  of  color  recognition  with 
separate  cortical  centres  for  both.  It  is  altogether  probable  also  that 
for  color  as  for  light  there  are  lower  and  higher  macular  centres,  but 
we  are  not  yet  in  position  to  demonstrate  this  fact. 

The  clinicopathological  evidence  as  to  the  position  of  the  centres 
of  color  recognition  in  the  cortex  is  meagre  and  of  an  unsatisfactory 
character.  It  favors,  however,  the  convolutions  situated  ventrad  of  the 
calcarine  fissure  as  its  probable  seat.  In  a  case  reported  by  Henschen  ^ 
the  lingual  and  fusiform  iQbules  were  involved  in  the  destructive  lesion, 
although,  unfortunately  for  the  purposes  of  argument  regarding  the 
color  centres,  other  portions  were  also  implicated. 

Two  of  the  most  interesting  cases  of  achromatic  defect  with  lesions 
of  the  brain  demonstrated  by  necropsy  are  those  of  Brill  and  of  Verrey. 
As  the  case  of  Brill,^*  although  reported  twenty-two  years  since,  has 
received  little  or  no  attention  from  writers  on  the  subject,  I  shall  refer 
to  it  in  some  detail. 

The  patient,  a  man  sixty -three  years  old,  in  October,  1881,  had  an 
apoplectic  attack  resulting  in  right  hemiparesis,  right  hemianesthesia, 
incontinence  of  urine,  and  increased  right  knee  jerk.  After  several 
weeks  these  symptoms  were  changed  and  supplemented ;  the  anaesthesia 
of  the  thigh  became  hypencsthesia,  tremor  of  the  paralyzed  side  de- 
veloped, and  the  patient  suffered  from  headache,  restlessness  and  in- 
somnia, with  spells  of  tinnitus  and  dazing  and  also  dulness  of  mind. 
His  sight  became  dull  and  hazy.  Four  months  after  the  attack  the 
patient  was  examined  by  Dr.  Brill  and  Dr.  E.  C.  Spitzka  in  consulta- 
tion. He  failed  to  recognize  green ;  he  was  doubtful  as  to  whether  vio- 
let was  greenish  or  bluish ;  green,  he  said,  was  red  or  rather  pinkish ; 
he  could  always  recognize  red  and  yellow.  The  color  loss  involved  both 
eyes.  Gross  objective  vision  was  retained  except  a  subjective  blurring; 
the  fields  of  vision  were  not  limited.  The  patient  was  illiterate,  so  that 
his  condition   as  to  letter-blindness  and   word-blindness  could  not  be 


"Henschen,  Klin.  u.  Anat.  Beitr.  z.  Pathol,  des  Gehirns,  Upsala,  1890,  1903. 

*♦  Brill,  Amer.  Jour,  of  Neurology  and  Psychiatry,  vol.  i,  N.  Y.,  1882.  Previous 
to  the  report  of  the  necropsy,  Brill  had  reported  this  case  clinically  in  the  Chicago 
Med.  Review,  April,  1882. 


CORTICAL  CENTRES 


95 


determined.     He  had  worked  on  the  railroad  for  thirty'  years  before 
his  illness,  and  was  able  to  recognize  all  colors. 

He  died  about  six  and  a  half  months  after  admission  to  the  hos- 
pital. At  the  necropsy  a  softened  area  was  found  at  the  apex  of  the 
left  cuneus  and  the  adjoining  upper  part  of  the  lingual  convolution. 
Brill  exactly  describes  the  area  of  softening  as  follows :  "  The  focus 
was  triangular,  the  apex  being  directed  forward  and  the  short  side 
posteriorly.  It  extended  to  within  a  centimetre  and  a  half  of  the  apex 
of  the  occipital  lobe,  and  involved  half  the  altitude  of  the  cuneus  in 
the  middle  of  the  latter  and  almost  the  entire  apex  of  this  lobe.  The 
g}'rus  lingualis  was  involved  for  about  one-quarter  of  its  width  on  the 
surface,  and  evenly  from  the  posterior  almost  to  the  anterior  extent 
of  the  lesion  described  (Fig.  14).     The  calcarine  fissure  could  not  be 


Fig.  14. — Mesal  face  of  the  occipital  lobe,  showing  the  superficial  position  and  extent  of  an  area 
of  softening  causing  achromatopsia ;  the  calcarine  fissure  is  indicated  where  the  white  interspaces  are 
shown ;  the  lesion  involved  the  inferior  portion  of  the  cuneus  and  a  bordering  zone  of  the  lingual 
lobule.    (Brill.) 

identified  excepting  its  most  anterior  extremity,  and  even  at  this 
spot  both  borders  were  softened.  At  its  lowest  border  the  softening 
reached  almost  to  the  posterior  cornu."  Brill  cites  chiefly  from  Ex- 
ner's  -^  work  on  cerebral  localization  a  number  of  cases  of  impairment 
or  loss  of  color  recognition  from  cerebral  disease. 

It  is  unfortunate  that  Brill  in  the  report  of  this  most  interesting 
case  does  not  clearly  indicate  whether  the  color-blindness  in  both  eyes 
was  hemiachromatopsia  or  an  absence  of  the  power  of  recognizing 
colors  without  reference  particularly  to  the  half-fields.  Nevertheless  the 
case  is  of  value  in  connection  with  the  necropsy,  which  clearly  indi- 


^  Exner.  Unterstichungen  iiber  die  Localization  der  Functionen  in  der  Grcsshirn- 
rinde  des  Menschen,  Wien,  1881. 


96  THE    EYE    AND    NERVOUS    SYSTEM 

catea  that  cerebral  achromatopsia  in  this,  as  in  the  other  cases  referred 
to,  was  associated  with  disease  of  the  region  ventrad  to  the  calcarine 
fissure,  although  it  also  included  this  fissure  and  a  part  of  the  cuneus. 

Verrey  reported,  in  1888,  the  most  interesting  case  of  hemiachro- 
matopsia  with  necropsy,  showing  the  lesion  to  which  the  loss  of  the 
chromatic  sense  was  due,  which  has  yet  been  put  on  record.  The 
patient  was  a  woman  sixty  years  old,  whose  symptoms  were  difficulty 
in  reading,  with  good  general  visual  acuity,  slight  concentric  contrac- 
tion of  the  field,  complete  absolute  right  hemiacliromatopsia,  and  a 
sensible  diminution  of  visual  acuity  for  the  right  half  of  the  visual 
field.  The  patient  had  paroxysmal  headache  and  attacks  of  vertigo 
with  vomiting.  She  gradually  developed  fatigue  in  reading,  with  letter 
dancing  and  dyslexia.  Examination  showed  that  with  her  central  field 
she  could  read  letters  and  words  written  in  different  colors.  Twenty 
days  before  her  death  the  patient's  symptoms  were  much  the  same  as 
above  summarized,  with  the  exception  of  a  further  enfeeblement  of 
luminous  perception.  Eight  days  before  her  death  she  fell,  and  at 
this  time  developed  a  complete  left  hemiplegia,  entire  cutaneous  irre- 
sponsc,  violent  vomiting  and  clonic  and  tonic  convulsions  of  the  face 
and  right  side.  The  necropsy  showed  recent  large  hemorrhage  in  the 
right  hemisphere  destroying  the  centrum  ovale  and  the  basal  ganglia, 
and  filling  the  right  lateral  ventricle. '  A  fresh  hemorrhage  the  size 
of  a  bean  was  situated  at  about  the  junction  of  the  anterior  and  middle 
third  of  the  left  callosum.  A  second  lesion  was  found  between  the 
floor  of  the  posterior  horn  of  the  left  lateral  ventricle  and  the  basal 
surface  of  the  occipital  lobe.  Close  examination  showed  a  large  throm- 
bosed vessel,  and  that  the  parts  destroyed  were  the  white  substance  of 
the  third  occipital  convolution,  more  or  less  completely  the  white  sub- 
stance of  the  occipital  extremities  of  the  lingual  and  fusiform  convolu- 
tions, and  the  posterior  inferior  point  of  the  cuneus.  The  superficial 
involvement  of  the  lingual  and  fusiform  convolutions  is  shown  in 
Fig.  15. 

The  following  case  of  double  hemiachromatopsia,  recorded  by  Mac- 
kay  and  Dunlop,^^  also  throws  some  light  on  the  probable  position  of 
the  cortical  color  centres.  The  lesions  present  were  in  the  temporo- 
occipital  lobe  almost  entirely  ventrad  of  the  calcarine  fissure.  The 
patient,  a  man  sixty-two  years  old,  had  a  sarcoma  of  the  stomach.  He 
had  an  attack  of  general  disturbance  of  vision,  and  examination  on  the 
same  day  showed  that  he  was  completely  color-blind.      Examination 


Maokay  and  Dunlop.  Scottish  Mod.  and  Surgical  Journ.,  December,  1899. 


COKTICAL  CEXTRES 


97 


showed  a  double  homonymous  hemianopsia  with  greater  involvement 
of  the  left  side.  Later  the  power  of  vision  for  light  and  form  improved, 
while  that  for  color  remained  i^ermanently  lost.  Five  months  after  the 
first  attack  causing  disturbance  of  vision  and  color-blindness  he  had  an 
attack  of  right  hemiplegia  from  which  he  died.  At  the  necropsy  the 
oj)tic  nerves  were  found  to  be  intact.  Both  occipital  lobes  showed 
atrophic  areas.  The  atrophy  included  the  posterior  parts  of  the  tem- 
l^oro-occipital  convolutions  on  both  sides  with  an  extensive  destruction 
of  the  tissues  on  the  right  side.  On  the  left  side  the  atrophy  extended 
deeply  and  involved  a  small  area  of  the  gray  substance  of  the  cal- 
carine  fissure.     On  both  sides  the  lower  borders  of  the  optic  radiations 


Fig.  15.— Mesal  aspect  of  the  left  hemicerebrum,  showing  the  cortical  extent  of  a  lesion  causing 
"hemiachromatopsia ;  the  subcortical  radiations  of  the  lesion  are  described  in  the  text.  A  small  lesion 
of  the  callosum  is  also  shown.    (Verrey.) 

were  affected.  The  process  was  supposed  to  be  due  to  hyaloid  de- 
generation of  the  vessels.  The  case  was  not  a  pure  one  of  hemiachro- 
matopsia ;  the  visual  fields  for  light  and  form  were  to  some  extent 
affected. 

In  this  case  the  chief  destruction  causing  the  hemiachromatopsia 
was  in  the  lingual  and  fusiform  lobules  (fourth  and  fifth  temporo- 
occipital  convolutions)  and  the  posterior  inferior  portion  of  the  cuneus 
and  calcarine  fissure  were  implicated.  This  case  again  points  to  the 
convolutions  situated  ventrally  to  the  calcarine  fissure  as  the  part  of 
the  temporo-occipital  region  most  probably  concerned  with  color  rec- 
ognition. 

A  case  recorded  by  Swanzey  ^^  carries  with  it  some  suggestion  as 
to  the  site  of  the  centres  for  color  recognition.     A  man  seventy-seven 

*^  Swanzey,  Trans.  Oph.  Soc.,  vol.  iii.  p.  185. 
7 


98  THE    EYE    AND    NERVOUS    SYSTEM 

Years  old  smklenly  Wame  affected  with  a  slight  hoiuonyinoiis  defect 
for  light  in  the  left  upper  quadrant  of  the  field  of  vision,  while  over 
the  entire  left  field  color  sensation  was  absent.  This  case,  as  pointed 
out  by  Gowers,  suggests  that  the  centre  for  colors  is  adjacent  to  that 
part  of  the  half  vision  centre  in  which  the  upper  quadrant  is  repre- 
sented. It  will  be  remembered  that  the  case  of  quadrant  anopsia  of 
Beevor  and  Collier  seems  to  show  that  the  upper  quadrant  of  each 
visual  field  is  represented  in  the  lower  part  of  the  cuneus  and  the 
calcarine  fissure  and  perhaps  in  the  lingual  lobule.  If  this  be  true 
the  case  of  Swanzey  would  point  to  the  parts  adjoining  this  quadrautic 
centre,  most  })robably  to  the  lingual  or  fusiform  lobule  as  the  seat  of 
color  representation. 

A  few  other  cases  of  achromatopsia  probably  due  to  cortical,  or  at 
least  to  cerebral,  disease  are  worthy  of  citation,  although  little  is 
taught  by  them  with  regard  to  the  position  of  the  color  centres.  In  a 
case  reported  by  Eperon,^*  a  man  seventy  years  old  had  suffered  witli 
hemiparesis,  which  almost  entirely  disappeared  except  a  sensation  of 
numbness  in  the  right  arm ;  suddenly  he  developed  word-blindness. 
On  examination  it  was  found  that  in  the  entire  right  half  of  the  field 
the  sensation  for  color  was  missing.  Perception  of  light  and  acuteness 
of  vision,  although  present,  were  considerably  reduced.  A  man  fifty- 
four  years  old  had  diminution  of  vision  with  glimmerings  or  flickerings 
before  the  eyes  for  ten  or  twelve  days.  He  had  complete  left  hemian- 
opsia for  all  colored  objects;  he  also  had  hemianopsia  for  small  white 
objects,  but  not  for  those  of  larger  size.  His  vision  was  five-sixths  of 
the  normal  when  black  characters  were  on  a  white  ground,  but  when 
the  characters  were  i)ale  the  vision  was  reduced  to  six  and  a  half.  Im- 
provement took  place,  but  synmietrical  scotomata  remained  on  each 
side,  about  fifteen  millimetres  from  the  point  of  fixation.  Sensation  for 
colors  returned,  but  not  as  distinctly  as  l)efore  the  attack.  After  this 
decided  improvement  a  sudden  right-sided  hemiana»sthesia  appeared 
and  vision  became  worse  again.  A  small  defect  for  white  was  present 
in  the  whole  of  the  upper  right  quadrant.  This  case  is  recorded  by 
^leisling.-"  In  another  case,  which  we  owe  to  Ziehl,^"  a  man  on  awaken- 
ing in  the  morning  found  that  he  was  unable  to  read.  Hemianopsia 
was  absent,  but  right  homonymous  hemiachromatopsia  was  present, 
the  dividing  line  passing  vertically  through  the  fixation  point.     In  the 

**  Kperon,  Archives  d'Oplithalmologie,  iv.    1884,  Juillet-Aout. 
™  Meislinp,  Ref.  Jahresb.  f.  Ophth.,  1897,  p.  .314. 

^"Ziehl,  Vcrhandl.  d.  (iesellseh.  Deutsclier  Nuturf.  u.  Aerzte.  67.  Vers,  zu  Liibeck. 
ii.  Teil.;     ii.  Hiilfte,  p.   184,   ISO.'i. 


CORTICAL  CENTRES  99 

affected  fields  all  colors  appeared  as  gray.  In  a  case  recorded  by 
Gaiidenzi,^^  relative  hemialeucopsia  and  hemiacliromatopsia  were 
present. 

In  a  case  of  hemiacliromatopsia,  reported  by  Samelsohn,^^  the 
autopsy  showed  a  gliosarcoma  of  the  optic  tract  with  some  invasion  of 
the  thalamus  and  geniculate  body,  the  optic  radiations  and  cortex  being 
free  from  disease.  In  a  case  of  Uhthoff,'^"^  with  normal  sharpness  of  • 
vision,  unilateral  hemianopsia  for  color  and  normal  limits  for  white 
were  present,  the  autopsy  revealing  a  sarcoma  of  the  pons  which  com- 
pressed downwards  the  cerebral  peduncles  and  reached  into  the  third 
ventricle.  On  the  left  the  tumor  had  grown  into  the  anterior  portion 
of  the  median  fossa  causing  protrusion  of  the  dura.  The  chiasm  was 
much  attenuated.  The  right  tract  was  somewhat  contracted  in  the 
jjosition  of  the  chiasm.  In  another  case  put  on  record  by  Samelsohn  ^* 
the  patient  had  some  difficulties  of  vision  following  an  apoplectiform 
seizure.  At  this  time  the  entire  right  side  was  i)aralyzed,  but  when 
examined  by  Samelsohn  only  a  slight  hemiparesis  of  the  right  arm  and 
thigh  without  any  disturbance  of  sensation  could  be  found.  The  pa- 
tient had  a  typical  left-sided  hemianopsia  for  colors.  From  the  vertical 
dividing  line  all  colors  were  seen  on  the  right,  but  none  on  the  left. 
Rjerrum  ^^  has  reported  the  case  of  a  man  thirty-nine  years  old,  who, 
after  headache  lasting  fourteen  days,  developed  indistinct  vision.  The 
outer  limits  of  the  light  field  were  normal,  as  was  also  the  field  for 
form.  Upon  examining  him  with  colored  objects  it  was  found  that  he 
was  suffering  with  complete  color-blindness  in  the  left  half  of  the  visual 
fields  for  both  eyes,  the  dividing  line  being  vertical  to  the  fixation 
point.  All  colors  in  the  achromatopsic  fields  were  recognized  as  gray. 
The  day  after  he  was  examined  intense  headache  and  inflammatory 
swelling  in  the  left  temporal  region  were  present.  An  abscess  formed; 
an  incision  was  made,  and  the  patient  died,  but  necropsy  was  refused. 
Besides  the  case  of  Mackay  and  Dunlop,  already  referred  to,  a  few 
other  cases  of  double  hemiacliromatopsia  have  been  recorded.  Steffan^* 
recites  the  case  of  a  man  sixty-two  years  old,  a  printer  of  colors,  who, 
before  the  attack,  having  had  a  perfect  sense  for  colors,  after  an 
apoplectiform  attack  with  dizziness  and  darkness  before  the  eyes,  but 

"  Gaudenzi,  Ref.  Jahresb.  f.  Ophthalm.,  1899,  476. 

'■''-  Samelsohn,    Kin    Fall    von    Hemiachroniatopie.      Berliner    klin.    VVochenschr., 
IS90,  p.  331;    Niederrhein.   Gesellsch.  zu  Bonn. 

^  Uhthoff,  Berliner  klin.  Wochenschr.,,  Nr.  12,  1897. 
^  Samelsohn,  Cfentralbl.  f.  Wissenseh.r  1881,  Nr.  47  u.  50. 
'M3jerrum,  Ref.  Centralbl.  f.  prakt.  Augenheilk.,  1881,  471. 
^^Steffan,  Arch.  f.  Ophth.,  xxvii.  1881. 


100  THE  EYE  AND  NERVOUS   SYSTEM 

without  paresis  or  loss  of  consciousness,  had  total  color-blindness  in 
both  eyes  without  affecting  the  sharpness  of  vision  or  the  visual  fields. 
Examination  of  the  eyes  showed  nothing  abnormal.  Examinations 
were  made  shortly  after  the  attack  and  also  four  years  later.  At  this 
time  he  was  not  totally  color-blind,  but  tests  showed  pronounced  blind- 
ness for  red  and  green  and  diminution  for  other  colors.  The  central 
sharpness  of  sight  and  the  visual  fields  were  still  normal.  No  dis- 
turbance was  present  of  the  sense  of  white  or  black.  Later  on  the  sen- 
sation for  red,  yellow,  and  blue  was  present,  but  only  when  the  colors 
were  extremely  intense.  Alexander  ^"  reports  the  case  of  a  man  who 
had,  a  short  time  before  coming  under  observation,  lost  the  ability  to 
recognize  colors.  On  examination  with  samples  of  colored  paper,  light 
green  appeared  to  be  yellow,  dark  gray  violet,  red,  and  scarlet  and 
brown,  green.  The  acuity  of  vision  was  normal.  The  eycgrounds  were 
normal,  with  the  exception  that  they  were  considerably  pigmented. 
Two  years  later  the  condition  had  changed  but  little.  The  color-blind- 
ness was  extended  over  almost  the  entire  visual  fields.  Xo  scotomata 
were  present.  A  man  fifty-four  years  old,  whose  history  is  given  by 
Quaglino,^^  after  an  apoplectiform  attack  was  found  to  be  completely 
amaurotic  and  paralyzed  on  the  left  side.  The  hemiplegia  disappeared 
slowly  and  the  visual  power  gradually  imi)roved.  In  a  year  the  hemi- 
plegia had  disappeared  entirely.  The  visual  acuity  was  now  excellent, 
he  being  able  to  read  the  smallest  type  and  see  the  small  birds  at  the 
top  of  the  trees.  He  had,  however,  incomplete  left  lateral  homonymous 
hemianopsia.  After  the  attack  he  could  not  recognize  any  colors  but 
black  and  white;  all  faces  appeared  to  him  pale  and  colorless.  The 
color-blindness  was  not  confined  to  the  hemianopsic  field,  but 
extended  over  the  fields  of  both  eyes.  This  patient  lost  the  ability  to 
recall  the  form  and  configuration  of  objects,  although  he  knew  what 
they  were  when  they  were  first  seen  by  him.  In  a  case  of  Schoeler  and 
Uhtlioff,^*'  a  man  sixty-two  years  old,  in  full  possession  of  the  color 
sense,  developed  a  right-sided  hemiachromatopsia.  He  also  had  upper 
quadrantic  achromatopsia  in  the  opposite  fields.  It  will  be  seen  that 
this  was  not  a  complete  double  hemiachromatopsia. 

In  some  cases  in  which  the  color  sense  is  affected  a  person  can  dif- 
ferentiate between  different  colors,  and  yet  not  have  the  power  to  pick 
out  particular  colors.     He  may  recognize  the  fact  that  red  differs  from 


^  Alexander,  Arcli.  f.  Ophth.,  xv.  iii.,  102. 
"■' t^uaglino,  (Jiornale  d'oftalmologia.  1867. 

•^*  Schoeler  and   Ulithoff.   Beitriipe  zur  Pathologic  der  Sehncrven  und  dcr  Netz- 
haut..  Berlin,   1884;    H.  Peters,  p.  88. 


CORTICAL  CENTRES  101 

green  and  green  from  blue  or  yellow,  or  that  any  one  color  from  one 
or  all  other  colors,  and  yet  he  may  not  be  able  to  say  which  is  this  or 
that  color.  In  a  case  of  Lissauer  *'^  the  patient  could  differentiate  be- 
tween colors  in  wools,  but  when  asked  to  pick  out  special  colors  he  made 
the  grossest  errors.  With  his  color-blindness  a  diminution  in  visual 
acuity  was  associated. 

Under  such  names  as  color  amnesia  and  amnesic  color-blindness  is 
described  a  symptom  which  is  exhibited  by  the  inability  of  the  person 
to  name  a  color  which  he  evidently  recognizes.  It  is  a  variety  of  the 
visual  anomia  (word-dumbness,  optic  aphasia)  hereafter  described,  see 
page  148.  The  patient  may  be  unable  to  name  any  color,  or  his  word- 
dumbness  may  be  for  only  one  or  two  colors.  In  a  case  recently  stud- 
ied by  me,  for  instance,  when  a  green  testing  object  was  presented  to 
the  patient,  he  could  not  name  the  color,  although  indicating  that  he 
knew  it.  Finally  he  said,  "  It  is  the  Irishman,"  showing  in  this  way 
his  appreciation  of  the  fact  that  it  was  green.  He  instantly  recog- 
nized blue  and  red ;  yellow  he  called  a  "  dirty  white."  The  same 
answers  were  given  for  either  the  right  or  the  left  eye  and  for  both 
eyes  together.  When  he  was  given  a  number  of  objects  of  different 
colors  and  asked  to  pick  out  particular  colors,  he  always  did  so  cor- 
rectly, thus  differing  from  the  patient  of  Lissauer,  above  referred  to, 
who  could  not  do  this,  although  he  knew  that  the  colors  were  different. 
This  symptom  should,  I  believe,  be  termed  color  aphasia  or  color 
anomia  rather  than  color  amnesia  or  amnesic  color-blindness.  It  is 
probably  dependent  upon  some  disturbances  of  the  tracts  associating 
the  cortical  centres  for  color  with  the  speech  centres,  sensory  or  motor. 

Some  of  the  hysterical  disorders  of  the  color  sense  are  of  particular 
interest  in  connection  with  the  discussion  of  the  question  of  cortical 
centres  for  color,  both  in  the  lower  and  in  the  higher  visual  fields.  The 
mere  fact  that  these  disorders  are  nearly  always,  if  not  always,  uni- 
lateral, points  to  unilateral  cerebral  centres  of  representation.  It 
is  not  within  the  scope  of  this  article  to  direct  attention  in  detail 
to  this  subject,  which  is  considered  in  chapter  xvi.  I  would  like, 
however,  to  point  out  in  this  connection  that  the  most  probable  ex- 
planation of  the  phenomena  is  similar  to  that  which  is  given  for 
unilateral  amblyopia  when  discussing  the  crossed  amblyopias.  The 
visuo-sensory  centres  for  color  are  functioning  normally,  or  in  condi- 
tion to  so  function.  The  visuo-psychic  centres  for  color  are  in  abey- 
ance or  are  temporarily  functionally  disjointed  from  the  primary  cen- 

*"  Lissauer,  Archiv  fiir  Psvchiatrie  iind  Nervenheilkunde,  vol.  xxi,  1889-1890. 


102  THE    EYE    AND    NERVOUS    SYSTEM 

tres.  The  affection  is  psychic,  as  Bernheiiu  "*'  believes.  The  same  ex- 
])laiiation  holds  as  would  be  given  for  various  phenomena  of  hypnosis. 
A  few  remarkable  cases  of  total  uniocular  color-blindness  have  been 
put  on  record,  the  acuity  of  vision  and  the  fields  for  light  and  form 
l)eing  normal.  The  difficulty  of  explaining  such  cases  on  the  doctrine 
of  cortical  arrest  or  destruction  is  evident.  The  centres  for  color,  like 
those  for  light,  are  not  for  each  eye,  but  for  the  homonymous  fields  of 
both  eyes ;  and  it  is  therefore  necessary  to  believe  that  the  centres 
related  to  the  direct  fibres  of  the  optic  path  on  one  side  and  those 
relating  to  the  crossed  fibres  on  the  other  side  are  arrested  in  develop- 
ment. This,  although  improbable,  is  by  no  means  impossible.  Con- 
genital arrest  may  depend  upon  bilateral  defect  of  centres  which  work 
together  in  function.  Uniocular  color-blindness  is  better  explained  on 
the  theory  of  deficiency  or  disease  in  front  of  the  chiasm,  as,  for  in- 
stance, absence  of  the  color  substance  in  one  eye,  or  unilateral  defect 
of  the  peripheral  nerve  organs  concerned  with  color  selection.  In  some 
cases  this  unilateral  color-blindness  is  partial ;  it  may,  for  instance, 
be  for  red-green  or  blue-yellow.  In  bilateral  cases  of  congenital  color- 
blindness, which  is  the  most  common  form,  the  powers  of  visual  recogni- 
tion for  light  and  form  are  retained,  but  all  colors  fail  to  be  recognized. 
Such  a  total  congenital  color-blindness  may  be  due  to  the  arrested  de- 
velopment of  the  entire  apparatus  concerned  with  color  sensation;  or 
of  some  one  portion  of  the  apparatus,  as  of  its  periphery,  of  its  basal 
centres,  or  of  its  cortical  centres  in  their  entirety. 

AMBLYOPIAS  DUE  TO  DISEASE  OF  THE  VISUAL  CORTEX  OR 
OF  ITS  ENTERING  AND  CONNECTING  TRACTS. 

Besides  the  anopsias,  hemianopsias  and  achromatopsias  described 
as  the  resiilts  of  lesions  located  in  certain  portions  of  the  areas  for 
vision  of  the  cerebral  surface,  other  forms  of  impairment  of  sight,  due 
to  disease  of  the  visual  cortex,  or  of  its  entering  or  connecting  tracts, 
are  sometimes  met  with  and  are  usually  included  under  the  general 
head  of  amblyopias.  Crossed  amblyopia  is  an  affection  of  sight  due  to' 
cerebral  disease,  in  which  the  sole  or  chief  impairment  is  on  the  side 
opposite  to  the  lesion.  In  the  few  organic  cases  of  this  disease  which 
have  been  recorded,  moderate  or  slight  dimness  of  vision  on  the  same 
side  as  the  lesion  has  been  present  in  connection  with  the  marked  con- 
tralateral impairment  or  loss. 


"  Bcrnhcim,  Brain,  vol.  16,  1893. 


COETICAL  CENTRES  103 

The  discussion  of  the  subject  of  crossed  amblyopia  is  intimately 
related  to  that  of  cortical  macular  representation.  I  have  already  indi- 
cated my  belief  in  the  existence  of  an  area  of  macular  representation 
in  the  primary  or  lower  visual  centres,  and  I  have  subscribed  for  the 
present  to  the  view  of  Henschen  that  this  macular  centre  is  probably 
situated  in  and  around  the  anterior  extremity  of  the  calcarine  fissure. 
In  the  higher  visual  zone,  in  the  angular  or  angulo-occipital  region,  is 
also  situated  a  cortical  area  for  the  macula.  The  lower  macular  area 
subserves  clear  vision  in  what  might  be  termed  its  most  primitive  form. 
Luminosity,  or,  rather,  whiteness  and  blackness,  are  here  perceived ;  ob- 
jects here  first  receive  their  clearest  recognition.  The  centre,  however, 
is  purely  perceptive ;  it  is  simply  a  part  of  the  visuo-sensory  area  of 
Campbell  and  Mott.  Natural  objects,  faces,  persons,  designs,  words, 
letters  or  numbers  are  perceived  through  the  functioning  of  this  lower 
macular  cortical  area,  but  without  a  recognition  of  their  significance ; 
they  are  perceived,  but  they  are  not  apperceived.  Perception  by  the 
lower  macular  centre  does  not  evoke  concrete  ideas  the  result  of  visual 
memories.  Such  visual  ideas  are  the  outcome  of  the  activities  of  the 
higher  visual  field,  including  its  macular  subdivision. 

It  may  be  well  to  direct  attention  here  to  the  different  views  which 
have  been  held  with  regard  to  macular  representation.  While  Hen- 
schen, whom  the  writer  had  chosen  to  follow,  would  limit  it  to  the 
anterior  and  ventral  portion  of  the  cuneo-calcarine  visual  centre,  others 
have  suggested  as  its  location  the  posterior  extremity  of  the  calcarine 
fissure  and  its  bordering  cortex.  Von  Monakow  holds  that  the  macula 
has  a  variable  representation  spread  out  over  both  the  lateral  and  mesal 
aspect  of  the  occipital  lobe ;  in  other  words,  over  both  the  lower  and 
higher  visual  fields.  The  facts  at  our  command  do  not  seem  to  me 
to  support  this  view.  The  physiolog}^  of  sight  and  clinical  experience 
point  to  a  more  or  less  concentrated  cortical  representation  of  the 
macula. 

From  his  own  experiments  and  those  of  Schiifer  and  Munk,  Ferrier 
argues  that  the  angular  gyres  are  more  particularly  related  to  the  areas 
of  distinct  vision, — that  is,  that  they  represent  the  macula.  He  also 
holds  that  the  macula  is  represented  in  both  hemispheres,  but  most  in 
the  angular  convolution  of  the  opposite  side.  His  views  are  indicated 
schematically  in  Fig.  16. 

Lannegrace  *^  and  Ferrier  and  Yeo,  who  have  performed  numerous 
extirpation  experiments  on  the  occipital  lobe  and  the  angular  gyre  of 

*-  Lannegrace,  Influence  des  Lesions  Corticales  sur  la  Vue,  Archives  de  Medicine 
Experimentale,  1889. 


104  TIIK  EVK   AND   NERVOUS   SYSTEM 

the  brains  of  monkeys,  hold  that  destruction  of  the  occipital  lobe  causes 
no  ai)preciable  impairment  of  vision,  while  destruction  of  the  angular 
convolution  causes  crossed  amblyopia  of  a  temporary  character.  In 
one  of  Ferrier's  most  striking  experiments  he  removed  one  of  the  eyes 
of  a  monkey  and  then  destroyed  the  angular  convolution  of  the  same 
side;  this  caused  total  blindness  in  the  remaining  eye  of  the  opposite 
side,  as  was  completely  verified  by  numerous  experiments.  The  ani- 
mal, however,  showed  some  signs  of  returning  vision  the  next  day,  but 
died  as  the  result  of  exhaustion,  the  weather  being  extremely  cold.     In 


Fic.  16.— Scheme  of  the  optic  tracts  and  visual  centres.  A,  the  right  and  A',  the  left  angular  gyrus ; 
C,  optic  chiasm ;  E,  the  right  and  E'.  the  left  eye ;  n,  the  right  and  n',  the  left  optic  nerve ;  O,  the  right 
and  C,  the  left  occipital  lobe ;  T,  the  right  and  T',  the  left  optic  tract ;  the  thin  continuous  line  repre- 
sents the  retinal  relations  of  O ;  the  thick  continuous  line  represents  the  retinal  relations  of  O' ;  the 
interrupted  line  indicates  the  retinal  relations  of  A,  and  the  dotted  line  the  retinal  relations  of  A' ;  the 
relations  of  A  and  A'  with  the  eye  on  the  same  side  are  indicated  by  finer  interrupted  and  dotted  lines 
respectively.    (Ferrier.) 

another  exj)eriment  Ferrier  carefully  examined  the  condition  of  vision 
in  a  monkey  in  which  he  had  completely  destroyed  both  angular  gyres, 
and  recorded  the  results  as  follows: 

"  There  was  no  ptosis,  the  ocular  movements  were  normal,  the  con- 
junctival reflexes  unimpaired,  sensibility  was  intact  everywhere,  and 
the  motor  powers  were  perfect,  but  for  four  days  at  least  the  animal 
was  evidently  absolutely  blind.  When  urged  to  move  it  ran  against 
every  obstacle  in  its  i)ath,  paid  no  attention  to  threats,  could  not  find  its 
food,  except  by  groping,  and  appeared  insensible  to  light  flashed  in  its 
eyes.  On  the  fifth  day  there  were  evidences  of  returning  vision.  It 
did  not  knock  its  head  against  obstacles;  would  not  walk  over  the  edge 
of  the  table ;  showed  signs  of  perception  of  light  flashed  in  its  eyes  and 
occasionally    seemed    to  wince    when    threatened.      Vision    gradually 


COKTICAL  CENTRES  105 

improved,  but  continued  very  imperfect,  especially  for  minute  objects 
which  it  rarely,  if  ever,  seized  quite  precisely;  groping  at  them  with 
the  whole  hand,  and  reaching  short,  or  over,  or  to  the  side.  It  ap- 
peared to  see  objects  held  above,  below,  or  to  either  side  much  better 
than  those  held  in  front  of  its  eyes.  Six  weeks  after  the  operation  my 
colleague.  Professor  McHardy,  examined  the  animal,  which  was  very 
docile  with  me,  testing  every  portion  of  the  visual  field  by  pieces  of 
apple  suspended  by  a  delicate  thread.  It  was  concluded  that  vision 
was  better  in  every  part  of  the  periphery  than  in  the  centre.  Objects 
held  directly  before  the  eyes  and  at  a  little  distance  were  apparently 
not  clearly  seen,  and  never  laid  hold  of  with  precision.  The  condition 
remained  unchanged  for  three  months  after  the  operation,  similar  tests 
being  from  time  to  time  applied,  and  with  the  same  result.  I  noted  also 
that  the  animal,  when  examining  any  object,  always  held  it  at  full 
arm's  length  from  its  eyes.  The  phenomena  observable  in  the  animal 
were  such  as  would  be  best  explained  by  impairment  or  loss  of  central 
vision." 

A  few  cases  have  been  recorded  by  Demange,  Sharkey,  and  others 
which  appear  to  indicate  that  a  destructive  lesion  of  the  angular  gyre 
of  one  side  in  man  will  cause  loss  or  great  impairment  of  vision  of  the 
opposite  eye,  with  contraction  of  the  visual  field  of  the  same  side.  In 
a  case  recorded  by  Demange,'*^  complete  loss  of  sight  on  the  side  oppo- 
site to  the  lesion  was  present,  as  well  as  hemiangesthesia  and  loss  of 
hearing,  taste,  and  smell.  The  lesion  in  this  case  was  a  large  subcorti- 
cal area  of  softening  extending  beneath  the  convexity  of  a  large  part 
of  the  right  hemisphere.  The  mesal  aspect  of  the  hemicerebrum  and 
the  internal  capsule  were  nowhere  implicated. 

One  of  the  cases  frequently  referred  to  and  especially  used  by 
Gowers  and  Ferrier  in  support  of  the  view  that  crossed  amblyopia  is 
due  to  lesion  of  the  angular  convolution,  is  that  of  Sharkey.^*  In  this 
case  there  were  left  hemiplegia,  more  marked  in  the  arm  and  leg  than 
in  the  face,  dimness  of  vision,  impairment  of  hearing  on  the  left,  loss 
of  taste  and  of  common  sensibility  on  the  left  half  of  the  tongue,  and 
also  loss  of  sensibility  in  the  entire  left  half  of  the  body.  The  paralysis 
of  the  leg  largely,  and  the  anaesthesia  entirely,  disappeared.  The  entire 
right  hemisphere  was  much  reduced  in  size,  and,  according  to  the 
report,  the  convolutions  supplied  by  the  right  Sylvian  artery  were 
nearly  all  necrosed  and  atrophied.     The  crossed  amblyopia  was  one  of 


"Demange,  Revue  Dc  M6d.,  May,  1883,  p.  391 
"Sharkey,  Med.-Chir.  Trans.,  London,  1884,  p.  205 


106  Tilt:    EYE    AND    XERVOUS    SYSTEM 

the  most  interesting  of  the  clinical  phenomena  present.  The  blindness 
in  the  left  eye  was  almost  complete  at  first.  Improvement  in  sight,  as 
in  the  other  special  senses,  took  place  in  about  four  weeks,  and  in  six 
weeks  and  a  half  vision  was  completely  restored. 

A  number  of  clinical  cases  without  necropsy,  in  which  crossed  am- 
blyopia has  been  present,  have  been  recorded  by  Ferrier  and  others.  In 
these  cases  a  series  of  other  unilateral  phenomena  have,  as  a  rule,  been 
associated  with  the  dimness  of  sight,  these  being  usually  such  as  hemi- 
anopsia, hemiana^sthesia,  partial  or  complete  impairment  of  other 
special  senses,  astereognosis,  and  hemiparesis.  Doubtless  in  not  a  few 
instances  the  partial  amblyopia  is  present  in  organic  cases  with  uni- 
lateral symptoms,  but  is  overlooked.  In  one  of  the  cases  reported  by 
Ferrier,  the  patient  was  unaware  of  the  impairment  of  vision  in  the 
eye  on  the  side  of  her  other  symptoms  until  her  attention  was  called 
to  the  fact  by  special  examination.  Xot  infrequently  one  eye  with 
perfect  or  good  vision  serves  so  well  the  purposes  of  the  individual  that 
impairment  of  sight  in  the  other  does  not  attract  attention  until  some 
incident  or  investigation  reveals  its  existence.  In  these  clinical  cases 
of  crossed  amblyopia,  marked  impairment  of  the  color  sense  is  often 
present.  In  one  case  green  w^as  the  only  color  recognized.  The  visual 
fields,  determined  with  such  sight  as  is  left,  are  greatly  contracted. 

Gowers  gives  brief  notes  of  a  case  which  is  instructive  in  connec- 
tion with  this  question  of  crossed  amblyopia.  The  patient  was  a 
woman  fifty  years  old  whose  right  eye  had  been  removed  many  years 
before.  She  had  marked  concentric  restriction  of  the  field  of  the  left 
eye  accompanied  by  amblyopia.  Accompanying  symptoms  were  left 
hemiana^sthesia,  dulness  of  the  senses  other  than  sight,  and  persistent 
headache.  The  patient  was  not  an  hysteric,  but  syphilis  was  probable. 
The  patient's  amblyopia  and  other  symptoms  improved  under  the  use 
of  iodide. 

^Vhile  these  cases  undoubtedly  show  the  possibility  of  the  occur- 
rence of  a  form  of  crossed  amblyopia  from  lesion  of  the  angulo-occipital 
region,  it  is  not  to  be  inferred  that  they  prove  that  this  region  is  the 
only  area  of  representation  of  clear  or  macular  vision.  In  the  cases 
of  Demange  and  of  Sharkey,  the  lesions  were  such  as  to  involve  the 
angulo-fjccipital  subcortex,  and  may  have  severed  the  connections  be- 
tween a  lower  and  higher  macular  centre  or  even  the  macular  bundle 
from  the  pregcniculum  to  the  primary  macular  centre.  The  now  some- 
what numerous  cases  of  precortical  and  cortical  word-blindness,  with 
or  without  letter-blindness,  have  not  shown,  or  at  least  not  in  the  rec- 
ords given,  that  clear  visual  perception  was  lost.     Such  cases  sometimes 


CORTICAL  CENTRES  107 

have  normal  acuity  of  vision  for  objects  other  than  words  and  letters 
hoth  near  and  remote,  as  in  one  case  studied  by  me  recently.  If  the 
angulo-occipital  cortex  is  the  sole  area  of  clear  or  macular  vision  in 
man,  acuity  of  vision  for  all  purposes  would  suffer  in  such  cases,  instead 
of  the  defect  being  confined  to  vision  for  words  and  letters.  The  an- 
gular gyre  is  simply  a  part  of  the  higher  visual  or  psycho-visual  area, 
the  particular  part  in  which  is  located  vision  for  the  highest  and  last 
form  of  visual  svmbolization — that  of  \ansniase. 

The  experiments  of  Ferrier  and  Yeo  and  Lannegrace  might  be 
interpreted  as  indicating  that  even  in  the  monkeys  experimented  upon 
there  is  a  higher  area  of  clear  or  macular  vision  located  in  the  position 
asserted  by  Ferrier.  These  animals,  from  destruction  of  the  angular 
<;onvolution  on  the  one  side,  had  transient  loss  of  vision  in  the  opposite 
«ye,  and  even  when  the  same  region  of  the  opposite  side  was  destroyed, 
the  total  loss  of  vision  which  resulted  was  partially  recovered  from  if 
the  animal  survived  sufficiently  long.  This  does  not  necessarily  show 
that  the  cortical  area  destroyed  was  the  only  area  for  clear  or  macular 
vision,  but,  taken  together,  the  experiments  might  be  regarded  as  show- 
ing that  lower  as  well  as  higher  macular  cortical  centres  are  present 
in  the  monkey  as  well  as  in  man.  In  the  man  these  centres,  as  the 
result  of  evolution,  become  cortical  areas  of  representation  of  designs 
-and  symbols;  in  the  monkey  they  are  probably  the  seat  of  memorial 
images  of  objects  of  small  size  and  particular  form.  The  fact  must 
also  not  be  lost  sight  of  that  in  the  experiments  upon  the  monkey,  the 
macular  bundles,  geniculo-cortical  and  intercortical,  or  both,  must 
have  been  injured  or  severed.  I  believe  in  an  angulo-occipital  macular 
area  in  the  human  being,  but  it  is  one  which  is  concerned  not  only  with 
€lear  or  macular  vision,  but  with  vision  whose  purpose  is  the  recogni- 
tion of  words,  letters  and  numbers,  and  probably  also  geometric  and 
other  designs.  The  proper  place  for  the  further  discussion  of  the  symp- 
toms which  result  from  destruction  of  this  higher  macular  area  of  the 
■cortex  is  where  word-blindness  and  other  forms  of  visual  aphasia  are 
later  considered. 

It  is  not  improbable  that  there  is  for  the  macular  field,  as  for  the 
peripheral  fields,  a  half  representation,  both  primary  or  lower  and  sec- 
ondary or  higher,  in  each  hemisphere;  in  other  words,  that  a  strictly 
limited  lesion  of  the  calcarine  cortex  on  the  one  hand  and  of  the  angular 
region  on  the  other  may  cause  blindness  in  half  of  the  macular  field 
of  the  corresponding  sides.  At  the  same  time  it  is  probable  that  thi:^ 
half  macular  representation  is  not  so  strictly  defined  by  a  vertical 
"dividing  line  as  is  the  peripheral  retinal  representation.     Putting  it  in 


10S  THE  EYE  AND  NERVOUS   SYSTEM 

another  way,  the  macular  centre  of  one  side  is  more  or  less  representa- 
tive of  the  macular  fields  of  both  sides. 

Elsewhere  ^^  I  have  recorded  two  interesting  cases  which  seem  to 
indicate  that  a  very  limited  lesion  of  the  cortical  area  for  word-vision 
or  of  the  tract  leading  to  it  from  the  lower  macular  centre,  might 
cause  a  loss  of  vision  for  one-half  of  a  word,  what  might  be  termed 
hemianopsic  amblyopia.  One-half  of  the  central  field  is  obscured 
or  lost,  the  peripheral  field  being  maintained,  or  it  may  be  partially 
obscured.  The  patient  is  half  blind  for  words.  A  word  of  more  than 
four  or  five  letters  will  only  be  half  seen,  or  at  least  will  be  partially 
cut  off,  unless  the  patient  turns  his  eyes  so  that  the  unimpaired  half 
of  the  central  field  can  take  in  different  parts  of  the  word  in  succession. 

It  is  probable  not  only  that  lower  and  higher  cortical  macular  cen- 
tres exist,  but  also  lower  and  higher  peripheral  representation  in  the 
cortex;  it  is  probable,  moreover,  that  the  lower  macular  region  is  con- 
nected with  the  higher  macular  region  by  its  own  bundle,  and  the  lower 
peripheral  portion  of  the  fields  with  the  non-macular  portion  of  the 
higher  visual  field  by  its  own  fibre  tracts.  It  follows  that  certain  symp- 
toms will  result  from  disease  of  the  lower  macular  centre  or  of  the  tracts 
going  from  it  to  the  higher  centre  of  the  same  side  and  to  that  of  the 
other  side ;  it  also  follows  that  certain  affections  will  result  from  lesion 
of  the  fibres  connecting  the  lower  and  higher  peripheral  cortical  visual 
fields.  The  macula  is  not  only  the  organ  of  acute  vision,  but  of  the 
highest  form  of  psychic  vision.  The  retinal  periphery  and  its  lower 
and  higher  cortical  areas  of  representation  are  concerned  with  percep- 
tions and  apperceptions  of  a  lower  order. 

With  the  facts  just  given  before  us,  in  order  to  apply  the  views 
advanced  to  all  clinical  macular  phenomena,  let  us  glance  for  a  moment 
at  the  different  forms  of  focal  disease,  peripheral  and  central,  giving 
macular  symptoms.  Macular  or  central  amblyopia  will  of  course  be 
produced  by  the  destruction  of  the  macula  itself,  but  lesions  of  intra- 
ocular origin  come  almost  exclusively  under  the  attention  of  the  ophthal- 
mologist. I  would,  however,  direct  attention  to  the  fact  that  the  macula 
is  the  part  of  the  retina  which  is  chiefly  concerned  with  the  recogni- 
tion of  words,  letters  and  numbers.  Complete  destruction  of  a  large 
portion  of  the  macula  produces  what  might  be  termed,  for  convenience, 
a  peripheral  alexia  and  visual  agraphia,  while  at  the  same  time  very 
considerable  peripheral  vision  for  form  remains;  in  other  words,  the 


"Mills,   The    Nervous    System   and    its    Diseases, — Diseases    of    the    Brain    and 
Cranial  Nerves,  p.  760.    J.  B.  Lippincott  Company,  Philadelphia. 


COKTICAL  CENTRES  109 

patient  suffering  from  a  large  degree  of  macular  destruction  will  still 
be  able  to  orient  himself  with  his  retinal  periphery  and  also  recognize 
natural  objects,  individuals,  buildings,  and,  to  a  considerable  extent, 
designs  and  structures  of  various  sorts. 

Central  amblyopia  results  from  a  small  infiltrating  lesion,  a  nodule 
of  sclerosis  or  a  focus  of  hemorrhage,  for  instance,  which  may  by  rare 
chance  be  so  situated  as  to  destroy  the  macular  bundle  alone  in  the  optic 
nerve  and  tracts  in  any  part  of  its  course.  Clinical  experience  has 
shown  that  this  bundle  is  especially  liable  to  be  affected  by  certain 
toxic  agents. 

As  the  fibres  of  the  macular  bundle  are  undoubtedly  distributed  to 
the  pregeniculum,  complete  destruction  of  this  body  or  of  a  special 
portion  of  it,  should  cause,  central  amblyopia.  This  central  amblyopia 
would  be  of  the  crossed  variety,  and  if  only  one  external  geniculate  body 
were  destroyed  it  would  be  transient,  or  would  at  least  be  recovered  from 
in  part.  It  is  not  yet  positively  proved  that  the  pregeniculum  is  the 
basal  centre  for  the  macular  bundle  alone,  as  is  held  by  Knies  ^®  and 
others.  Some  facts  point  otherwise,  as  for  instance  the  case  reported 
by  Henschen,  which  seemed  to  show  that  hemianopsia  in  the  lower  quad- 
rant may  be  caused  by  a  lesion  of  the  dorsal  part  of  the  pregeniculum. 
In  any  case,  if  the  periphery  of  the  retina  is  represented  in  the  pre- 
geniculum the  macula  is  also  represented  in  this  ganglion,  and  if  the 
terminus  of  the  macular  bundle  is  involved  in  a  completely  destructive 
lesion,  crossed  amblyopia,  as  above  indicated,  would  result.  Partial 
lesions  of  the  basal  macular  centre,  on  one  side  or  both,  cause  scotomata 
which  may  be  transient  or  disregarded  if  the  lesion  is  unilateral,  or  per- 
sist in  consciousness  if  bilateral  geniculate  lesions  are  present.  Para- 
central scotomata,  peripheral  vision,  and  central  vision  in  the  main 
remaining  intact,  should  result  from  lesions  which  only  partially  de- 
stroy the  macular  geniculate  centre.  Such  a  clinical  case  has  been 
recorded  by  Knies.  In  this  case  diminution  of  the  hemianopsic  pupil- 
lary response  was  present,  that  is,  light  thrown  on  one  side  of  the  retina 
caused  a  prompt  iritic  response,  while  on  the  other  side  the  reflex  was 
sluggish.  A  form  of  dyslexia  apparently  due  to  the  impairment  of 
macular  vision  was  present  in  this  case,  which  would  seem  to  bear  out 
the  view  that  the  macula  is  the  part  of  the  retina  chiefly  concerned  with 
word-  and  letter-vision. 

Leaving  the  basal  centres,  a  lesion  of  the  macular  bundle  in  the  optic 


**   Knies.  The  Relation  of  the  Eye  and  its  Diseases  to  the  Diseases  of  the  Body, 
New  York.   1894. 


no  THE   EYE   AND   NERVOUS   SYSTEM 

radiations  should  cause  some  degree  of  central  amblyopia ;  this  would 
be  transient  and  more  or  less  partial  according  to  the  degree  of  involve- 
ment of  the  fibres  constituting  this  bundle.  Here,  as  in  the  case  of  the 
pregeniculum  and  the  optic  tract,  small  destructive  lesions  would  be 
represented  symptomatically  by  scotomata.  Bilateral  lesions  of  the  oc- 
cipital macular  bundle  would  cause  permanent  amblyopia.  In  all  the 
cases  which  have  been  considered,  the  amblyopia  would  be  of  the  crossed 
variety  from  unilateral  lesion,  and  in  all  probability  close  examination 
would  show  some  impairment  of  vision  with  contraction  of  the  field  on 
the  side  of  the  lesion. 

In  our  consideration  of  this  subject  we  have  now  reached  the  macu- 
lar centre  in  the  primary  or  lower  cortical  visual  field.  Presuming 
the  existence  of  this  centre,  have  we  any  clinical  evidence  in  support 
of  this  view  ?  As  has  already  been  stated,  its  existence  is  predicated 
by  the  occurrence  of  hemianopsia  less  a  small  central  portion  of  the 
half  field  near  the  fixation  point  from  a  cuneo-calcarine  lesion,  and  it 
is  made  still  more  certain  by  those  cases  of  double  hemianopsia  in  which 
a  small  central  area  of  acute  vision  remains.  Cases  which  suggest  a 
lower  macular  representation  are  those  in  which  outer  portions  of  the 
retinal  half  field  retain  vision,  while  the  rest  of  the  half  field  to  the 
dividing  line  is  obscured.  Here  again  also  the  question  of  paracentral 
and  central  scotomata  becomes  of  interest.  A  lesion  partially  destruc- 
tive of  the  cortical  macular  centre  of  one  side  should  cause  a  scotoma, 
paracentral  or  central  according  to  its  position.  This  would  doubt- 
less be  transient  or  disregarded,  but  might  be  elicited  by  special  inves- 
tigation. The  cortical  macular  centre  of  the  opposite  side  would  serve 
the  i)urposes  of  vision,  especially  if  the  nasal  macular  centres  were  intact. 
Bilateral  disease  of  the  cortical  centres  for  the  macula  causes  persistent 
scotomata,  or  central  amblyopia  if  the  lesions  completely  destroy  the  cen- 
tres on  both  sides.  Higher  central  vision,  that  for  letters  and  words, 
for  instance,  would  necessarily  go  with  the  partial  or  complete  destruc- 
tion of  the  i)rimary  visual  centres,  although,  as  has  been  indicated,  the 
reverse  is  not  the  case.  Word-blindness  or  letter-blindness  may  be 
present  without  destruction  of  central  visual  acuity  for  other  purposes 
than  the  recognition  of  language  symbols,  but  a  lesion  or  lesions  destroy- 
ing primary  central  perception  would  necessarily  disturb  or  destroy  cen- 
tral apperception. 

The  manner  in  which  hemianopsia  from  cortical  or  cortico-subcor- 
tical  lesion  sometimes  clears  up  or  rather  diminishes,  indicates  how  a 
central  or  paracentral  scotoma  might  be  produced  from  a  lesion  limited 
to  the  lower  cortical  macular  centre  of  one  side.     Hemianopsia  in  some 


CORTICAL  CENTRES  111 

cases  diminishes  with  comparative  uniformity  from  centre  towards 
periphery,  the  region  of  visual  perception  gradually  expanding.  In 
other  cases  it  may  clear  in  a  lower  or  upper  quadrant,  or  in  an  irregular 
area  in  any  portion  of  the  hemianopsic  field.  In  still  others,  however, 
an  irregular  scotoma  may  remain  as  the  field  clears  peripherally.  In  a 
case  of  this  kind  a  portion  of  the  cortical  macular  field  has  been  com- 
l^letely  destroyed,  the  same  being  true  of  that  portion  of  the  peripheral 
cortical  area  farthest  removed  from  the  macular  centre.  An  inter- 
vening area  of  both  central  and  peripheral  representation  has  escaped 
complete  destruction. 

As  already  indicated,  I  believe  that  a  distinct  bundle  passes  from 
both  primary  cortical  macular  centres  to  the  higher  centre  for  clear  vis- 
ion on  each  side.  The  tract  from  the  lower  centre  of  the  same  side  prob- 
ably passes  by  way  of  the  occipito-parietal  subcortex,  while  the  tract 
from  the  other  side  crosses  through  the  splenium  of  the  callosum  to  con- 
join with  it,  the  conjoined  tracts  going  to  the  angular  convolution.  A 
lesion  of  this  conjoined  tract  or  lesions  of  the  tracts  from  both  primary 
centres  before  they  unite  would,  of  course,  cause  disturbance  or  loss  oi 
the  functions  of  the  higher  centre  for  which  they  are  destined  ;  in  other 
words,  as  will  be  shown  more  fully  later,  if  the  precortical  lesions  were 
left-sided,  a  variety  of  word-blindness  would  occur.  Doubtless  also  close 
examination  in  such  cases  would  show,  at  least  at  first,  some  impairment 
of  vision  in  the  eye  of  the  side  opposite  to  the  lesion,  this  being  quickly 
recovered  from  because  both  eyes  are  represented  in  each  macular  centre. 

Recently,  in  conjunction  with  Dr.  C.  D.  Camp  "*"  I  have  published 
the  history  of  a  case  of  unusual  interest  in  connection  with  the  subject 
of  crossed  amblyopia,  and  also  of  that  of  visual  hallucinations  the  result 
of  organic  disease  of  the  cerebral  cortex  and  subcortex.  The  patient,  a 
woman  sixty-three  years  old,  had  suffered  with  impairment  of  vision  in 
both  eyes  fur  nearly  two  years  before  coming  under  observation.  This 
dimness  of  vision  was  attributed  to  glaucoma  for  which  a  double  iridec- 
tomy was  performed  some  months  before  coming  under  observation. 
After  this  operation  her  vision  was  fairly  good.  I  am  indebted  to  Dr. 
G.  C.  Harlan,  who  performed  the  operation  for  the  relief  of  the  glau- 
coma, for  notes  regarding  the  state  of  the  patient's  vision  and  eyes  at 
this  time.  Sight  was  reduced  to  20/40  in  the  right  eye  and  20/50  in 
the  left  and  the  disc  of  each  eye  was  cupped  to  the  extent  of  about  1.50 
D.  The  fields  were  practically  normal  with  the  exception  of  a  slight 
peripheral  cut  in  the  right  eye. 

"Mills  and  Camp,  Trans,  of  the  American  Medico-Psycholopfical  Association,  vol. 
xii.  1905;  also  Anier.  Jour,  of  Insanity,  1905. 


112  THE    EYE    AND    NERVOUS    SYSTEM 

I  saw  this  patient  first  in  September,  1904.  A  few  days  before  my 
visit  she  had  a  cerebral  seizure  in  which  the  right  eye  became  totally 
blind  and  vision  in  the  left  eye  was  greatly  impaired.  She  had  also  lost 
the  power  of  standing  and  was  abnormally  excitable. 

She  was  brought  to  Philadelphia  where  she  was  attended  until  her 
death  by  Dr.  Harlan  and  the  writer.  A  careful  examination  of  her  eyes 
by  Dr.  Harlan  showed  that  in  the  right  eye  she  had  scarcely  more  than 
light  perception.  The  cupped  nerve  had  filled  up  to  the  level  of  the 
retina.  In  the  left  eye  vision  was  reduced  to  5/40 ;  the  field  was  periph- 
erally contracted  to  40  degrees  on  the  nasal  and  to  20  degrees  on  the 
temporal  side.  The  disc  was  cupped  but  the  fundus  was  otherwise  nor- 
mal. Later  this  patient  developed  acute  delirious  mania  with  vivid  and 
varying  hallucinations  of  sight,  which  are  described  in  some  detail  in 
the  paper  to  which  reference  has  been  made. 

The  patient  died  about  three  months  after  her  sudden  attack  of  loss 
of  vision. 

Macroscopic  examination  showed  the  arteries  at  the  base  of  the  brain 
highly  sclerotic  and  both  pregeniculae  atrophied. 

In  sections  from  the  calcarine  fissure  from  each  side  were  seen  areas 
of  intense  congestion,  with  the  formation  of  numerous  very  fine  new 
capillaries.  They  were  mostly  in  the  second  and  third  layers  of  the  cor- 
tex, and  the  pyramidal  cells  in  their  vicinity  appeared  degenerated  on 
examination  by  the  Nissl  method.  Sections  from  the  cortex  of  the  lat- 
eral surface  of  each  occipital  lobe,  near  the  occipital  pole,  showed  areas 
not  involving  the  whole  section  of  a  similar  vascular  appearance,  as  in 
the  calcarine  fissure,  and  probably  a  part  of  the  same  pathological  pro- 
cess. The  nerve  cells  in  the  location  of  these  areas  were  of  the  pyra- 
midal variety,  but  they  had  lost  their  normal  shape  and  structure  com- 
pletely. The  white  substance  beneath  these  areas  appeared  degenerated 
by  the  Weigert-hffimatoxylin  method,  and  full  of  small  holes  as  though 
nerve  fibres  had  dropped  out. 

The  left  optic  nerve  was  about  one-third  of  the  normal  size  of  an 
optic  nerve,  and  surrounded  by  a  greatly  thickened  sheath.  There  was 
much  connective  tissue  overgrowth  between  the  nerve  bundles,  which 
were  small  and  degenerated.  The  chiasm  appeared  to  be  degenerated 
by  the  Weigert-hjematoxylin  method,  but  there  was  no  round  cell  infiltra- 
tion about  it. 

WHiether  the  complete  blindness  in  one  eye  and  the  serious  loss  of 
vision  in  the  other  were  to  be  regarded  as  constituting  a  genuine  case 
of  crossed  amblyopia  such  as  has  been  reported  by  Ferrier  and  Gowers, 
and  attributed  to  lesion  of  the  angulo-occipital  region  or  of  the  macular 


CORTICAX  CENTRES  113 

bundle,  may  perhaps  be  regarded  as  doubtful.  If  due  to  the  lesions  un- 
doubtedly present  in  the  calcarine  cortex,  this  crossed  amblyopia  is  of 
much  interest  in  connection  with  the  question  of  the  existence  of  a  sepa- 
rate macular  representation  in  the  primary  or  lower  cortical  visual  cen- 
tres of  the  calcarine  region  and  in  the  higher  angulo-occipital  visual 
areas.  Dr.  Harlan  agreed  with  me  that  the  final  amblyopia  in  this  case 
could  not  be  attributed  to  the  preceding  glaucoma,  at  least  not  to  the 
peripheral  conditions  which  Avere  present.  With  the  exception  of  the 
cupped  nerves,  the  fundus  of  each  eye  was  normal  and  the  filling  up  of 
the  nerve  cup  seemed  to  indicate  the  occurrence  of  some  intracranial 
lesion.  The  attacks  of  sudden  loss  of  vision  which  occur  in  chronic  glau- 
coma may,  in  some  instances,  be  due  to  intracranial  lesions  affecting  the 
cerebral,  and  especially  the  macular,  bundles  or  centres.  It  is  possible 
of  course  that  the  atrophy  of  the  left  optic  nerve  and  tract  may  have  ac- 
counted for  the  great  loss  of  acuity  of  vision  and  contraction  of  the  vis- 
ual field 'in  the  left  eye,  but  in  any  case  the  sudden  amblyopia  in  the 
right  eye  was  doubtless  due  to  a  sudden  vascular  lesion  in  the  left  hemi- 
sphere. 

The  phenomena  of  hysterical  unilateral  amblyopia  would  seem,  when 
considered  carefully,  to  confirm  the  theories  held  regarding  crossed  am- 
blyopia as  the  result  of  a  unilateral  organic  lesion.  In  unilateral  am- 
blyopia of  hysterical  origin,  which  is  usually  but  not  invariably  asso- 
ciated with  unilateral  impairment  of  other  senses,  as  of  touch,  pain, 
temperature,  hearing,  smell  and  taste,  the  vision  is  greatly  impaired  on 
the  affected  side.  Examination  shows  in  addition  concentric  contrac- 
tion of  the  field  on  this  side.  At  the  same  time,  some  dimness  of  vision 
and  less  contraction  of  the  field  may  be  present  on  the  other  side.  The 
same  is  true  with  regard  to  color  phenomena.  Dyschromatopsia  may 
be  marked  on  the  affected  side,  and  affected  to  a  less  and  perhaps  a  slight 
degree  on  the  other.  These  phenomena  are  the  counterpart  of  what  has 
occurred  in  the  recorded  cases  of  crossed  amblyopia  from  organic  lesion, 
the  differences,  if  any,  being  in  degree  as  to  the  impairment  of  vision, 
and  also  in  the  fact  that  the  hysterical  affection  is  usually  transient, 
while  the  other  is  permanent  or  at  least  much  more  persistent. 

"Whatever  theory  may  be  accepted  in  explanation  of  the  hysterical 
amblyopia,  it  is  clear  that  the  affection  is  in  some  way  dependent  upon 
interference  with  the  functions  of  the  visual  centres  of  one  hemicere- 
brum.  ^^^lethe^  this  is  in  the  nature  of  an  angiospasm  of  cortical  vessels, 
as  once  held,  or  of  a  temporary  impairment  of  nutrition  of  the  cortical 
cells,  the  fact  remains  that  the  symptoms  point  to  a  unilateral  cerebral 
disturbance.  It  is  significant  that  in  hysterical  eases  hemianopsia  in 
8 


114  THE    EYE    AND    NERVOUS    SYSTEM 

any  of  its  varieties  is  not  present,  but  that  the  loss  of  vision  is  central  or 
general  for  the  side  affected.  This  is  similar  to  what  occurs  in  the  ex- 
perimental lesions  produced  by  destruction  experiments  upon  the  mon- 
kev,  or  in  cases  like  those  of  Sharkey,  Demange  and  others,  in  which  in 
the  human  subject  cortical  or  subcortical  substance  has  been  destroyed. 
All  the  cases,  organic  or  functional  in  man  and  organic  in  the  lower 
animals,  show  the  possibility  of  the  occurrence  of  a  crossed  affection  of 
vision  distinct  from  that  which  gives  anopsias  or  hemianopsias  for  light, 
color  and  form.  It  is  true  that  the  recorded  cases  of  crossed  amblyopia 
are  few  in  number,  but  as  Ferrier  suggests,  and  as  one  or  two  of  his  clin- 
ical cases  seem  to  demonstrate,  the  amblyopia  in  some  instances  may  be 
overlooked.  Whenever  a  patient  presents  such  symptoms  as  hemianes- 
thesia and  astereognosis,  the  case  should  be  examined  for  unilateral  dis- 
turbance of  vision  and  of  the  other  senses.  The  investigator  should  not 
be  satisfied  with  demonstrating  that  hemianopsia  is  not  present,  or  even 
that  some  central  vision  is  retained  in  both  eyes,  but  should  examine  not 
only  for  the  presence  of  amblyopia,  but  for  the  degree  of  the  retention 
or  loss  of  central  vision  in  both  eyes. 

The  visual  aura3  of  epilepsy,  like  the  spectra  of  migraine,  have  been 
considered  clinically  in  the  present  volume  by  Dr.  Lloyd.  I  shall  only 
direct  brief  attention  to  the  well-known  facts  which  indicate  that  these 
aura;  and  spectra  are  usually  the  evidences  of  cortical  disease,  indicating 
either  discharge  or  inhibition  or  both  of  the  cortical  centres  for  sight, 
primary  and  secondary.  These  aur£E  take  the  form  of  either  simple  sen- 
sations of  luminosity,  of  colors,  or  of  persons  or  objects  or  of  groupings 
of  these. 

In  many  instances  the  primary  visual  phenomenon  is  a  light  sensa- 
tion, as  a  flash  or  a  ball  of  fire.  In  other  cases  the  aura  is  a  sensa- 
tion of  color  which  often  assumes  some  definite  form,  as  that  of  a  ball  or 
a  star.  It  is  significant  that  the  colors  seen  are  those  which  are  recog- 
nized by  most  authorities  as  primary  colors,  as  red,  blue,  green  or  yel- 
low, or,  in  rare  cases,  purple  or  violet.  In  the  recorded  cases  red  and 
blue  have  been  most  frequently  present,  sometimes  alone,  sometimes  in 
combination.  One  or  the  other  has  been  almost  invariably  present. 
Aura?  representing  visual  ideas  are  comparatively  infrequent,  but  have 
nevertheless  been  observed  in  considerable  number.  These  in  particu- 
lar cases  have  taken  the  appearance  of  an  individual  in  a  certain  colored 
dress,  as  for  instance,  a  little  old  woman  in  brown.  The  primary  sensa- 
tion of  light  or  the  sensation  of  color  in  a  few  cases,  has  preceded  in  the 
same  seizure  that  of  some  objects  representing  a  visual  idea ;  and  Hugh- 
lings  Jackson  has  explained  this  sequence  of  events  by  the  discharge  of 


VISUAL  APHASIAS  115 

primary  visual  centres  followed  by  that  of  secondary  or  higher  visual 
centres.     The  latter,  however,  may  take  place  alone. 

Some  of  the  most  carefully  recorded  observations  regarding  the  spec- 
tra of  migraine  show  that  these,  whatever  form  they  may  take,  tend  to 
occupy  only  the  peripheral  portion  of  the  retinal  field.  The  central  field 
is  nearly  always  free  from  these  spectra,  and  the  zigzag  or  other  lines 
diminish  in  length  as  they  approach  it ;  but  this  central  field  is  subject 
on  the  other  hand  to  inhibition,  becoming  obscured  or  absolutely  dark- 
ened. 

It  is  only  necessary  in  this  connection  to  emphasize  that  the  clinical 
phenomena  thus  recorded  clearly  indicate  in  the  vast  majority  of  cases 
that  the  cause  of  the  phenomena  is  the  discharge  of  centres  and  areas  of 
the  cortex  which  either  act  independently  or  in  more  or  less  frequent  as- 
sociation. Inhibition  of  cortical  visual  centres  is  indicated  by  the  dimi- 
nution or  loss  of  sight  which  occurs  as  the  sequence  of  luminous  and 
chromatic  sensations. 

THE  VISUAL  PORTION  OF  THE  CEREBRAL  ZONE 
OF   SPEECH   AND    VISUAL   APHASIAS. 

Under  the  general  designation  visual  aphasia  are  included  all  those 
forms  of  interference  with  speech  and  with  writing  which  result  from 
lesion  or  disturbance  of  the  visual  centres  concerned  with  language  and 
the  tract  associating  these  centres  with  lower,  co-ordinate,  and  higher  cer- 
ebral centres  and  areas.  While  our  concern  is  chifefly  with  the  visual 
aphasias  and  the  visual  subdivision  of  the  general  zone  of  language  in  the 
cerebrum,  a  glance  at  this  zone  in  its  entirety  forms  a  proper  introduc- 
tion to  the  discussion  of  those  parts  of  it  which  relate  to  speech  as  it  is 
understood  through  sight.*** 

The  cerebral  zone  of  speech  includes  an  extensive  cortical  region, 
probably  not  less  than  one-third  of  the  cortical  expanse  of  the  lateral  as- 
pect of  the  left  hemicerebrum,  as  shown  in  the  shaded  portion  of  Fig. 
17.  While  for  descriptive  purposes  it  is  best  to  designate  it  as  a  cortical 
area,  it  must  be  understood  that  both  the  cortex  and  the  sub-cortex  of 
the  entire  region  enter  into  its  formation. 

Within  this  great  zone  are  several  important  centres  or  sub-areas  con- 
cerned with  particular  attributes  of  language.  In  Fig.  17  these  centres 
are  WH,  a  centre  for  word-hearing,  the  auditory  portion  of  the  zone  of 


*'  A  sketch  of  the  cerebral  zone  of  speech  given  in  the  text  and  the  diagram 
illustrating  it  are  taken  from  the  article  by  me  published  in  the  American  Journal 
of  the  Medical  Sciences,  September,  1904,  the  text  of  this  article  having  been 
adapted  to  the  present  chapter. 


116  THE    EYE    AND    NERVOUS    SYSTEM 

language ;  at  S  and  S  a  great  centre  for  speech,  often  called  motor ;  at 
WS,  LS,  NS,  a  visual  subarea  concerned  with  the  representation  of 
words,  letters  and  numbers ;  at  W  a  graphic  or  writing  centre,  usually 
spoken  of  as  the  motorgraphic  centre;  and  at  N  a  naming  centre  in 
which  it  is  believed  by  those  who  hold  to  its  existence  that  names  are  cor- 
related with  the  concrete  concepts  of  the  objects  named. 

Briefly  stated,  the  auditive  centre  for  speech  is  situated  at  the  junc- 
tion of  the  first  and  second  temporal  gyres  about  opposite  the  point 
where  the  horizontal  branch  of  the  Sylvian  fissure  turns  upward  pos- 
teriorly;   the  dynamic  or  motor  speech  centre  occupies  the  hinder  por- 


FiG.  17.— Cerebral  zone  of  language.  H',  writing  centre ;  S,  S,  speech  centre  (language  arrangement) 
for  emission ;  117/,  word-hearing  or  auditory  centre ;  WS,  word-seeing ;  LS,  letter-seeing ;  AS,  number- 
seeing  ;  N,  naming-centre ;  PS,  PS,  PS,  higher  psychic  region ;  WM,  centre  for  movements  of  upper 
extremity  concerned  in  writing ;  S.V,  centre  for  movements  concerned  in  speaking ;  OS,  OS,  OS,  area 
of  object-seeing. 

tion  of  the  subfrontal  or  third  frontal  convolution,  and,  according  to  the 
view  of  the  writer,  the  insula  or  at  least  its  anterior  half;  the  visual 
centres  immediately  concerned  with  speech  are  located  in  the  angular 
gyre;  the  site  of  the  graphic  or  writing  centre  is  the  caudal  third  of 
the  second  frontal  convolution,  and  the  naming  centre  is  probably  situ- 
ated in  the  third  temporal  convolution. 

Connecting  these  centres  with  each  other,  and  also  with  other  por- 
tions of  the  brain,  are  subcortical  tracts  composed  of  the  processes  of  the 
cell  bodies  which  are  located  in  the  cortex  and  of  their  fibrillary  con- 
stituents. These  associating  tracts  are  of  a  complicated  kind,  although 
their  number  and  arrangement  are  capable  of  analysis  if  sufiicient  time 


VISUAL  APHASIAS  117 

and  study  are  given  to  the  matter.  Only  one  or  two  points  regarding 
them  need  here  be  emphasized, — namely,  that  the  different  centres  are 
connected  each  with  the  other  by  a  system  of  to-and-fro  association,  so 
that  the  impulses  from  special  areas  probably  pass  in  both  directions. 

Surrounding  the  zone  of  language  on  all  sides  are  centres  and  areas 
which  have  important  relations  with  the  function  of  speech.  These  are 
of  at  least  three  kinds,  and  an  understanding  of  them  may  be  reached 
by  a  study  of  Fig.  17.  On  the  sensory  or  receptive  side  of  the  brain  are 
areas  or  centres  concerned  with  such  functions  as  object-seeing  and  ob- 
ject-hearing. Objects  which  are  not  recognized  cannot  of  course  be 
named,  and  these  centres  for  object  recognition  therefore  are  neces- 
sarily contributors  to  the  sensory  concept  side  of  the  zone  of  language. 
They  are  represented  in  Fig.  17  at  the  points  marked  OS.  On  the  mo- 
tor or  emissive  side  of  the  cerebral  speech  zone  are  centres  at  SM  and 
WM  which  are  concerned  with  the  execution  of  the  work  prepared  for 
them  by  the  speech  and  writing  centres.  They  are,  in  other  words,  the 
centres  of  representation  of  movements  of  articulation  and  enunciation, 
and  of  the  movements  of  the  hand  and  upper  extremity  generally.  At 
PS  in  the  prefrontal  portion  of  the  brain  is  a  region  where  the  highest 
psychical  functions  are  especially  represented.  It  is  evident  that  in  using 
language,  attention,  volition,  and  reasoning  play  a  part,  although  this 
part  may  vary  within  wide  limits.  This  higher  psychical  region,  there- 
fore, has  its  particular  relation  to  the  ::one  of  language,  and  especially 
to  its  dynamic  side. 

Let  us  now  concentrate  our  attention  on  that  subdivision  of  the  cer- 
ebral zone  of  speech  which  is  especially  concerned  with  visual  language, 
its  lesions  causing  the  different  varieties  of  visual  aphasia.  As  indi- 
cated in  what  has  just  been  said,  at  WS,  LS,  and  NS  in  Fig.  17  is  situ- 
ated the  cortical  centre  for  one  of  the  forms  of  higher  visualization,  the 
centre  or  region  in  which  are  stored  the  visual  images  of  words,  letters 
and  numbers.  This  portion  of  the  cortex  is,  as  has  already  been  shown, 
one  of  the  subdivisions  of  the  concrete  memory  field.  It  is  connected  on 
the  one  hand  with  the  lower  or  primary  cortical  areas  for  vision  in  both 
hemispheres ;  and  on  the  other,  with  the  areas  for  speech,  writing  and 
movements  in  the  cortical  zone  cephalad  of  the  central  fissure,  while  at 
the  same  time  it  has  numerous  associating  tracts  with  centres — contrib- 
utory or  co-ordinate  in  function — like  those  for  object-seeing,  for  stere- 
ognostic  perception,  for  hearing,  taste  and  smell.  It  is  also  connected 
with  the  special  centre  or  area  (N),  whose  chief  function  is  the  associa- 
tion of  names  with  objects,  concepts  of  which  are  obtained  through  the 
special  senses.    If  the  reader  has  a  clear  idea  of  the  location  and  connec- 


118  THE    EYE    AND    NERVOUS    SYSTEM 

tioiis  of  this  higher  portion  of  the  visual  area,  which  is  usually  spoken 
of  as  the  area  for  word-seeing,  he  can  readily  comprehend  the  different 
varieties  of  aphasia  which  result  when  this  centre,  its  outlets  or  inlets, 
or  its  associated  or  associating  structures  are  diseased. 

The  most  important  varieties  of  visual  aphasia  and  allied  disorders 
are:  (1)  mind-  or  object-blindness;  (2)  word-blindness;  (3)  letter- 
blindness;  (4)  number-blindness;  (5)  alexia,  paralexia  and  dyslexia; 
(6)  visual  agraphia;  paragraphia  and  dysgraphia;  (7)  optic  aphasia 
or  word-dumbness.  Some  objection  might  be  made  to  including  mind- 
blindness  in  the  category  of  visual  aphasias,  and  such  objection  is  doubt- 
less valid,  but  as  a  matter  of  convenience  and  with  some  scientific  basis, 
T  have  thought  it  well  to  include  mind-blindness  under  the  general  head 
of  visual  aphasias.  The  patient  who  is  unable  through  sight  to  recog- 
nize a  familiar  person  or  object,  or  to  recall  the  visual  memories  of  such 
person  or  object,  cannot  of  course  name  that  of  which  he  is  not  cogni- 
zant, and  hence  is  aphasic  in  this  indirect  sense.  Psychic  blindness  is, 
however,  a  disorder  distinct  in  most  respects  from  visual  aphasia,  and  it 
will  be  therefore  understood  that  it  is  only  included  here  because  of  con- 
venience, and  because  it  is  so  frequently  associated  with  the  true  cerebral 
sensory  disorders  of  speech. 

MIND-BLINDNESS. 

!Mind-blindness  is  that  symptom  or  condition  in  which  the  indi- 
vidual, because  of  cerebral  lesion  or  disturbance,  is  unable  to  recognize 
by  sight  once  familiar  objects.  Old  visual  memories  of  the  kind  re- 
quired for  recognition  of  particular  objects  are  no  longer  recalled. 

In  order  that  mind-blindness  shall  result,  it  is  necessary  that  the 
disease  shall  be  situated  either  in  the  cerebral  cortex  or  in  the  tracts 
which  associate  certain  parts  of  the  cortex.  Such  disease  being  present, 
all  other  parts  of  the  intracranial  apparatus  may  be  normal,  and  yet 
the  patient  will  exhibit  this  symptom. 

Numerous  synonyms  of  mind-blindness  are  used  and  serve  to  a  cer- 
tain extent  to  confuse  the  subject.  The  most  important  of  these  are 
soul-blindness,  psychic  blindness,  and  object-blindness.  Visual  apraxia, 
to  which  brief  reference  will  presently  be  made,  is  still  another  syno- 
nym, and  Wyllie  has  suggested  the  term  visual  pragmatagnosia,  from 
the  Greek  r^fmyim^  an  object ;  and  ayvtoaia^  a  not  knowing.  It  is  pos- 
sible to  have  lost  the  power  of  recognizing  an  object  at  sight  and  yet 
be  able  to  recall  the  appearance  of  the  object;  or  both  the  ability  to 
recognize  the  object  at  sight  and  to  recall  its  image  may  be  lost  together. 
"Wyllie  has  suggested  that  the  term  visual  pragmatamnesia  be  applied 


MIND-BLINDNESS  119 

to  the  loss  of  the  power  of  calling  up  in  memory  the  appearance  of  an 
object.  A  little  consideration  will  show  that  in  the  difference  between 
visual  pragiuatagnosia  and  visual  pragmatamnesia  we  have  a  distinction 
similar  to  that  which  is  drawn  between  cortical  word-blindness  and  pure 
word-blindness,  or  between  true  color-blindness  from  cerebral  disease 
and  cerebral  color  amnesia.  When  the  patient  has  lost  the  power  of 
recognizing  an  object  by  sight,  but  retains  the  ability  of  recalling  it  as 
a  visual  memory,  the  cortex  concerned  with  the  function  has  escaped 
disease,  but  the  tracts  associating  it  with  the  lower  or  primary  centres 
for  vision  are  destroyed ;  just  as  in  cortical  word-blindness  the  visual 
memories  of  words  cannot  be  recalled,  whereas  in  the  precortical,  or 
so-called  pure  word-blindness,  the  visual  memories  can  be  recalled,  but 
the  individual  is  not  able  to  recognize  words  through  vision. 

The  term  mind-blindness  or  object-blindness  might  in  a  general  sense 
be  made  to  cover  word-blindness,  letter-blindness  and  number-blindness ; 
indeed  these  are  but  varieties  of  a  general  object-blindness.  As  our 
knowledge  of  cerebral  function  and  cerebral  disease  increases,  it  is  prob- 
able that  we  shall  be  able  to  classify  mind-blindness  into  a  number  of 
sub-types.  The  uncivilized,  like  the  higher  forms  of  lower  animal  life, 
have  keen  powers  of  recognizing  natural  objects  and  the  living  beings 
around  them.  As  evolution  advances,  added  powers  of  recognition  are 
developed.  Doubtless  some  of  these,  like  the  ability  to  recognize  colors 
with  their  various  shades  and  tones,  artificial  designs  and  geometric 
forms  have  their  special  regions  of  representation  in  the  general  area  of 
•object  seeing ;  and  disease  limited  to  such  special  areas  or  centres  gives 
rise  to  special  types  of  mind-blindness.  The  area  concerned  with  the 
recognition  of  w^ords  and  letters  is  not  the  only  one  developed  in  the 
educated  or  trained  human  being,  but  it  and  another  sub-area  of  large 
extent  concerned  with  the  recognition  of  persons,  places  and  objects,  nat- 
ural and  artificial,  are  all  that  have  so  far  been  differentiated.  It  is  for 
the  reasons  here  indicated  that  I  have  subdivided  the  area  for  object  see- 
ing as  shown  in  Fig.  3  and  Fig.  5.  While  mind-blindness  in  its  known 
forms  is  often  associated  with  word-  and  letter-blindness  and  other  forms 
of  visual  impairment  or  loss,  this  is  not  always  the  case ;  and  in  a  few 
recorded  instances  the  inability  to  recognize  faces  or  persons  has  been 
greater  than  that  for  objects  of  various  sorts.  A  woman  in  advanced 
years  under  my  care,  who  as  the  result  of  one  or  more  cerebral  seizures, 
suffered  from  word-blindness,  mind-blindness  and  other  often  associated 
phenomena,  was  entirely  unable  to  recognize  her  own  sons  by  sight,  al- 
though she  was  largely  able  to  recognize  her  surroundings  and  orient 
lierself  with  regard  to  them. 


120  THE   EYE   AND   NERVOUS   SYSTEM 

The  term  apraxia  introduced  by  Kussmaul,  is  not  exactly  synony- 
mous with  mind-blindness  or  soul-blindness,  but  includes  this  affection 
and  more.  As  defined  by  Kussmaul,  apraxia  is  the  loss  of  the  memory 
of  the  uses  of  things  and  the  understanding  of  the  signs  by  which  things 
are  expressed.  Usually  these  are  matters  of  visual  memory  and  the  signs 
usually  become  known  to  us  through  sight,  hence  mind-blindness  and 
apraxia  in  such  cases  signify  the  same  thing;  but  the  patient  may  be 
apraxic  through  other  senses  than  sight ;  if,  for  instance,  he  is  unable  to 
recall  through  hearing  the  nature  of  the  object  or  objects  which  emit  the 
sound,  he  is  mind-  or  soul-deaf, — that  is,  he  is  apraxic  through  the  sense 
of  hearing.  The  rustling  of  leaves  or  the  sound  of  a  waterfall,  or  the 
notes  of  a  bird  may  fail  to  recall  the  objects  producing  the  sound,  just  as 
in  the  word-deaf,  the  sounds  of  words  convey  no  meaning  and  in  the 
music-deaf,  musical  tones  are  unrecognized  as  such.  Tactile  apraxia  is 
exliibited  when  the  patient  is  unable  to  recognize  the  nature  and  use  of 
an  object  through  touch.  In  this  section  we  are  concerned  with  apraxia 
only  so  far  as  it  relates  to  sight. 

Burr  ^^  reports  the  case  of  a  woman  sixty  years  old  who,  while  sit- 
ting at  the  table,  suddenly  lost  vision.  This  patient,  when  examined  by 
Burr,  was  mind-blind,  and  in  addition  she  failed  to  recognize  objects 
with  the  hands.  She  could  not,  for  instance,  tell  what  a  watch  was  when 
placed  in  her  hands,  and  she  could  not  feed  herself  because  of  her  in- 
ability to  recognize  a  spoon,  knife  and  fork.  She  seemed  to  recognize 
the  hand  of  another  person  when  it  was  grasped  and  also  a  few  other 
objects.  Here  the  apraxia  was  through  touch  and  perhaps  stereognostic 
perception  as  well  as  sight.  Weir  Mitchell  ^^  had  recorded  a  similar 
case  in  1892. 

What  has  just  been  said  regarding  apraxia  refers  to  sensory  apraxia, 
— that  is,  to  the  disorder  due  to  disease  on  the  sensory  or  receptive  side  of 
the  brain.  Cases  are  on  record,  however,  which  seem  to  show  that  a 
motor  as  well  as  a  visual  or  other  form  of  sensory  apraxia  may  be  exhib- 
ited, and  it  is  worth  while  to  briefly  refer  to  these  cases  as  they  belong  to 
the  matter  in  hand  in  a  diagnostic  way  because  it  may  sometimes  become 
necessary  to  differentiate  motor  apraxia  from  visual  or  other  type  of  the 
sensory  disorder.  Pick,^^  for  example,  has  recorded  a  remarkable  case 
which  he  believes  to  be  one  of  motor  apraxia.  A  man  sixty-two  years 
old  had  for  three  years  before  coming  under  observation,  peculiar  at- 
tacks.   He  would  suddenly  find  himself  unable  to  speak  the  words  which 


*  Burr,  Jour,  of  Nerv.  and  Ment.  Disease,  May,  1897. 
^  Mitchell,  Medical  News,  vol.  Ixii.  Oct.  1892. 
"  Pick,  Neurol.  Centralbl.,  vol.  xxi.  Nov.  1,  1902. 


MIND-BLINDNESS  121 

he  wished  to  utter  or  to  do  anything  desired.  He  could  not  read  or 
write,  and  everything  seemed  to  swim  before  his  eyes.  He  had  neither 
paralysis  nor  loss  of  hearing,  and  in  three  days  he  was  again  normal. 
Similar  attacks  occurred  about  once  in  two  weeks,  and  were  associated 
with  slight  tonic  niasseter  spasm.  On  one  occasion  he  saw  a  mist  and 
fiery  rings. 

One  of  the  most  remarkable  cases  of  apraxia  ever  recorded  has  been 
reported  by  Liepmann.^^  In  this  case  the  disorder  was  unilateral ;  the 
patient  was  neither  hemianopsic  nor  mind-blind.  The  tactile  and  pain 
senses  were  much  diminished  in  the  right  half  of  the  body,  especially  in 
the  right  limbs.  The  power  of  localization  and  the  sense  of  position  and 
of  movement  of  the  limbs  seemed  to  be  absent.  The  only  motor  paraly- 
sis was  in  the  lower  branch  of  the  left  facial  nerve.  This  patient 
had  motor  aphasia  and  could  only  say  a  few  words,  these  being  al- 
ways the  same ;  in  other  words,  they  were  the  recurring  utterances 
of  Hughlings  Jackson.  He  understood  what  was  said  to  him,  could 
read,  and  obeyed  simple  commands  when  they  were  written,  provided 
his  movements  were  not  performed  with  the  right  limbs  alone.  The 
movements  of  the  head,  including  those  of  the  face  and  tongue,  were 
apraxic  on  both  sides.  \\"hen  he  was  asked  to  make  any  movement  with 
his  right  hand,  such  as  touching  the  nose  with  the  finger,  his  attempts 
were  unsuccessful ;  but  with  the  left  hand  the  command  was  promptly 
obeyed.  Wlien  he  was  tickled  in  the  right  ear  he  made  no  movement 
of  defense;  but  with  the  left  hand  he  tried  to  remove  any  object  that 
was  placed  in  his  left  ear.  He  was  unable  to  remove  with  his  right  hand 
a  needle  stuck  in  his  right  thigh.  When  asked  to  pick  up  with  his 
right  hand  one  of  several  objects  placed  before  him,  he  was  unable  to 
comply  with  the  request.  It  is  probable  that  he  understood  what  was 
asked  of  him,  because  after  he  had  failed  to  pick  up  with  the  right  hand 
the  desired  article,  he  was  able  to  pick  it  up  with  the  left  hand  when 
simply  told  to  use  this  hand,  without  the  name  of  the  article  being  men- 
tioned again.  With  the  right  hand  he  was  completely  agraphic;  but 
with  the  left  he  was  able  to  do  mirror  writing,  although  the  letters  were 
very  irregular.  His  gait  was  normal.  Occasionally  he  performed  an  in- 
tentional movement  correctly. 

In  this  case  the  patient  was  supposed  to  be  suffering  from  aphasia 
with  dementia,  a  fact  of  medicolegal  as  well  as  of  neurological  inter- 
est.   Liepmann  believed  the  lesion  existed  in  the  third  frontal  gyrus  and 

"Liepmann,  Monatschrift  fiir  Psychiatric  und  Neurologie,  July,  August,  and 
September,  1900,  vol.  viii.  This  case  is  cited  by  Spiller,  with  comments,  in  Pro- 
gressive Medicine,  1900-1901. 


122  THE    EYE    AND    NERVOUS    SYSTEM 

insula,  without  implicating;  to  any  extent  the  motor  area,  or  the  inferior 
or  superior  parietal  convolutions.  It  is  difficult,  however,  to  believe  that 
tho  lesion  was  so  placed,  or  at  least  so  limited,  as  I  have  seen  and  re- 
ported a  case  of  destructive  lesion  of  the  left  third  frontal  and  insula, 
in  which  no  such  unilateral  apraxia  was  present  although  the  case  pre- 
sented typical  aphasia. 

The  discovery  of  the  symptom  known  as  mind-blindness  was  due  to 
the  physiologist  Munk,  who  had  observed  it  as  the  result  of  several  extir- 
pation experiments  on  dogs.  He  found  in  some  cases,  when  he  removed 
a  portion  of  the  occipital  lobes,  the  dog,  while  still  retaining  the  power  to 
see  objects,  and  to  avoid  them  through  the  medium  of  sight,  was  evi- 
dently unable  to  recognize  the  meaning  and  use  of  such  objects.  The  dog 
from  which  a  portion  of  the  occipital  lobe  had  been  removed,  for  exam- 
ple, no  longer  recognized  his  master,  other  dogs  with  which  he  had  been 
long  familiar,  or  the  food  which  was  placed  before  him, — except,  in  the 
last  case,  through  the  sense  of  smell.  He  gave  to  this  symptom  the  name 
of  seeJenhlitidheit  (soul-blindness  or  mind-blindness).  He  believed  that 
this  symptom  was  due  to  lesion  of  the  region  for  visual  memories,  the 
visual  memory  field,  the  visual  portion  of  the  concrete  memory  field  or 
concrete  concept  area  of  the  writer.  Flechsig  holds  practically  to  the 
same  view,  believing  that  not  only  mind-blindness  but  mind-deafness 
are  due  to  lesions  of  portions  of  this  great  association  area. 

The  truth  is  that  all  symptoms  which  result  from  impairment  or 
disturbance  of  concrete  concepts  or  concrete  memories  are  to  be  sought 
for  in  lesions  of  centres  located  in  the  great  concrete  concept  area,  or  in 
the  tracts  going  to  or  coming  from  the  centres  of  this  area,  or  associating 
them  together.  Mind-blindness  results,  or  may  result,  from  lesion  of  the 
visual  cortex  or  subcortex  of  this  area ;  mind-deafness,  from  the  audi- 
tory portion  of  this  area ;  astereognosis,  from  lesions  of  that  portion  of 
this  area  devoted  to  the  muscular  sense  and  stereognostic  perception; 
tactile  amnesia,  from  lesions  of  the  centres  and  tracts  concerned  with 
tactile  recognition ;  music-deafness,  from  lesions  of  the  centres  and 
tracts  related  to  the  function  of  intonation ;  and  so  on  throughout  the 
entire  list  of  concrete  memories  and  their  centres  and  tracts. 

!Munk's  views  regarding  the  extent  of  cortex  sufficient  to  cause 
mind-blindness  do  not  appeal  to  the  writer,  as  they  have  not  to  others,  as 
correct, — this  for  theoretical  reasons  and  because  of  clinicopathologi- 
cal  observation.  Munk  expressed  the  opinion  that  extirpation  of  the 
circular  area  of  the  cortex  so  small  as  fifteen  millimetres  in  diameter 
and  two  millimetres  in  thickness  would  cause  in  the  animal  the  phe- 
nomena  which  he   designated   as   seelenblindheit.      The   term   seelen- 


MIXD-BLIXDNESS  123 

blindheit  was  used  by  Munk  in  contradistinction  to  rindenhlindheit  or 
cortical  blindness,  in  which  the  animal  becomes  totally  blind  to  all  im- 
pressions; it  is  unable,  in  other  words,  to  see  any  objects  presented  to 
it  as  well  as  to  recall  the  image  of  one  formerly  seen.  Observations  on 
man  show  that,  whatever  may  be  the  case  with  dogs,  it  is  necessary 
that  a  somewhat  larger  area  of  the  cortex  on  both  sides  shall  be  destroyed 
than  that  indicated  by  Munk ;  and  yet  he  was  not  as  far  wrong  as 
Goltz  and  some  of  his  most  caustic  critics  would  have  us  believe.  Cases 
are  now  on  record,  some  of  which  will  be  presently  given,  in  which  com- 
paratively limited  lesions  of  the  visual  cortex  of  both  hemispheres  have 
given  rise  to  mind-blindness.  It  is  not,  however,  correct  to  regard  the 
regions,  destruction  of  which  causes  mind-blindness,  as  those  for  clear 
or  central  vision ;  while,  as  has  been  stated,  the  centre  for  the  macula 
is  the  centre  for  clear  vision  and  for  the  recognition  of  minute  objects 
and  objects  with  special  forms,  such  as  those  for  words,  letters,  numbers 
and  geometrical  figures,  the  cortical  areas  of  peripheral  representa- 
tion, like  the  peripheral  retina  itself,  are  capable  of  recognizing  many 
objects.  The  truth  is,  as  I  have  already  stated,  that  mind-blindness 
itself  needs  division  and  has  a  considerable  sub-divisional  cortical  rep- 
resentation. The  individual  may  be  mind-blind  for  persons,  or  places, 
or  figures,  words  or  letters,  or  any  of  these  or  any  combinations  of  these, 
the  powers  of  recognizing  the  others  remaining. 

One  of  the  most  important  of  the  early  cases  of  mind-blindness  is 
that  recorded  by  Lissauer  "^  in  great  detail  and  with  a  discussion  by  him 
of  the  pathology  and  symptomatology'  of  the  affection  and  the  methods 
of  studying  it.  The  patient  was  a  man  eighty  years  old,  who  for  three 
years  before  coming  under  observation  had  had  attacks  of  dizziness 
which  were  often  so  violent  that  he  fell.  For  about  a  year  he  had  been 
forgetful ;  he  often  could  not  recall,  for  instance,  the  ages,  names  and 
number  of  his  children.  After  a  special  attack  he  complained  that  he 
could  not  see  as  well  as  before.  He  bumped  against  doors  and  other 
objects  and  could  not  go  around  alone.  He  mistook  parts  of  his  dress 
and  all  sorts  of  objects ;  he  did  not  recognize  his  own  daughter.  His 
mind  was  clear  for  familiar  business  matters.  The  attacks  of  mind- 
blindness  were  transient. 

On  examination,  while  he  showed  signs  of  old  age,  he  was  not  in 
any  way  demented.  While  he  could  speak  intelligently  and  easily  he 
was  unable  to  recognize  everything.  By  touch  and  hearing  he  at  once 
recognized  everything.     His  sight,  except  for  his  mind-blindness,  was 

"  Lissauer,  Archiv  fur  Psychiatric  und  Nervenlieilkunde,  vol.  xxi.  1889-90. 


124  THE    EYE    AND    NERVOUS    SYSTEM 

good.  He  drew  objects  or  designs  but  afterwards  he  could  not  recognize 
them.  He  perceived  birt  could  not  apperceive.  With  the  exception  of 
the  mind-blindness,  nothing  else  of  importance  could  be  made  out.  His 
hearing  was  dull,  but  he  showed  no  cerebral  affection  of  hearing  or  of 
speech.  He  was  not  paralyzed  and  had  no  impairment  or  disturbance 
of  sensation.    His  ocular  movements  and  reflexes  were  good. 

The  patient  during  six  months  after  he  first  came  under  observation 
made  some  improvement,  and  then  failed  to  make  any  further  progress. 
At  times  during  this  period  considerable  vacillations  were  observed  in 
the  amount  and  degree  of  his  mind-blindness.  He  would  recognize 
objects  at  one  time  while  he  would  fail  to  do  so  at  another.  Even  after 
he  had  made  considerable  improvement  he  would  occasionally  show  a 
degree  of  inability  to  recognize  objects  as  bad  as  he  had  at  any  time. 
The  case  was  not  followed  until  death  but  is  nevertheless  one  of  much 
value  because  of  the  careful  manner  in  which  it  was  investigated  and 
described. 

In  a  case  reported  by  F.  Miiller,^^  the  left  occipital  lobe  was  almost 
destroyed  by  a  tumor  and  softening,  and  the  posterior  part  of  the  cal- 
losum  was  implicated  in  the  softening,  and  the  associating  tracts  con- 
necting the  right  occipital  lobe  with  the  left  hemisphere  and  its  speech 
centres  were  interrupted.  In  this  way  Miiller  explains  the  optic  apha- 
sia and  alexia  which  were  present.  Mind-blindness  was  also  present  in 
this  case. 

Miiller  mentions  that  mind-blindness  is  often  associated  with  optic 
aphasia  and  asymbolia,  and  he  gives  the  reference  to  a  number  of  cases. 
Even  more  commonly  it  is  associated  with  alexia,  and  he  refers  to  sev- 
eral examples.  In  a  number  of  cases  right  hemianopsia  existed.  Miil- 
ler says  that  in  all  cases  of  pronounced  mind-blindness  with  satisfactory 
necropsy  (1892)  disease  of  the  occipital  lobe  was  found,  or  of  the  parts 
adjoining,  as  in  two  cases  in  which  the  parietal  lobe  was  affected.  Of 
twenty  cases,  both  hemispheres  were  diseased  in  fourteen ;  and  of  these 
both  occipital  lobes  were  affected  in  twelve ;  in  one,  the  right  occipital 
lobe  and  the  left  frontal  lobe  were  affected ;  and  in  one  the  left  occipital 
and  the  right  parietal  lobes  were  affected.  In  six  cases  the  lesion  was 
unilateral,  and  of  these  the  left  hemisphere  was  diseased  in  four  cases 
(in  two  the  left  occipital  and  in  two  the  left  parietal).  In  two  cases 
the  right  occipital  lobe  alone  was  diseased. 

Wernicke,  W^ilbrand,  Bruns,  Stolting  and  others  believe  that  the 
occipital  lobes  are  of  equal  value  in  function,  especially  in  the  recogni- 

"  Miiller,  Arch,  fiir  Psvch.,  1892,  vol.  x.xiv.  p.  856. 


MIND-BLIN^DNESS  125 

tion  of  objects,  and  that  mind-blindness  can  exist  only  when  both  occip- 
ital lobes  are  diseased.  Miiller  thinks  it  has  been  shown  satisfac- 
torily that  mind-blindness  may  be  caused  by  unilateral  lesion,  as  the 
examination  of  the  brain  must  be  microscopic  and  not  merely  macro- 
scopic. It  seems  to  him  more  probable  that  lesions  of  the  outer  portion 
of  the  occipital  lobes,  especially  on  the  left  side,  cause  mind-blindness. 
The  destruction  of  the  tracts  in  the  callosum  connecting  the  occipital 
lobes  or  one  occipital  lobe  with  other  parts  of  the  opposite  hemisphere 
is  of  much  importance  in  the  causation  of  mind-blindness. 

Liepmann  ^^  has  reported  a  case  of  practically  uncomplicated  mind- 
blindness  in  a  music  teacher,  aged  fifty  years,  who  had  an  apoplectic 
attack,  September,  1901.  After  this  he  did  everything  in  a  reverse  or 
foolish  manner  as,  for  instance,  washing  in  his  soup.  He  did  not  recog- 
nize large  objects,  nor  his  own  reflection  in  the  mirror,  but  recognized 
persons  by  the  voice.  He  did  not  know  his  own  wife  by  sight;  color 
perception  was  much  disturbed.  A  small  defect  was  present  in  the 
upper  right  quadrant  of  the  visual  field ;  vision  was  two-thirds.  He 
could  imitate  large  movements  fairly  well.  Well-known  objects  were 
often  wrongly  named.  Recognition  through  touch  was  slightly  dis- 
turbed, but  through  hearing  was  normal.  Motor  aphasia  was  absent,  as 
was  also  sensory  aphasia,  unless  his  wrongly  naming  objects  might  be 
so  considered. 

The  patient  died  in  1904,  two  years  after  the  case  was  first  recorded 
by  Liepmann.  A  necropsy  showed  most  interesting  results.  Lesions 
were  found  on  both  sides  of  the  brain,  on  the  left  in  the  lower  occipital 
convolutions,  and  on  the  right  in  the  angular  and  second  occipital  convo- 
lutions. In  the  right  hemicerebrum  the  area  of  softening  was  on  the 
convexity,  and  extended  from  the  angular  gyre  through  the  gray  and 
white  matter  of  the  second  occipital  gyre. 

A  case  reported  by  Serieux  ^^  is  of  value  notwithstanding  the  com- 
plication of  object-blindness  with  other  symptoms  like  word-blindness. 
This  case,  which  was  published  in  1891,  is  cited  by  Wyllie.  The  pa- 
tient was  a  woman  sixty-two  years  old,  who  in  1888  had  a  temporary 
paralytic  attack  with  lasting  visual  disturbances,  and  in  1890  had  a  sec- 
ond seizure  with  epileptiform  spasms,  hallucinations  of  hearing,  and 
delirium.  When  examined  six  months  later,  she  was  word-blind, 
agraphic,  word-deaf,  and  paraphasic.  She  also  showed  marked  mind- 
blindness  with  intact  general  intelligence.     She  was  unable  to  recognize 


'^  Liepmann,  Allgemeine  Zeitschrift  fiir  Psychiatrie,  Berlin,  vol.  lix,  1902. 
^  Serieux,  Compt.  Rend.  Soc.  de  Biol.,  December,  1891. 


120  THE    EYE    AND    NERVOUS    SYSTEM 

her  nearest  relations,  said  that  people  around  her  seemed  to  be  wearing 
masks,  that  her  daughter's  face  appeared  "  flat  as  a  skate."  The  object- 
blindness  was  most  marked  with  regard  to  faces,  although  exhibited  in 
less  degree  with  reference  to  other  things.  She  could  recognize  some  ob- 
jects, but  not  others;  a  comb,  for  instance,  was  mistaken  for  a  pen 
holder.  She  died  of  pneumonia  a  few  months  after  the  observations  of 
Serieux  were  made.  At  the  autopsy,  lesions  were  found  in  both  hemis- 
pheres. In  the  left  hemisphere  were  present  patches  of  softening 
in  the  supramarginal  (subparietal)  gyre,  and  in  the  caudal  extremity 
of  the  first  temporal  gyre ;  in  the  right  were  large  areas  of  softening  in- 
volving the  angular  and  supramarginal  gyres,  and  also  the  caudal  ex- 
tremities of  the  first  and  second  temporal. 

So  far  as  is  known  to  the  writer,  the  few  other  cases  of  mind-blind- 
ness which  are  recorded  are  not  of  equal  value  with  those  of  Liepmann 
and  of  Serieux,  but  all  point  to  the  existence  of  centres  for  object  recog- 
nition by  sight  in  the  temporo-occipital  convolution  from  the  second  to 
the  fourth  or  fifth. 

Two  cases  have  fallen  under  my  observation,  of  transient  or  recur- 
ring mind-blindness.  In  both  of  these  cases  operation  was  performed 
for  the  removal  of  a  brain  lesion,  presumed  to  be  a  tumor,  although  in 
one  case  at  least  the  result  did  not  confirm  this  opinion.  In  this  ease 
the  patient  presented  in  the  right  half  of  the  body  disorders  of  sensa- 
tion, stereognosis  and  co-ordination.  He  was  partially  word-deaf,  word- 
blind,  and  letter-blind,  and  late  in  the  case  showed  marked  hemiparesis. 
The  operation  was  performed  with  the  view  of  exposing  the  brain  at 
the  posterior  extremity  of  the  first  temporal  convolution.  A  large  patch 
of  necrotic  tissue,  having  somewhat  the  shape  of  a  pyramid,  with  its 
base  towards  the  occipital  pole  of  the  brain,  was  uncovered.  The  lesion 
may  have  extended  further  than  was  revealed  by  the  operation.  It  not 
improbably  included  the  caudal  extremities  of  the  first  and  second  tem- 
poral convolutions,  and  possibly  more. 

The  operation  was  performed  four  years  prior  to  the  writing  of  this 
article,  and  the  patient  is  still  living  and  in  fair  health,  but  is  hemi- 
plegic,  partially  hemiana^sthetic  and  aphasic.  lie  suffered  at  times 
from  curious  attacks  which  can  properly  be  described  as  periods  of  mind- 
blindness  or  object-blindness.  Recently,  I  re-examined  this  patient. 
I  found  that  his  hemiplegic  condition  had  gradually  become  worse,  the 
paralysis  having  visibly  increased,  especially  during  the  last  year.  He 
showed  some  slight  improvement  in  the  ability  to  name  objects  looked 
at,  also  in  being  able  to  name  many  words.  He  could  "not,  however, 
form  phrases  or  sentences.     He  seems  still  to  be  partially  word-deaf^ 


WOKD-BLIXDNESS:    ALLIED    DISORDERS  127 

being  thrown  into  confusion  in  trying  to  understand  much  that  was 
said  to  him.  He  continued  largely  word-blind,  but  recognized  an  occa- 
sional word,  writing  being  understood  rather  better  than  printed  matter. 

His  wife,  who  accompanied  him,  stated  that,  teginning  about  two 
months  after  the  operation,  the  patient  had  at  irregular  intervals  spells 
of  temporary  mind-blindness  or  object-blindness.  In  these,  which  would 
last  from  a  few  minutes  to  one  or  two  hours,  while  he  would  seem  to 
know  his  wife,  he  was  apparently  unable  to  recognize  others.  In  these 
attacks  also  he  appeared  unable  to  recognize  objects  or  his  surroundings ; 
he  seemed  dazed,  would  look  around  as  if  wondering  who  the  persons 
and  what  the  objects  around  him  were,  repeating  almost  constantly  one 
word,  "  ^^^lat,  what."  He  could  not  understand  anything  that  was  said 
to  him  at  the  time  of  these  spells,  and  became  greatly  confused  and  irri- 
tated. His  wife  said  that  he  remembered  these  spells  after  they  passed 
off,  and  would  say  "  Awful,  awful,"  as  if  in  great  dread  of  them.  Ex- 
amination showed  that  he  understood  the  use  of  most  objects,  such  as  the 
telephone,  a  pen,  ink,  shears,  and  paper.  An  effort  was  made  to  examine 
him  for  color-blindness.;  he  could  not  recognize  red  and  blue,  but  seemed 
to  know  green  and  yellow;  but  when  the  examination  was  continued, 
he  became  confused,  and  was  unable  to  recognize  any  colors. 

In  the  second  case  referred  to,  in  which  operation  exposing  the  occip- 
ital lobe  was  performed,  and  portions  of  the  cortex  removed  or  con- 
tused, corresponding  to  the  parts  of  the  first  and  second  occipital  con- 
volutions, the  man  suffered  not  only  from  optic  aphasia,  but  tem- 
porarily from  object-blindness.  At  first,  he  did  not  at  times  recognize 
persons  and  things.     The  impairment  was  only  partial. 

Briefly  stated,  the  pathological  cause  of  mind-blindness  is,  in  the 
first  place,  a  lesion  or  lesions  of  the  visual  portion  of  the  concrete  mem- 
ory field.  Usually  the  disease  is  on  both  sides  of  the  brain,  but  transient 
forms  of  the  affection,  and  perhaps,  in  exceptional  instances,  the  per- 
sisting disorder  may  be  caused  by  a  destructive  lesion  of  the  left  optic 
memory  field.  In  the  second  place,  lesions  of  the  tracts  which  connect 
the  higher  visual  field  with  the  lower  or  primary  visual  centres  may 
cause  this  symptom.  When  the  affection  is  due  to  a  lesion  of  the  asso- 
ciation fibres  it  is,  if  unilateral,  so  situated  as  to  destroy  the  tracts  which 
connect  the  primary  or  low^er  visual  centres  on  both  sides  with  the  higher 
visual  area  on  one  side. 

WORD-BLINDNESS  AND  ALLIED  DISORDERS. 

In  several  places  in  the  present  chapter  reference  has  been  made  to 
the  fact  that  in  the  angular  convolution  of  the  left  cerebral  hemisphere 


128  THE    EYE    AND    NERVOUS    SYSTEM 

and  its  immediate  neighborhood  is  situated  a  centre  for  the  storage  of 
the  visual  images  of  words.  It  is  not  infrequently  spoken  of  as  the 
centre  for  word-seeing.  It  is  the  last  and  most  important  development 
of  the  higher  visual  area,  the  visual  portion  of  the  concrete  memory  field. 
A  destructive  lesion  of  the  cortex  of  this  region  or  of  the  tracts  entering 
it  from  the  primary  or  lower  visual  centre  will  cause  the  symptom  to 
which  Kussmaul  gave  the  name,  word-blindness.  One  of  the  best  defini- 
tions of  word-blindness  is  that  of  Bateman,  who  describes  it  as  a  form 
of  verbal  amnesia  in  which  the  patient  has  lost  the  memory  of  the  con- 
ventional meaning  of  graphic  symbols.  He  sees  these  symbols,  he  knows 
tliat  they  differ  from  their  environments,  but  he  cannot  translate  them 
mentally  into  words.  They  are  to  him  as  if  they  had  never  had  any 
significance. 

The  two  chief  varieties  of  word-blindness  now  usually  described  are 
kno\vn  respectively  as  cortical  word-blindness  and  pure  word-blindness. 
By  cortical  word-blindness  is  meant  that  variety  of  this  form  of  aphasia 
which  is  due  to  lesion  limited  to  the  cortex ;  while  the  term  pure  word- 
blindness  was  applied  by  Dejerine  to  the  form  which  is  dependent  upon 
a  lesion  of  the  tracts  which  enter  the  cortical  centre  for  word-seeing  from 
the  primary  visual  centres.  The  term  pure  word-blindness  like  pure 
word-deafness  does  not  seem  to  be  a  happy  one,  but  is  perhaps  better 
retained,  as  it  has  become  fixed  in  the  literature  of  the  subject.  It  is 
objectionable  in  the  sense  that  a  number  of  similar  designations  are  of 
doubtful  clearness, — as,  for  instance,  Munk's  soul-blindness  (seelen- 
hlindlieit),  which  is  used  as  a  contrasting  term  with  his  cortical  blind- 
ness (rindenhlindheit)  :  one  designation  is  founded  upon  the  psycholog- 
ical idea,  that  of  psychic  blindness,  and  the  other  is  anatomico-patho- 
logical in  its  origin,  referring  to  the  blindness  caused  by  the  destruction 
of  cortical  brain  substance. 

In  cortical  word-blindness  the  patient  is  unable  to  comprehend  words 
by  sight  and  is  also  agraphic.  Owing  to  the  loss  of  the  memory  of  visual 
images,  he  cannot  recall  these  for  the  purposes  of  writing.  In  the  pure 
word-blind  patient,  while  he  cannot  comprehend  words,  either  printed  or 
written,  by  looking  at  them  he  may  be  able  to  write  spontaneously  or 
from  dictation,  as  his  cortical  centre  for  the  visual  images  of  words  and 
letters  is  not  destroyed,  but  if  his  eyes  be  removed  even  for  a  moment 
from  the  written  page  he  cannot  recognize  the  word  which  he  has  just 
written. 

^Vhile  we  owe  to  Dejerine  a  clearer  conception  of  the  manner  in 
which  word-blindness  usually  originates,  the  observation  of  two  forms 
of  word-blindness,  one  in  which  the  patient  is  agraphic,  and  the  other 


word-bli:n^dxess:  allied  disordees 


129 


in  which  he  retains  the  power  of  writing  from  dictation  or  spontane- 
ously, is  not  new.  Westphal,  who  is  cited  by  Kussmaul,  many  years  ago 
recorded  the  case  of  an  actor  who  was  hemiplegic  and  incompletely 
aphasic.  Although  he  could  speak  with  fluency,  he  could  not  recognize 
or  understand  some  words.  He  was  completely  alexic,  and  while  he 
could  write  from  dictation,  he  immediately  afterwards  could  not  com- 
prehend the  words  he  had  written. 


Auo.Gyt. 


Fig.  is.— Dejerine's  diagram  showing  the  position  of  the  lesion  causing  pure  word-blindness. 

The  exact  view  of  Dejerine  with  regard  to  the  manner  in  which  pure 
word-blindness  is  caused  can  be  best  shown  by  a  reference  to  the  diagram- 
matic illustration.  Fig.  18,  which  is  reproduced  by  permission  from 
Wyllie's  work  on  the  Disorders  of  Speech. 

"An  examination  of  this  diagram  (Fig.  18)  will  enable  the  reader  to 

understand  quite  easily  the  relation  of  the  eyes  to  the  primary  visual 

centre  in  the  occipital  lobe.     In  the  diagram,  the  left  optic  tract  and  its 

connections  are,  for  the  sake  of  distinctness,  given  in  deep  black.     The 

9 


130  THE    EYE    AND    NERVOUS    SYSTEM 

reader  will,  on  looking  at  the  diagram,  at  once  understand  how  section 
of  the  left  optic  tract,  BO,  would  produce  homonymous  hemianopsia  in 
the  opposite  half  of  the  field  of  vision.  He  will  also  see  that  the  same 
effect  would  be  produced  by  a  lesion  further  back,  either  in  the  optic 
thalamus,  in  the  radiations  of  Gratiolet,  or  in  the  primary  cortical  centre 
itself.  But  he  will  note  that  word-blindness  will  not,  in  any  of  these 
cases,  be  added  to  the  hemianopsia,  unless  the  lesion  be  situated  at  about 
X,  and  so  cut  across  not  only  the  fibres  which  connect  the  angular  con- 
volution with  the  left  occipital  lobe,  but  also  those  which  connect  it  with 
the  right  one." 

As  the  early  cases  illustrating  the  difference  between  cortical  and 
precortical  word-blindness  are  comparatively  well  known,  I  shall  refer 
here  only  to  a  few  cases  somewhat  recently  reported. 

Dejerine  and  Thomas  ^"^  report  an  additional  case  of  a  woman  who 
developed  right  hemiplegia  and  hemianesthesia  with  right  hemianopsia 
and  simultaneous  loss  of  the  power  of  writing  eight  years  before  her 
death.  After  a  slight  temporary  paraphasia  she  regained  the  power  of 
speaking;  she  understood  all  that  was  said  to  her;  she  was  able  to 
write,  but  immediately  afterwards  could  not  understand  what  she  had 
written ;  she  was  word-blind,  with  the  exception  that  she  could  recognize 
an  occasional  letter.  Two  years  after  her  first  apoplectiform  attack  she 
had  a  second,  and  after  this  she  became  unable  to  write  at  all,  either 
spontaneously  or  from  dictation,  her  other  symptoms  remaining  much 
the  same.  The  lesion  in  this  case  was  very  large ;  so  large,  indeed,  as  to 
interfere  a  little  with  reasoning  on  the  basis  of  the  findings.  It  was 
important  with  regard  to  the  views  of  Dejerine  as  to  the  cortical  word- 
blindness  and  pure  word-blindness  that  the  angular  convolution  escaped. 
A  large  focus  of  softening  involved  the  third  temporal  convolution  and 
the  posterior  ends  of  the  first  and  third  occipital,  while  ventromesially  it 
destroyed  the  cuneus,  the  fusiform,  lingual  and  subcallosal  gyres, 
the  hippocampus  and  cornu  ammonis ;  the  pulvinar  and  internal  genicu- 
late body  were  also  destroyed.  The  patient  died  from  recent  hemor- 
rhage. 

One  of  the  most  recent  cases  bearing  upon  the  subject  of  pure  word- 
blindness  is  recorded  by  Hinslielwood,  Macphail  and  Ferguson,'^®  this 
report  also  containing  several  other  facts  of  great  interest  to  the  student 
of  aphasia.  A  man,  fifty-three  years  of  age,  who  was  a  teacher  of  French 
and  German,  suddenly  found  that  he  had  lost  entirely  the  ability  to  read 


"  Dejerine  and  Thomas,  Rev,  Neurol.,  July  15,  1904,  p.  645. 

"  Hinshelwood,  Macphail,  and  Ferguson,  Brit.  Med.  Journ.,  Nov.  12,  1904. 


WORD-BLINDNESS:    ALLIED    DISORDERS  131 

words  or  letters.  He  could  write  correctly  to  dictation  but  could  not,  a 
short  time  afterwards,  read  what  he  had  written.  He  had  a  right  lateral 
homonymous  hemianopsia.  He  was  under  observation  for  nine  years 
until  his  death,  no  other  symptoms  developing.  He  was  able,  only  to  a 
very  limited  degree,  to  reacquire  the  visual  memories  of  words.  His 
death  was  due  to  a  hemorrhage  into  the  right  half  of  the  cerebellum  as 
was  shown  by  necropsy.  The  lesion  causing  the  word-blindness,  letter- 
blindness  and  hemianopsia  was  a  necrotic  cyst  in  the  occipital  lobe.  The 
cortex  destroyed  was  summarized  by  the  recorder  as  a  large,  triangular 
area  limited  by  the  calcarine  fissure  internally  and  by  the  third  tem- 
poral gyre  externally,  with  the  apex  at  the  occipital  lobe.  The  cor- 
tex of  the  cuneus  with  the  exception  of  the  posterior  portion  of  the 
calcarine  fissure  was  not  destroyed;  the  fibres  passing  to  and  from  the 
cuneus  were,  however,  involved  as  were  also  the  fibres  going  from  the 
cuneus  to  other  portions  of  the  occipital  cortex,  and  also  the  fibres  passing 
to  the  left  angular  gyre  from  both  the  left  and  right  primary  cor- 
tical visual  centres.  As  the  left  angular  gyre  was  intact,  it  will  be 
seen  that  the  case,  as  the  authors  assert,  supports  the  views  of  Dejerine 
and  of  Serieux  as  to  the  distinction  between  precortical  or  pure  word- 
blindness  and  cortical  word-blindness. 

I  have  emphasized  the  views  of  Dejerine  with  regard  to  the  distinc- 
tion between  his  pure  word-blindness  and  cortical  word-blindness,  or 
what  I  would  prefer  to  call  precortical  or  qualified  word-blindness,  and 
cortical  or  absolute  word-blindness,  and  also  have  given  his  hypothesis  as 
to  the  position  of  the  lesions  in  these  two  forms  of  visual  aphasia  illus- 
trating these  facts  by  references  to  a  few  recorded  cases.  It  must  not 
be  understood,  however,  that  the  views  of  Dejerine  necessarily  explain 
all  cases  of  his  so-called  pure  word-blindness.  Cases  are  on  record  which 
do  not  bear  out  the  supposition  that  this  form  of  visual  aphasia  is  always 
dependent  upon  a  precortical  lesion.  Several  cases  are  now  on  record 
in  which  the  so-called  pure  word-blindness  or  pure  word-deafness  or  both, 
in  the  same  case,  were  present,  yet  the  lesion  in  whole  or  in  part  affected 
the  cortical  centres  for  word-seeing  and  word-hearing.  Strohmayer  ^^ 
has  examined  the  eases  of  pure  sensory  aphasia  accessible  to  him,  six  in 
all.  He  points  out  the  important  fact  that  in  five  of  these  cases  the 
lesions  were  bilateral,  implicating  both  the  cortex  and  the  subcortex  of 
the  temporal  lobe.  In  only  one  case  was  the  lesion  unilateral,  and  here 
it  was  confined  to  the  left  temporal  cortex.    From  a  study  of  these  cases 


■*  strohmayer,  Deutsche  Zeitschrift  f .  Nervenheilkunde,  vol.  xxi.  Nos.  5  and  6, 
p.  371. 


132  THE    EYE    AND    NERVOUS    SYSTEM 

he  argues  that  the  lesions  causing  pure  sensory  aphasia  are  bilateral, 
although,  as  has  been  indicated,  a  few  cases  like  those  of  Dejerine  and 
Serieux,  for  instance,  seem  to  make  this  doubtful. 

As  has  been  shown  in  a  preceding  section  of  this  chapter,  according 
to  the  views  of  Lannegrace,  Ferrier,  Gowers  and  the  writer,  each  angular 
gyre  is  to  a  certain  extent  a  centre  of  representation  for  both  macula?. 
If  this  be  the  case,  it  might  be  possible  for  the  patient  to  be  word-blind 
from  lesion  of  the  higher  visual  area  of  one  hemisphere  with  or  without 
a  precortical  lesion  or  a  lesion  in  the  opposite  hemisphere  and  yet  retain 
some  power  of  writing  spontaneously  and  from  dictation  with  the  help 
of  the  undiseased  angular  convolution.  ' 

ITinshelwood  ^^  recently  reported  a  case  which  seems  to  show  that 
when  an  individual  knows  more  than  one  language  he  may  become  word- 
blind  to  one  language  alone,  or  the  word-blindness  may  vary  in  degree 
in  the  case  of  the  different  languages.  He  had  a  patient  who  became 
unable  to  read  English,  but  could  read  Greek,  and  had  partial  word- 
blindness  for  Latin.  On  testing  this  man  for  French,  a  greater  degree 
of  word-blindness  was  manifested  than  in  the  reading  of  Latin.  Even 
with  English  the  word-blindness  was  not  absolutely  complete,  and  there 
was  no  letter-blindness.  Hinshelwood  believes  that  in  the  case  of  a  per- 
son who  is  able  to  read  several  languages,  the  letter  and  word  visual 
images  of  each  language  will  be  grouped  together,  forming  thus  a  series 
of  separate  groups  within  the  centre  for  word-seeing.  If  the  whole  centre 
is  destroyed  or  its  functional  activity  abolished,  the  individual  will  be 
word-blind  to  all  languages ;  if  there  is  only  a  partial  interference  with 
the  centre,  then  the  word-blindness  may  be  only  partial  and  may  be  lim- 
ited to  a  single  group  of  these  visual  changes, — i.e.,  to  a  single  language. 
The  same  writer  refers  to  another  patient  who  was  word-deaf  to  French, 
his  native  tongue,  but  could  understand  what  was  said  to  him  in  Eng- 
lish. The  explanation  offered  is  similar  to  that  given  for  his  case  of 
word-blindness  to  languages;  in  other  words,  if  in  the  word-hearing 
centre,  the  auditory  images  of  different  languages  are  arranged  in  sep- 
arate groups,  in  a  partial  lesion  one  group  may  be  interfered  with  or 
destroyed,  while  the  others  are  preserved. 

Usually  in  word-blindness  as  in  any  other  form  of  sensory  aphasia 
or  of  motor  aphasia,  the  lesions  determining  the  symptomatology  are 
located  in  the  left  hemicerebrum ;  the  only  exceptions  to  this  presumably 
being  those  cases  in  which  the  cerebral  affection  occurs  in  left-handed 
persons.     Of  the  few  cases  which  have  been  seen  to  confirm  this  left- 


'  Hinshelwood,  Lancet,  Feb.  8,  1902,  p.  358. 


WORD-BLIXDXESS:    ALLIED    DISORDERS  133 

sided  localization  the  right-handedness  of  at  least  some  of  the  patients 
affected  with  the  aphasia  has  been  in  doubt. 

Recently  in  conjunction  with  Dr.  T.  H.  Weisenburg,  I  have  pub- 
lished the  account  of  a  case  of  marked  sensorimotor  aphasia  associated 
■svith  left  hemiplegia  and  some  other  phenomena.*'^ 

The  patient  at  the  age  of  thirty-five  had  a  severe  apopletic  stroke. 
He  was  seen  by  me  a  day  or  two  after  the  seizure  and  then  presented 
the  usual  phenomena  of  a  severe  cerebral  hemorrhage.  When  he  re- 
covered from  the  immediate  effects  of  the  attack,  he  was  found  to  be 
completely  hemiplegic  on  the  left  side  and  almost  absolutely  aphasic. 
He  was  seen  by  me  only  once  or  twice  at  times  closely  related  to 
his  apoplexy.  I  saw  him,  however,  a  year  later  when  he  regained 
the  use  of  his  left  extremities  so  that  he  was  able  to  walk  about  with 
the  usual  hemiplegic  gait,  and  his  aphasia  had  improved  to  a  certain 
extent. 

xVbout  a  year  after  his  first  seizure  he  had  a  convulsion,  three  or 
four  the  following  year,  and  the  third  year  still  more.  Dr.  Har\^ey 
Gushing,  of  Johns  Hopkins  University,  performed  an  osteoplastic  opera- 
tion designed  to  completely  cover  the  motor  region  of  the  right  side,  and 
to  a  less  extent,  the  area  posterior  to  it.  After  the  operation,  up  to  the 
time  of  his  last  coming  under  observation,  which  was  about  a  year  later, 
he  had  one  or  two  spasmodic  attacks.  These  attacks  were  mostly,  if  not 
altogether,  left  sided. 

Four  years  after  he  was  first  seen  by  me  in  his  acute  apoplectic  con- 
dition, this  patient  returned  and  remained  under  my  care  for  about  two 
months,  during  which  time  systemic  efforts  were  made  to  improve  his 
aphasia  by  training,  Dr.  Weisenburg  being  associated  with  me  in  this 
work.  At  this  time  a  careful  study  was  made  of  his  cerebral  symptoms. 
In  the  first  place,  he  still  had  marked  hemiplegia,  the  leg,  arm  and 
face  being  affected,  although  the  last  to  only  a  slight  extent ;  the  deep 
reflexes  on  the  paralyzed  side  were  exaggerated  and  a  typical  Babinski 
v/as  present;  on  the  entire  left  half  of  the  body  he  was  hypaesthetic  to 
touch,  pain,  temperature  and  to  vibration  sensation  (the  sensation  which 
results  from  the  placing  of  a  tuning  fork  on  bony  or  other  points)  ; 
sensation  of  position  was  partially  abolished  in  the  left  hand,  in  which 
he  was  also  largely  stereognostic ;  the  ophthalmoscope  showed  a  normal 
fundus  and  partial  left  lateral  homonymous  hemianopsia.  He  was  not 
word-deaf,  or,  if  so,  only  to  a  very  limited  degree ;  he  understood  what 
was  said  to  him  with  perhaps  rare  exceptions.     He  could  repeat  any- 

"  Mills  and  Weisenburg,  Medicine,  1905. 


134  THE    EYE    AND    NERVOUS    SYSTEM 

tiling  that  was  said  to  him,  his  articulation  and  enunciation  being 
excellent. 

His  most  interesting  aphasic  phenomena  were  in  the  visual  sphere. 
Immediately  after  his  attack  he  had  been  undoubtedly  word-blind  and 
letter-blind.  At  the  time  when  the  careful  examinations  were  made,  his 
word-blindness  and  letter-blindness  still  remained  in  very  large  degree. 
He  was  not,  however,  object-blind,  distinctly  recognizing  persons,  and 
also  the  use  and  nature  of  objects  aroimd  him.  He  knew  a  few  letters 
as,  a,  b,  d,  o,  s,  which  he  had  learned  as  the  result  of  many  months  of 
teaching.  Even  the  letters  which  he  knew  he  could  not  pick  out  from 
the  midst  of  the  word  in  which  the  letters  were  present.  As  the  result 
of  prolonged  teaching,  he  could  only  recognize  a  few  words,  and  these 
were  chiefly  those  with  which  he  had  been  extremely  familiar  in  his 
past  life.  He  could,  for  instance,  pick  out  the  words  apple  and  seed,  he 
having  been  in  a  business  in  which  such  words  were  frequently  used, 
both  by  mouth  and  by  written  signs.  He  could  sign  his  own  name  or 
he  could  write  his  own  name  with  the  appended  words  "and  wife;" 
if  what  he  had  written  was  presented  to  him  later,  however,  he  seemed  to 
be  utterly  oblivious  of  its  meaning.  He  had  learned  to  write  his  own 
name  with  the  added  words  "  and  wife"  in  order  to  register  at  hotels. 
He  could  do  this  as  well  with  his  eyes  closed  as  open.  He  was  an 
alexic  and  visual  agraphic. 

Close  investigation  of  this  man's  history  seemed  to  show  that  he 
was  bom  right-handed.  He  ate,  wrote,  and  did  by  preference  almost 
everything  with  his  right  hand.  When  he  was  four  years  old  he  broke 
his  right  arm,  and  during  the  time  that  he  was  unable  to  use  it,  he 
learned  to  do  many  things  with  his  left  hand.  H6  would,  for  instance, 
play  ball  with  his  left  hand  by  preference.  This  tendency  had  ap- 
parently arisen  out  of  the  conditions  of  the  fractured  right  arm. 

He  did  not  write  with  his  left  hand,  and  was  not,  in  the  ordinary 
sense  of  the  term,  ambidextrous.  We  can  assume,  therefore,  that  the 
patient  was  congenitally  and  by  acquired  habit  a  right-handed  individ- 
ual. The  lesion  in  this  case  was  undoubtedly  on  the  right  side,  and  was 
either  a  subcortical  hemorrhage  involving  the  tracts  going  to  the  pre- 
central,  postcentral,  superior  parietal  and  angular  convolutions,  and 
possibly  the  upper  part  of  the  first  temporal  convolution,  or  an  embolus 
of  the  right  middle  cerebral  artery.  The  optic  radiations  may,  to  some 
extent,  also  have  been  involved. 

The  important  features  of  this  case  were  (1)  that  in  a  right-handed 
man  with  left  hemiplegia  aphasia  largely  of  a  sensory  type  appeared  and 
this  aphasia  was  persistent;    (2)  the  aphasia  consisted  principally  in 


WORD-BLINDNESS:    ALLIED    DISORDERS  135 

word-  and  letter-blindness,  and  this  was  due  to  a  lesion  in  the  right 
side  of  the  brain. 

Instances  of  crossed  aphasia,  the  term  first  applied  bj  Bjrom  Bram- 
well,  are  very  rare.  By  crossed  aphasia  is  meant  aphasia  which  is,  so 
to  speak,  against  the  rule.  As  is  well  known,  aphasia  usually  occurs 
from  a  lesion  of  the  left  hemisphere  and  in  association  with  right-sided 
paralysis,  but  in  crossed  aphasia  the  symptom  is  due  to  a  right-sided 
lesion  in  a  patient  who  is  right-handed,  or  a  left-sided  lesion  in  a  person 
who  is  left-handed. 

Senator,^^  who  recorded  one  of  the  most  recent  cases  in  1904,  was 
able  to  collect  only  eleven  cases  from  literature,  one  or  two  of  which 
are  somewhat  doubtful;  and  in  all,  with  the  exception  of  Senator's 
case  and  the  one  we  now  report,  the  hemiplegia  was  right-sided  and 
occurred  in  left-handed  individuals. 

The  causes  of  crossed  aphasia  may  be  explained  in  several  ways: 
(1),  the  lesion  may  involve  the  cortex  of  both  sides;  this,  however,  is 
exceptional  and  was  not  present  in  any  of  the  recorded  cases;  (2),  there 
may  be  no  decussation  of  pyramidal  fibres,  the  lesion  being  on  the  same 
side  as  the  hemiplegia.  This  is  exceedingly  uncommon,  having  only 
been  recorded  in  a  single  case;  (3),  the  lesion  causing  aphasia  in  a 
right-handed  individual  with  left  hemiplegia  may  be  on  the  right  side, 
and  in  a  left-handed  person  with  right  hemiplegia  on  the  left  side. 

It  is  not  necessary  to  consider  the  first  two  hypotheses  in  the  case 
just  given,  for  a  lesion  of  the  right  side  undoubtedly  caused  the  left 
hemiplegia  and  aphasia.  It  is  difficult,  however,  to  explain  why  the 
centre  for  word-  and  letter-seeing  should  be  situated  on  the  right  side 
in  a  right-handed  individual. 

The  question,  of  course,  arises  as  to  what  factors  determine  on  which 
side  of  the  brain  the  motor  speech  centres  and  the  more  specialized 
centres,  as  of  word-  and  letter-seeing,  will  be  localized.  All  things 
considered,  in  a  child  which  has  not  as  yet  learned  to  write  or  read, 
heredity,  congenital  tendency  and  acquired  habit  determine  whether  the 
child  shall  be  right-  or  left-handed.  We  can  assume,  therefore,  that  a 
child  whose  parents  are  right-handed  and  in  whom  there  is  no  physical 
deformity  to  prevent  the  use  of  the  right  hand,  will  be  right-handed,  and 
that  its  leading  speech  centre  (presuming  that  both  sides  of  the  cortex 
have  to  do  with  motor  speech)  will  be  located  on  the  left  side  in  Broca's 
convolution.     The  converse,  of  course,  is  true  in  left-handed  persons. 

This,  however,  is  by  no  means  always  the  case.     Weber  ®^  records 

*"  Senator,  Charite  annalen,  vol.  xxviii.  1904. 
'^  Weber,  Centralbl.  f.  Physiol.,  xxiii.     Nr.  12. 


136  THE    EYE    AXD    NERVOUS    SYSTEM 

instances  of  children  who  had  not  as  jet  acquired  the  ability  to  write, 
in  which  a  lesion  on  the  right  side  would  cause  aphasia  in  right-handed 
persons,  and  vice  versa.  In  other  words,  in  children,  or  for  that  matter, 
in  adults,  who  had  not  as  yet  learned  to  write,  Weber  assumes  that 
the  motor  sj)eech  centres  are  potential  on  both  sides  of  the  cortex.  This 
is  probably  so,  for  it  is  improbable  that  the  speech  centres  are  equally 
divided  between  the  two  sides  of  the  cortex,  even  in  childhood.  Here 
heredity,  congenital  tendency  and  acquired  habit  will  probably  make 
the  child  either  right-  or  left-handed,  and  therefore  right-  or  left- 
brained. 

The  factor  which  determines  on  which  side  such  highly  specialized 
centres  as  those  for  word-  and  letter-seeing  would  be  localized  is  probably 
the  art  of  writing.  This,  no  doubt,  also  determines  on  which  side  the 
leading  motor  speech  centres  will  be.  This  was  well  sho^vn  in  a  patient 
of  Bramwell.®^  A  man  of  thirty-six,  who  was  left-handed  for  every- 
thing except  for  writing,  had  right  hemiplegia  with  persistent  motor 
and  sensory  aphasia,  evidently  from  a  lesion  in  the  left  hemisphere. 

A  case  of  Oppenheim  ®^  also  illustrated  this  point.  A  woman  of 
fifty-nine,  who,  since  she  was  seventeen,  had  been  left-handed  because  of 
an  injury  to  the  right  hand,  developed  complete  motor  and  sensory 
aphasia,  due  to  a  tumor  in  the  right  cerebral  hemisphere. 

In  the  patient  whose  case  is  just  recorded,  however,  the  man  always 
had  been  right-handed  and  had  always  written  with  his  right  hand.  At 
the  age  of  four,  as  already  stated,  he  had  broken  his  right  arm  and  had 
learned  to  do  many  things  with  his  left  hand.  Even  for  those  purposes 
for  which  he  commonly  used  his  left  hand,  he  could  and  sometimes  did 
employ  the  right.  We  must  assume,  therefore,  that  although  he  wrote 
with  his  right  hand,  and  was  born  and  continued  right-handed,  except 
as  stated,  his  motor  and  sensory  speech  centres  were  situated  in  the  right 
hemicerebrum.  On  inquiry  it  was  learned  that  the  patient's  parents, 
grand-parents  and  collateral  relatives  were  all  right-handed.  It  must 
be  assumed,  therefore,  that  in  exceptional  cases,  in  spite  of  the  usual 
laws  of  heredity  and  of  acquired  tendency,  there  is,  for  some  unknown 
reason,  an  inclination  toward  the  localization  of  the  motor  and  sensory 
speech  centres  in  that  side  of  the  brain  on  which  they  would  not  ordi- 
narily be  expected. 

It  is  equally  interesting  to  note  that  in  the  case  discussed  the  aphasia 
is  almost  wholly  confined  to  word-  and  letter-blindness.   Koster  ««  records 


"Bramwell,  The  Lancet,  June  3,  1899. 
"Oppenheim,  Archiv  f.  Psych.,  vol.  xxi.  1890,  p.  139. 

"Koster,   Abst.    in    Centralbl.    f.   Nervenheilkunde    and   Psychiatrie,    February, 
1901,  p.  117. 


WORD-BLI^DXESS:    ALLIED    DISORDERS  137 

a  case  with  necropsy  in  which,  with  left  hemiplegia  in  a  left-handed 
person,  there  was  word-blindness,  and  in  addition,  left  hemianopsia. 
At  the  necropsy  extensive  destruction  of  the  right  cerebral  hemisphere 
was  found.  Koster  believes  that  this  was  the  first  case  reported  with 
necropsy,  of  word-blindness  due  to  lesion  of  the  right  hemicerebrum. 
This  case  resembles  the  case  under  consideration  to  a  large  extent,  except 
that  the  patient  was  right-handed. 

The  case  here  recorded  is  also  instructive  from  another  standpoint, 
for  it  teaches  that  not  only  in  exceptional  cases  may  the  centre  for 
letter-  and  word-seeing  be  situated  on  the  right  side,  but  also  that  a 
unilateral  lesion  may  cause  permanent  loss  of  word-  and  letter-seeing, 
opposing  the  views  held  by  Bastian  and  others  that  these  functions  have 
a  bilateral  representation. 

Joffroy  ®^  records  the  case  of  a  right-handed  man,  aged  77  years,  who 
was  suddenly  seized  with  mental  agitation,  after  which  he  presented 
almost  complete  word-deafness  and  word-blindness.  He  could  recognize 
some  letters  and  could  appreciate  the  meaning  of  certain  words.  Para- 
phasia, paragraphia,  and  sometimes  jargon  utterances  were  present. 
He  could  write  his  name  with  difficulty,  but  could  not  copy  nor  write 
from  dictation.  Death  occurred  from  cerebral  hemorrhage  three  months 
later.  Examination  of  the  brain  showed  that  the  left  hemisphere  was 
normal.  The  right  hemisphere  presented  a  patch  of  softening  involving 
part  of  the  posterior  third  of  the  first  and  of  the  second  temporal  gyres. 
Around  this  patch  shrinking  of  the  convolutions  and  adlierence  to  the 
membranes  were  present,  extending  over  the  posterior  two-thirds  of  the 
first  and  second  temporal  gyres  and  involving  the  whole  angular  convo- 
lution. 

Joffroy  believed  that  this  man  was  congenitally  left-handed,  but 
that  he  had  become  trained  to  be  right-handed,  and  he  offers  this 
explanation  for  the  situation  of  the  speech  centres  upou  the  right  side 
of  the  brain,     ^o  ancestral  left-handedness  is  mentioned  in  the  history. 

Letter-blindness  (literal  blindness,  amnesia  literarum)  is  a  form 
of  blindness  due  to  cerebral  cortical  or  subcortical  disease  in  which  the 
patient  is  unable  to  recognize  letters,  although  the  visual  apparatus, 
from  the  periphery  to  the  primary  cortical  visual  centre,  or  cortical 
centre  for  optical  perception,  and  this  centre  itself  are  intact.  Letter- 
blindness  may  exist  without  word-blindness,  and  word-blindness  without 
letter-blindness,  although  both  are  usually  associated.  Curious  facts 
regarding  blindness  to  letters  from  lesions  of  the  cortical  centre  for 


Joffroy,  Rev.  Neurol.,  Jan.  31,  1903,  p.  112. 


138  THE    EYE    AND    :N^ERV0US    SYSTEM 

higlier  visualization,  or  the  entering  tracts  to  this  centre,  are  often 
observed.  The  patient  may  retain  the  ability  to  recognize  one  or  two 
or  several  letters  of  the  alphabet,  but  fail  to  comprehend  all  the  rest. 
We  believe  that  letters  and  words  are  represented  in  separate  cell 
groups  of  the  cortex,  although  these  are  adjacent. 

Alexia,  the  inability  to  read  because  of  cerebral  disease  or  dis- 
turbance, may  be  due  to  lesions  variously  situated.  In  its  most  complete 
and  typical  form  it  is  dependent,  as  has  already  been  shown,  upon  a 
lesion  of  tlie  cortical  centres  for  word-seeing  and  letter-seeing  of  the 
tracts  which  pass  to  these  from  the  optic  perception  centres  or  primary 
cortical  centres  for  sight  in  the  calcarine  cortex.  Some  degrees  of  alexia 
may,  however,  be  present  from  a  lesion  of  the  centres  for  word-hearing, 
naming,  or  of  Broca's  centre,  as  well  as  of  the  tracts  which  connect  these 
various  centres.  All  of  the  centres  in  the  zone  of  language  take  some 
part  in  the  verification  of  the  correctness  of  the  varieties  of  speech 
spoken  and  written. 

It  is  held  by  some  writers  on  aphasia  that  if  the  word-hearing 
centre  is  destroyed  the  patient  will  be  unable  to  read.  My  own  ex- 
perience, however,  shows  that  this  is  not  always  the  case,  and  Wyllie 
and  others  give  cases  in  which  word-deafness  was  present  without  alexia. 
In  these  cases  reading  is  probably  made  possible  by  direct  connections 
which  exist  between  the  visual  centres  for  words  and  letters  and  Broca's 
centre. 

In  so-called  motor  aphasia  from  lesion  of  the  left  third  frontal  and 
insula,  alexia  in  some  degree  may  be  present,  but  it  is  certainly  not 
absolute.  In  one  of  my  cases  which  was  under  observation  during  a 
period  of  many  years,  I  was  able  to  demonstrate  in  various  ways  that 
the  patient  was  able  to  read  silently,  although  his  reading  may  have 
been  imperfect.  When,  however,  he  attempted  to  read  aloud,  he  always 
repeated  the  same  words.  The  visual  images  in  this  patient  had  the 
power  to  revive  the  auditory  images,  although  perhaps  not  perfectly. 
Necropsy  in  this  case  revealed  a  destructive  lesion  confined  to  the  left 
third  frontal  and  insula. 

Paralexia,  in  which  the  patient  transposes  or  substitutes  words  and 
syllables  in  reading,  is  caused  by  lesions  variously  situated  in  the  zone 
of  speech,  as  by  partial  lesions  of  the  visual  centres,  by  lesions  of  the 
auditory  and  motor  speech  centre  and  of  the  tracts  connecting  these  cen- 
tres with  the  visual  centres  for  language.  Doubtless  there  is  a  silent 
paralexia,  the  patient  in  some  cases  transposing  silently. 

Dyslexia,  or  fatigue  in  reading,  the  result  of  lesion  or  disturbance 
of  the  cerebrum,  is  sometimes  observed.     The  patient  may  be  able  to 


WORD-BLINDNESS:    ALLIED    DISORDERS  139 

read  correctly  either  script  or  text,  to  read  aloud  or  to  repeat  what  is 
read  by  others,  but  the  effort  soon  becomes  fatiguing,  the  reader  tires 
and  may  be  unable  in  a  short  time  to  go  on  with  the  reading.  It  is  a 
•condition  comparable  to  that  which  is  observed  in  some  cases  of  word- 
deafness  which  have  partially  recovered,  and  is  sometimes  associated 
with  partial  word-blindness.  In  one  such  case  of  word-deafness  recently 
observed  by  me,  the  patient  could  listen  attentively  and  understandingly 
to  one  person  quietly  talking  to  him  for  a  short  time,  but  if  the  conversa- 
tion was  continued,  and  especially  if  it  became  more  varied,  his  attention 
soon  tired  and  before  long  he  would  entirely  fail  to  appreciate  what  was 
said  to  him.  If  several  persons  were  talking  to  him  at  the  same  time,  he 
also  became  confused  and  unable  to  comprehend  what  was  being  said. 
Reading  and  writing  of  an  imperfect  sort  could  be  continued  for  a  short 
time,  but  even  these  became  impossible  after  a  few  minutes. 

Dyslexia  may  be  due  to  a  partial  lesion  or  a  functional  disturbance 
•of  almost  any  portion  of  the  zone  of  speech.  In  its  most  typical  forms 
the  lesion  or  disturbance  is  usually  in,  or  in  close  connection  with, 
eithei^the  visual,  the  auditory,  or  the  motor  speech  centre.  It  is  some- 
times a  premonitory  symptom  in  apoplexy,  doubtless  indicating  some 
ischemia  in  a  region  which  subsequently  becomes  the  seat  of  hemorrhage 
or  occlusion. 

Agraphia  and  allied  conditions,  like  dysgraphia  and  paragraphia, 
may  be  due,  like  alexia,  dyslexia  and  paralexia,  to  lesions  variously 
fiituated  in  the  zone  of  speech,  as  in  the  centre  for  the  visual  images, 
that  for  auditory  images,  Broca's  centre,  and  the  so-called  motor-graphic 
centre  in  the  posterior  part  of  the  second  frontal  convolution.  More 
or  less  complete  agraphic  disturbance  may  also  be  due  to  lesions  of  the 
tracts  connecting  these  various  centres.  We  are  here,  of  course,  chiefly 
concerned  with  visual  agraphia,  that  form  of  impairment  of  reading 
which  is  dependent  upon  lesion  of  the  visual  centre  for  language  or  the 
tracts  which  lead  to  or  from  it. 

Stanley  Barnes  ^^  has  reported  a  case  of  graphic  aphasia  in  asso- 
ciation with  epilepsy  in  a  case  of  cerebral  tumor  lasting  nine  years. 
The  man's  speech  in  this  case  was  slow  and  paraphasic;  while  he 
was  able  to  name  most  articles,  some  with  which  he  was  less  familiar 
than  with  others,  he  could  not  name.  He,  therefore,  had  partial  optic 
aphasia  or  word-dumbness.  He  could  not  write  as  well  as  formerly; 
this  appeared  to  be  due  to  the  fact  that  he  could  not,  except  with 
difficulty,  make  out  words  on  the  right  side  of  the  line.     He  understood 

"Barnes,  Review  of  Neurology  and  Psychiatry,  vol.  i.  1903,  p.  531. 


140  THE    EYE    AND    NERVOUS    SYSTEM 

what  he  read;  he  wrote  slowly  from  dictation  and  very  inaccurately, 
few  words  of  more  than  three  letters  were  spelled  correctly ;  the  letters 
were  badly  formed.  He  copied  from  print  to  long-hand  very  slowly. 
In  spontaneously  writing  he  began  Avell,  but  his  efforts  became  worse  and 
worse  and  in  a  half  hour  he  could  not  write  more  than  ten  lines. 

The  temporal  field  of  the  right  eye  was  markedly  contracted  for 
all  colors.  In  the  left  eye  the  nasal  field  was  contracted  about  ten 
degrees. 

The  patient  grew  progressively  worse  until  his  death.  Many  symp- 
toms and  conditions  not  especially  relating  to  the  visual  fields  are 
omitted. 

Necropsy  showed  a  tumor  about  the  size  of  a  small  orange,  occupying 
j)ortions  of  the  postcentral,  superparietal  and  inferior  parietal  convolu- 
tions, extending  into  the  white  matter  but  not  as  far  as  the  ventricles. 
The  growth  was  a  gliosarcoma. 

Admitting  the  existence  of  a  cheiro-graphic  or  cheiro-kinaBsthetic 
centre  distinct  from  all  other  special  centres,  sensory  or  motor,  Barnes 
argues  from  his  case  that  the  connections  between  the  visual  qpntres 
and  both  the  graphic  and  motor  speech  centres,  would  be  severely 
injured,  this  being  accompanied  by  partial  destruction  of  the  visuo- 
auditory  and  cheiro-auditory  commissures.  It  will  be  seen  from  Barnes' 
explanation  that  all  forms  of  writing  would  be  much  impaired,  while 
other  aphasic  symptoms  were  referable  to  partial  injury  of  visuo-audi- 
tory  commissures.  His  partial  right  homonymous  hemianopsia  was 
evidently  due  to  lesion  of  the  optic  radiations. 

Barnes  accompanies  his  paper  with  an  interesting  illustration  which 
is  reproduced  in  Fig.  19. 

Gulbenk  ^'"  has  described  the  case  of  a  patient  with  a  rare  dis- 
turbance of  the  faculty  of  writing,  which  he  terms  dysantigraphia.  A 
man,  seventy  years  old,  came  into  the  hospital  for  difficulty  with  his 
speech  and  inferior  facial  paralysis,  which  had  been  present  for  about 
twenty  days.  Speech  was  slow  and  embarrassed,  the  words  being  badly 
pronounced  but  intelligible;  syllables  were  not  transposed  and  jargon- 
aphasia  was  absent.    He  had  no  paralysis  of  the  limbs. 

The  patient  could  write  pages  fluently  and  legibly  to  dictation ;  but 
if  asked  to  copy,  he  could  only  do  one  or  two  lines  and  then  would  begin 
to  misspell  and  the  writing  would  become  illegible  about  the  fourth  line. 
If  again  dictated  to,  he  could  immediately  write  without  fatigue. 

The  aphasia,  of  which  a  trace  was  present  on  his  admission,  soon 

•  Gulbenk,  Rev.  Neurol.,  February  15,  1904.  Abat.  in  Review  of  Neurology  and 
Psychiatry,  vol.  ii.  1904,  p.  322. 


WORD-BLIA^DKESS:    ALLIED    DISORDERS 


141 


developed  more  and  more,  becoming  almost  complete.     At  this  stage 
writing  was  quite  impossible. 

Gulbenk  holds  that  this  case  shows  that  the  truth  lies  between  the 
view  that  our  hand  copies  visual  images  stored  up  in  the  angular  gyre, 
and  the  other  that  our  hand  translates  directly  into  graphic  signs  the 
sounds  furnished  by  internal  speech.     In  writing  we  use  at  the  same 

Admitting  a  graphic  centre  at 

RIGHT  Cuneus  LEFT 


Angular  gyrus 

-••  Outer  fibres  of  op.  rad. 


time  visual  images  and  these  sounds, 


Op.  radiation 
cp.  quad.  a". 


Blind  part  of  visual 
field  of  R.  eye 


-  Tumor 

Corpora  genieulata 

Visuo-cheirographic 
commissure 

Asc.  frontal  convoL 
L.  op.  tract 


Retina  of  L.  eye 


•Visual  field 


Fig.  19. — Diagram  showing  the  manner  in  which  writing  and  speech  were  impaired  by  destructive 
lesions  involving  the  tracts  between  the  visual  and  the  cheiro-graphic  and  motor  speech  centres,  and 
also  involving  the  visuo-auditory  and  cheiro-auditory  commissures.    (Stanley  Barnes.) 

the  posterior  extremity  of  the  second  frontal  convolution,  *  Gulbenk  ex- 
plains the  symptoms  of  the  case  by  postulating  a  functional  ischemia, 
leaving  intact  the  connection  of  auditory  (temporal  lobe)  with  motor 
images,  but  interfering  with  the  connection  of  visual  images  (angular 
gyre)  with  the  same.  This  ischemia  would  affect  the  connecting  fibres 
in  their  passage  beneath  the  Rolandic  convolutions,  thus  causing  the 
paresis  of  the  inferior  facial.  The  author  compares  dysantigrapli'ia  with 
the  dyslexia  of  Bruns,  where  the  patient,  after  reading  fluently  a  few 
words,  finds  it  impossible  to  continue. 


142  THE    EYE    AND    NERVOUS    SYSTEM 

Numeral-blindness  without  either  letter-  or  word-blindness  is  occa- 
sionally recorded ;  it  would,  perhaps,  be  more  frequently  observed  if 
the  attention  of  the  examiner  was  directed  to  the  subject.  Hinshel- 
wood  reports  four  cases  of  word-blindness  in  only  one  of  which  blind- 
ness for  figures  or  numl)ers  was  present.  In  one  of  these  cases  the 
patient,  a  teacher  of  languages,  became  suddenly  totally  word-blind  and 
letter-blind,  and  yet,  when  he  was  examined  with  the  test-types,  while 
he  could  not  read  a  single  word,  he  could  at  once  recognize  and  repeat 
the  numbers  at  the  top  of  each  paragraph.  In  this  case,  the  lesion,  a 
considerable  area  of  softening,  did  not  involve  the  angular  convolution, 
although  it  did  the  white  matter  of  the  occipital  lobe,  including  the  tract 
from  the  primary  to  the  higher  visual  centres  and  the  optic  radiations 
as  well  as  some  portions  of  the  occipital  cortex. 

Dejerine  ''^  has  recorded  a  case  of  pure  word-blindness  without 
agraphia,  due  to  a  subcortical  lesion,  agraphia  being  added  with  the  sub- 
sequent cortical  lesion,  in  which  case  the  patient  was  totally  unable  to 
read,  and  eventually,  to  write ;  he  retained  the  power  of  reading  figures 
and  could  do  mental  calculations  as  well  as  formerly. 

I  have  seen  cases  of  word-blindness  in  which  the  power  to  recognize 
and  use  numbers  in  calculations  was  retained  in  whole  or  in  part.  In 
my  diagrams,  Figs.  3  and  5,  I  have  indicated  a  number-seeing  centre 
as  distinct  from  that  of  word-seeing  and  letter-seeing,  although  adjacent 
to  both.  Hinshehvood  accords  with  the  view  thus  indicated,  that  the 
groups  of  visual  memories  of  letters  and  figures  are  deposited  in  dis- 
tinct but  probably  adjacent  areas  of  the  cerebral  cortex. 

A  few  words  might  be  said  here  about  the  treatment  of  visual 
aphasia,  and  especially  of  word-  and  letter-blindness.  As  many  of  these 
cases  of  word-blindness  are  the  result  of  cerebral  hemorrhage,  Ilinshel- 
wood  urges  the  importance  of  complete  cerebral  rest,  and  above  all,  of 
preventing  the  patient  from  practising  reading  for  at  least  a  consider- 
able time  after  the  attack.  Many  of  these  cases  are  the  result  of 
syphilis,  and  it  is  wise  to  put  the  patient  on  specific  treatment  if  there 
is  the  slightest  possibility  of  syphilis  having  been  contracted.  When 
actual  destruction  of  the  centre  for  word-seeing  has  occurred,  re-educa- 
tion may  call  the  corresponding  centre  of  the  right  cerebral  hemisphere 
into  function,  but  re-education  should  not  be  attempted  until  all  signs 
of  acute  brain  disease  have  disappeared. 

The  treatment  of  alexia,  due  to  word-  and  letter-blindness  is  some- 


"  I>jerine,  Coniptes-Rendus  des  Stances  de  la  Soci6t€  de  Biologie,  March,  1892. 
p.  04. 


WOED-BLIXDNESS:    ALLIED    DISORDERS  143 

times  disheartening,  but  with  perseverance  much  can  be  accomplished, 
at  least  with  persons  of  intelligence  and  education.  Of  course,  as  audi- 
tory, and  to  some  extent,  other  forms  of  aphasia,  are  commonly  asso- 
ciated with  the  visual  speech  disorder,  training  for  word-deafness,  para- 
phasia and  partial  motor  aphasia,  may  need  to  come  hand-in-hand  with 
that  for  word-  or  letter-blindness,  but  I  shall  speak  here  only  or  chiefly 
of  direct  visual  training. 

The  patient  should  be  made  to  copy  numbers,  letters  and  words, 
separately  and  in  various  juxtapositions.  Efforts  should  also  be  made, 
as  time  progresses,  to  have  him  write  from  dictation  and  spontaneously. 
After  writing  from  dictation  or  copying,  the  patient  should  try  to  write 
the  same  numbers,  letters,  words  or  combinations  by  recalling  them  for 
this  purpose.  With  regard  to  words,  it  is  probably  best  to  begin  with 
nouns,  following  with  verbs,  and  later  with  qualifying  and  conjoining 
words,  which  are  best  taught  by  presenting  them  in  short  sentences. 

Reference  to  the  plan  pursued  in  a  recent  case,  the  one  recorded  by 
Dr.  Weisenburg  and  the  writer,  will  perhaps  give  the  best  idea  of  the 
methods  of  training  of  most  value  for  visual  aphasia. 

Two  methods  are  used  at  the  present  time  in  teaching  children  the 
recognition  of  words.  In  our  Philadelphia  public  schools,  the  word  sys- 
tem is  used, — that  is,  the  child  is  not  taught  to  spell  the  word,  but  to 
recognize  it  en  bloc.  An  apple  is  shown  or  is  drawn  on  the  board.  The 
child  is  told  that  it  is  an  apple,  and  after  this  is  firmly  impressed,  the 
word  apple  is  written  on  the  board,  and  an  attempt  is  made  to  fix  the 
image  of  the  word  in  the  child's  mind.  It  is  claimed  for  this  method 
that  by  it  words  are  acquired  much  faster.  The  child  later  learns  of 
himself  to  apply  the  method  of  spelling,  or,  what  is  by  no  means 
unusual,  never  becomes  a  good  speller. 

In  the  older  method  the  child  is  first  taught  the  alphabet,  and  then 
to  spell  the  word  letter  by  letter,  and  syllable  by  syllable.  While  this 
method  is  a  slower  one,  the  results  are  better  as  regards  spelling.  It 
is  probable  that  a  carefully  considered  combination  of  the  two  methods 
would  work  out  the  best  results. 

Lowenfeld  ''^  records  two  cases  of  amnesic  aphasia  in  which  the 
patient,  in  one  case,  could  not  read  short  but  could  read  long  words ;  in 
the  other  case,  if  the  patient  had  difficulty  in  reading  a  word,  instead  of 
trying  to  spell  it,  he  would  attempt  to  pronounce  it  en  bloc.  This  was 
the  case  with  the  patient  treated  by  Dr.  Weisenburg  and  the  writer. 
He  could  at  times  recognize  words,  and  often  when  he  would  try  to 

"  Lowenfeld.  Ueber  zwei  Ftille  von  amnestischer  Aphasie.  Deutsche  Zeitschr.  f. 
Nen-enheilk..  vol.  ii.  1892. 


144  THE    EYE    AND    NERVOUS    SYSTEM 

pronounce  a  word  he  would  not  attempt  to  either  spell  it  or  pronounce 
it  in  part,  but  as  a  whole.  On  being  asked  to  recall  a  word  with  which 
he  was  familiar,  his  mental  picture  of  the  word  was  of  its  entirety; 
he  could  not  recall  its  literal  components  nor  spell  it.  In  training  him, 
both  the  word  image  and  the  letters  method  were  used.  In  six  weeks  he 
was  able  to  recognize  nearly  all  the  letters  of  the  alphabet.  Considering 
that  he  was  only  able  to  recognize  about  six  letters  when  he  came  under 
our  care,  the  improvement  was  marked.  The  letters  were  impressed  in 
(lifTerent  ways.  He  was  shown  a  letter,  as  p,  made  to  pronounce  it,  to 
write  it,  to  pick  it  out  of  other  letters,  and  to  try  to  picture  it.  At  the 
same  time,  simple  words  were  taught  him  in  the  same  manner;  in  other 
words,  he  was  shown  the  word,  made  to  pronounce  it,  to  write  it,  to  pick 
it  out  of  other  words,  and  try  to  recall  its  appearance  mentally.  The 
])rogress  in  the  recognition  of  letters  and  words  was  about  equal. 

AGRAMMATISM. 

One  who  has  many  opportunities  to  study  aphasics  soon  becomes 
awar.e  that  in  those  cases  in  which  language  is  not  entirely  lost  or  is 
confined  to  a  few  simple  recurring  utterances,  like  "  Yes"  and  "  No"  or 
"  La  la,"  "  Oh  no,"  or  some  profane  ejaculation  like  "  Damn"  or  "  Oh 
God,"  the  chief  disturbance  of  speech  exhibits  itself  in  a  disorder  of 
grammatical  expression.  The  patient,  with  his  mutilated  speech,  tends 
to  recur  to  the  forms  of  expression  which  are  used  by  those  who,  like 
the  Chinese,  have  a  language  which  is  undeveloped  so  far  as  gram- 
matical construction  is  concerned,  or  like  the  imbecile,  the  demented, 
or  the  victims  of  confusional  insanity.  Various  parts  of  speech  which 
are  necessary  to  the  full  expression  of  the  language  ordinarily  employed 
by  the  educated,  or  even  by  those  who  are  largely  uneducated,  are 
omitted.  The  words  used  are  transposed  or  emitted  in  grotesque  con- 
fusion. The  grammar  of  language  has  fallen  into  confusion  or  chaos. 
\Mien  this  disorder  of  speech  is  the  result  of  disease  or  defect,  it  is 
termed  agrammatism  or  akataphasia,  a  subject  which  has  received  large 
attention  from  a  few  German  and  other  writers,  like  Steinthal,  Kuss- 
maul,  and  Pick.  Kussmaul  has  devoted  an  interesting  chapter  to  these 
syntactical  disturbances  of  speech.  He  shows  that  a  speaker,  in  order 
to  express  himself  correctly  in  language,  must  have  an  unbroken  flow 
of  words,  perfect  grammatical  diction,  and  a  correct  arrangement  of 
words.  One  or  two,  or  all,  of  these  requirements  may  be  impaired  or 
lost. 

In  the  production  of  agrammatism  or  akataphasia  it  is  evident  that 
■one  must  take  into  consideration  not  only  the  destruction  of  centres 


AGRAMMATISM  145 

and  of  tracts,  but  also  the  more  or  less  general  disturbance  of  the 
entire  zone  of  speech  as  it  has  been  defined  and  described  in  this  chapter. 
According  to  the  position  and  extent  of  a  lesion,  the  grammatical  or 
syntactical  derangement  may  be  of  this  or  of  that  kind.  ^Yhen  auditory 
speech  centres  or  their  incoming,  outgoing,  or  associating  tracts  are  the 
seat  of  disease,  the  grammatical  as  well  as  the  philological  disturbance 
of  speech  will  be  dominated  by  losses  or  impairments  which  shoAv  that 
auditory  language  has  been  the  subject  of  the  most  severe  and  destructive 
assault;  when  visual  centres  and  tracts  are  the  seat  of  lesions,  visual 
language  will  suffer  the  inost  in  grammatical  as  in  ordinary  graphic 
expression ;  and  in  like  manner,  when  the  anatomical  substrata  of  the 
sphere  of  language  on  its  motor  or  emissive  side  has  borne  the  brunt  of 
disease  or  injury,  the  disarrangement  will  be  most  shown  in  the  power 
of  conception  and  of  expression.  In  each  of  these  cases  grammatical 
construction  will  suffer  to  a  greater  or  less  extent. 

According  to  some  authorities,  these  grammatical  and  syntactical 
disturbances  of  speech,  or  some  of  them,  are  due  to  the  disease  or  defect 
in  speech  centres  concerned  with  the  co-ordination  of  languages ;  accord- 
ing to  others,  the  morbid  phenomena  are  the  result  of  the  dissociation 
of  the  separate  centres  and  areas  concerned  with  language.  Probably 
both  views  have  some  foundation  in  fact. 

As  I  have  elsewhere  pointed  out,"^^  the  recognition  of  the  part  played 
by  the  impairment  or  loss  of  the  power  of  grammatical  construction  or 
arrangement  must  receive  special  consideration  in  attempts  at  the 
restoration  of  speech  by  training.  A  citation  from  this  paper  on  the 
Treatment  of  Aphasia  may  be  of  service  in  this  connection. 

"  It  became  a  matter  of  interest  to  me  in  observing  aphasics  who 
were  striving  to  recover  their  lost  powers  to  note  the  particular  parts 
of  speech  which  were  most  obscured  and  most  difficult  to  regain,  and 
also  the  particular  difficulties  which  attended  their  reacquirement  or 
prevented  this.  A  patient  who  has  so  nearly  lost  language  that  he  has 
only  one  or  two  recurring  utterances  regains,  by  repetition  and  recall, 
word  after  word,  especially  nouns.  Later  a  few  verbs  are  acquired. 
Adjectives,  adverbs,  prepositions,  and  articles,  the  parts  of  speech  whose 
uses  are  to  modify,  limit,  or  express  relation,  are  so  entirely  lost  to  the 
patient  as  to  cause  more  than  anything  else  his  difficulty  in  language. 
The  same  remark  applies  to  conjunctions  like  and  and  hut,  and 
with  a  special  force  to  the  auxiliary  verbs  in  all  their  moods  and  tenses. 


"Mills,    The    Treatment    of    Aphasia   by    Training,    Journal    of    the    American 
Medical  Association,  December  24,  1904. 
10 


146  THE    EYE    AND    NERVOUS    SYSTEM 

The  meaning  and  use  of  such  words  or  combination  of  words  as  is  and 
was  and  has  been  are  in  eclipse  or  lost  to  a  varying  extent.  The  impair- 
ment or  loss  of  the  faculty  of  constructing  sentences  or  propositionizing 
in  language  is  one  of  the  most  important  if  not  the  fundamental  defect. 
While  thought  is  at  times  expressed  by  a  single  word,  the  sentence  is  the 
usual  unit  of  thought  expression,  and  the  aphasic  patient,  or  at  least 
some  aphasic  patients,  find  their  chief  difficulty  in  regaining  those  parts 
of  speech  which  are  concerned  with  qualifying  and  correlating.  The 
grammar  of  language  no  longer  exists  for  them. 

"  Considerations  of  this  kind  led  me  to  the  use  of  primers  and  gram- 
mars in  retraining  of  aphasics,  and  these  with  individuals  previously 
reasonably  educated  are  of  great  value.  Patients  should  be  taught  the 
grammar  as  a  child  is  instructed, — in  other  words,  by  teaching  him  the 
meaning  of  the  different  parts  of  speech  and  the  exact  methods  of  using 
them  in  phrases  and  sentences.  The  significance  and  value  of  the 
qualifying,  relating  and  conjoining  parts  of  speech  should  be  enforced 
by  numerous  examples.  When  the  aphasic,  in  his  efforts  to  repeat  after 
another  or  to  respond  to  what  another  asks  or  indicates,  uses  incomplete 
sentences,  the  complete  sentences  should  be  given  and  explained.  In 
every  possible  way  the  manner  of  using  adjectives,  prepositions,  con- 
junctions, and  auxiliaries  should  be  impressed.  The  patient,  with  the 
book  before  him,  should  be  taught  to  conjugate  verbs,  decline  nouns  and 
pronouns,  compare  adjectives,  and  in  other  ways  go  through  the  routine 
methods  of  studying  language  employed  in  the  schools,  these  being 
modified  by  the  particular  requirements  of  the  case.  The  dictionary 
can  often  be  used  as  a  valuable  adjunct  to  the  grammar,  some  patients 
taking  a  particular  interest  not  only  in  reacquiring  words  in  this  way, 
but  in  thus  learning  their  meaning  and  their  uses  as  parts  of  speech." 

Close  examination  of  the  subject,  especially  in  connection  with  the 
study  of  actual  cases,  shows  that  the  faults  in  language  arrangement 
from  which  these  patients  suffer  are,  in  a  strict  sense,  syntactical  rather 
than  grammatical,  and  the  term  akataphasia  is  probably  more  suitable 
than  that  of  agrammatism.  Syntax  is  "  the  due  form  and  arrangement 
of  words  or  members  of  sentences  in  their  mutual  relations  according 
to  established  usage.  Syntax  includes  the  proper  use  of  parts  of  speech 
and  of  forms  in  their  combinations  to  make  sentences  and  their  proper 
arrangement  or  collocation."  (Century  Dictionary.)  A  consideration 
of  the  numerous  examples  furnished  by  partial  aphasics,  or  by  aphasics 
recovering  from  various  forms  of  disturbance  of  speech  due  to  cerebral 
disease,  shows  that  their  great  impairment  is  of  the  faculty  of  construct- 
ing sentences, — that  is,  of  arranging  the  words  of  a  sentence  in  their 


AGRAMMATISM  147 

proper  position  and  order.  Grammar  is  a  more  general  term,  including 
syntax ;  but  grammatical  mistakes  as  they  are  commonly  understood 
refer  to  the  misuse  of  parts  of  speech  which  may  be  arranged  in  a 
sentence  correctly  from  the  syntactical  point  of  view.  It  is  a  curious 
observation  that  aphasics,  in  the  process  of  re-education,  usually  employ 
the  parts  of  speech  correctly  or  incorrectly  according  to  their  previous 
education, — that  is,  in  so  far  as  they  regain  their  linguistic  powers.. 
Their  sentences  may  be  mutilated.  Xouns  may  be  present  in  consid- 
erable number,  and  they  may  employ  some  verbs  and  some  qualifying 
and  conjoining  words;  but  the  verbs  used,  if  the  patient  is  educated, 
are  of  the  right  mood  and  tense,  and  the  substantives  and  pronouns  are 
of  the  correct  person  and  number.  They  do  not  use  the  plural  for  the 
singular  or  the  present  for  the  past.  The  uneducated,  however,  in 
proportion  to  their  degree  of  education  or  their  lack  of  it  previous  to 
the  onset  of  their  aphasia,  make  grammatical  mistakes.  As  speech  is  re- 
acquired, the  professor  or  Jimmie  Fadden,  whatever  may  be  his  syn- 
tactical reacquirements,  reasserts  himself  grammatically  each  after  his 
own  kind. 

Pick,'^  in  an  article  on  agrammatism  as  the  result  of  a  focal  lesion, 
gives  several  theories  which  have  been  held  regarding  this  symptom. 
He  believes  that  it  is  a  so-called  disorder  due  to  lesion  somewhere  in 
the  cerebral  cortex,  and  holds  that  this  lesion  is  situated  in  the  left 
temporal  lobe  adjoining  Broca's  area.  The  case  reported  seems  to  prove 
this  contention,  for,  although  two  areas  of  softening  were  present  in 
the  left  hemicerebrum,  one  in  the  anterior  part  of  the  left  supratemporal 
convolution  and  the  other  in  the  left  third  frontal  convolution,  Pick 
gives  reasons  for  the  opinion  that  the  lesion  in  the  anterior  portion  of 
the  first  temporal  gyre  was  first  in  order  of  development  and  gave  rise 
to  agTammatism.  Dejerine  and  his  school,  as  is  well  known,  hold  to 
the  sufficiency  of  Broca's  area  for  all  the  purposes  of  motor  speech  and 
writing,  and  contend  that  agrammatism,  like  aphasia  and  agraphia,  is 
only  one  of  the  varieties  of  a  comprehensive  motor  aphasia.  Bernheim  '^* 
has  adopted  the  views  of  Dejerine,  but,  as  shown  by  Pick,  an  important 
study  of  the  cases  reported  by  Bernheim  will  show  that  the  lesion  in 
these  cases  was  in  every  case  in  the  temporal  lobe,  or  at  least  involved 
this  lobe. 

Pick  discusses  the  relations  of  grammatical  expression  to  a  cerebral 
substratum.     Ziehen,  for  instance,  holds  that  grammatical  expression 


"  Pick,  Zeitschrift  f.  Heilk.,  vol.  xxiii.  No.  ii.  1902. 
"Bernheim,  De  1'  Aphasie  motrice,  1901.  Ausdruck  verleihen. 


148  THE    EYE    AND    NERVOUS    SYSTEM 

is  not  dependent  upon  the  integrity  of  a  co-ordinating  speech  centre,  but 
is  rather  the  result  of  associative  combinations.  Pick,  however,  holds 
to  the  existence  of  a  language  co-ordinating  or  grammatical  centre  which, 
as  has  been  indicated,  he  places  in  the  anterior  part  of  the  temporal 
lobe. 

VISUAL    ANOMIA     OR    WORD-DUMBNESS. 

One  of  the  most  interesting  of  aphasic  clinical  phenomena  is  that 
which  is  illustrated  by  the  inability  of  a  patient  having  good  vision  for 
ordinary  purposes,  suffering  from  a  lesion  in  the  cerebral  zone  of  lan- 
guage, to  name  by  sight  an  object  the  use  and  nature  of  which  he 
recognizes.  For  this  symptom,  which  has  long  been  observed  by  clini- 
cians, Freund,''^^  who  made  the  earliest  elaborate  study  of  the  subject, 
suggested  the  name  visual  aphasia.  Another  term  frequently  used, 
especially  by  the  Germans,  is  optic  aphasia.  Merkens  "^^  proposed  to 
call  the  symptom  word-dumbness,  a  term  which  has  much  descriptive 
appropriateness.  It  is,  as  Langdon  "^"^  suggests,  name  memory-loss  or 
anomia,  or  rather  visual  anomia. 

The  preferable  names  are  visual  anomia  or  word-dumbness,  although 
the  term  optic  aphasia  has  probably  had  the  largest  use.  The  expression 
visual  aphasia  employed  by  Freund  is  objectionable,  as  it  may  lead  to  a 
misunderstanding.  I  prefer  to  use  this  name  rather  to  designate  the 
entire  class  of  visual  aphasic  phenomena,  and  in  that  sense  it  has  already 
been  used  in  the  present  chapter  as  a  general  caption  for  all  the  visual 
affections  of  language  which  have  an  organic  cerebral  substratum. 

Occasionally  word-dumbness  is  observed  as  an  isolated  form  of 
anomia, — that  is,  it  is  only  through  sight  that  the  inability  to  name 
kno^vn  objects  is  exhibited;  frequently  the  patient  is  unable  to  name 
objects  which  he  recognizes  through  the  sense  of  sight,  and  is  also 
unable  to  denominate  them  through  other  senses, — as,  for  instance,  that 
of  touch  or  of  hearing.  The  name  memory-loss,  in  other  words,  may 
be  not  only  for  one  but  for  several  or  all  of  the  special  senses.  In  one 
of  my  cases  the  names  of  objects  could  not  be  recalled  either  through 
sight  or  touch,  but  could  by  means  of  hearing.  A  gustatory  or  olfactory 
aphasia  is  possible,  the  patient  being  unable  to  name  a  rose,  for  example, 
through  the  sense  of  smell, — vision  and  the  other  senses  being  excluded, 
an  apple  through  the  sense  of  taste,  with  the  same  sensory  ex- 


"  Freund,  Archiv  filr  Psychiatrie  und  Nervenkrankheiten,  vol.  xx.  1888,  p.  404. 
"Merkens,  Deutsche  Zeitschrift  f.  Chirurgie,  vol.  Ix.,  p.  417,   1000. 
"  Lanf^don,  The  Aphasias  and  their  Medico-Legal  Relations.     The  Laning  Print- 
ing Co.,  Norwalk,  Ohio,  1898. 


VISUAL    ANOMIA  149 

elusions.  In  still  other  cases  the  patient  may  be  unable  to  name  an 
object  with  the  help  of  any  senses,  although  he  recognizes  the  nature 
and  use  of  the  object  through  all  the  senses. 

In  order  to  place  clearly  before  the  reader  the  idea  of  general 
anomia  or  loss  of  naming  power  through  any  of  the  senses,  a  well- 
recorded  case  by  Potts  '^^  is  worthy  of  citation.  A  man  who  was  left- 
handed  for  all  acts  except  writing  lost  the  power  of  naming  objects, 
whether  they  were  seen,  heard,  smelled,  felt,  or  tasted.  He  compre- 
hended all  that  was  said  to  him,  knew  the  names  of  objects,  and  could 
converse  fluently  with  the  exception  that  he  omitted  the  names  of  per- 
sons, places,  and  objects.  He  could  not  repeat  names  pronounced  in  his 
hearing,  but  he  knew  his  own  name  and  address  and  could  write  them ; 
but  otherwise  spontaneous  writing  was  impossible.  He  copied  fairly 
well  but  soon  tired.  He  had  had  an  apoplectic  attack,  the  immediate 
symptoms  of  which,  with  the  exception  of  the  visual  aphasia,  had  dis- 
appeared. In  about  two  months  he  made  such  marked  improvement 
that  he  was  able  to  name  after  reflection  most  objects  presented  to  him, 
and  he  was  also  able  to  write  a  letter.  This  man,  although  word-blind, 
could  read  numerals.  Hemianopsia  was  not  present.  Potts  argued 
that  it  was  necessary  to  assume  in  this  case  either  that  the  lesion  cut  a 
number  of  tracts  leading  respectively  from  the  centre  of  visual,  tactile, 
auditory,  olfactory,  and  gustatory  memories  to  the  motor  centre,  or 
else  that  tracts  from  each  of  these  centres  converged  to  a  common 
centre  where  the  memories  of  names  are  stored  up,  to  be  called  into 
consciousness  by  stimulation  of  one  or  all  of  the  percipient  centres,  and 
that  from  this  centre  a  tract  passes  to  the  motor  speech  centre  and  that 
this  tract  was  destroyed. 

Personal  experience  with  a  number  of  cases  with  necropsies  has 
demonstrated  for  the  writer  the  fact  that  this  symptom  is  present  in 
lesions  variously  situated  in  the  cerebral  zone  of  speech,  as  the  hinder 
part  of  the  left  third  frontal  convolution  and  the  insula,  in  the  third 
and  fourth  temporal  convolutions,  including  both  cortex  and  subcortex, 
at  the  junction  of  the  posterior  thirds  of  the  first  and  second  temporal 
convolutions,  and  again  in  these  convolutions  and  the  adjacent  portions 
of  the  occipital  lobe.  Cases  have  been  recorded  which  indicate  that  the 
symptom  may  arise  from  lesions  situated  in  various  portions  of  the 
occipital  cortex  or  subcortex.  Freund,  after  recording  several  cases  of 
his  own  and  reviewing  the  literature  of  the  subject,  explains  the  mech- 
anism of  the  occurrence  of  the  symptom  with  the  assistance  of  a  diagram. 

"Potts,  Journal  of  the  American  Medical  Association,  May  4,  1901. 


150  THE    EYE    AND    x\ERVOUS    SYSTEM 

Bearing  in  mind  the  facts  already  presented  regarding  the  centres  for 
object-seeing  and  the  various  tracts  and  centres  of  the  cerebral  zone  of 
speech,  the  production  of  the  symptom  from  differently  located  focal 
lesions  can  readily  be  understood. 

It  is  first  necessary  to  recall  the  writer's  views  regarding  a  naming 
centre.  I  have  long  held  that  between  the  sensory  and  motor  cerebral 
centres  for  speech  exists  a  concept  area,  in  which  probably  is  a  naming 
centre.  This  has  been  located  on  theoretical  grounds  and  as  the  result 
of  a  few  clinicopathological  observations  in  the  midtemporal  region. 
Presuming  the  existence  of  this  centre,  a  centre  concerned  with  the 
affixing  of  names  to  concrete  ideas  of  objects  recognized  through  the 
lower  and  higher  centres  for  the  special  senses,  it  is  evident  that  word- 
dumbness  will  occur  as  the  result  of  destructive  lesions  variously 
situated. 

It  has  been  shown  that  in  the  lateral  occipito-parietal  and  occipito- 
temporal regions  is  situated  an  area  concerned  witli  object  recognition; 
also  that  destruction  of  the  whole  or  a  part  of  this  area,  in  some  cases 
on  one  side  of  the  brain  and  in  others  on  both  sides  of  the  brain,  causes 
the  symptom  which  is  known  as  object-blindness  or  mind-blindness.  Of 
course,  if  the  patient  cannot  recognize  objects  he  will  not  be  able  to 
name  them.  The  symptom,  therefore,  may  result  from  a  lesion  of  the 
region  or  regions  of  object  recognition.  Word-,  letter-,  and  number- 
blindness,  for  instance,  are  due  to  a  destructive  lesion  of  the  angulo- 
occipital  region  or  the  tracts  leading  to  it,  and  inability  to  name  words, 
letters,  or  numbers  will  be  present  as  a  part  of  these  forms  of  cortical 
and  precortical  visual  aphasia.  When  objects  other  than  words,  letters, 
and  numbers  cannot  be  recognized,  it  is  probable  that  a  bilateral  lesion 
is  usually  necessary  for  the  production  of  not  only  mind-blindness,  but 
of  visual  anomia.  Tracts  pass  from  the  various  centres  for  object  seeing 
to  the  naming  centre  in  the  left  temporal  lobe.  A  lesion  of  these  tracts 
in  the  left  hemisphere  will  cause  word-dumbness  for  those  objects 
whose  recognition  is  confined  to  this  hemisphere.  For  objects  recognized 
by  both  occipital  lobes  it  will  be  necessary  for  the  complete  production 
of  this  symptom  that  the  tracts  passing  from  both  hemispheres  to  the 
naming  centre  shall  be  destroyed.  This  may  be  brought  about  by  a 
single  lesion  after  the  tracts  unite,  by  bilateral  lesions,  one  in  the  left 
and  the  other  in  the  right  occipital  lobe,  or  one  in  the  left  hemisphere 
and  the  other  in  the  splenium  of  the  callosum,  throng  which  it  is 
probable  that  the  tract  passes  from  the  right  to  the  left  side. 

Lesion  of  the  naming  centre  itself  will  also  cause  this  symptom,  and 
in  this  case  it  is  probable  that  the  word-dumbness  will  be  not  only  for 


VISUAL    ANOMIA  151 

objects  recognized  by  sight,  but  for  those  recognized  by  the  other  senses, 
as  the  tracts  from  all  the  centres  of  the  special  senses  probably  converge 
or  concentrate  in  the  concept  naming  area.  A  case  like  that  reported 
by  Potts  would,  for  instance,  be  most  likely  due  to  a  lesion  situated 
here. 

From  the  naming  centre  a  tract  also  passes  to  Broca's  centre,  and 
destruction  of  this  tract  will  produce  word-dumbness.  It  will  also  be 
caused  by  lesion  of  the  centre  for  word  hearing,  as  the  auditory  verifica- 
tion of  words  used  will  not  be  possible.  Certainly  the  symptom  will 
result  from  lesion  of  Broca's  centre,  to  which  all  tracts  concerned  with 
speech  on  its  sensory  and  concept  sides  pass.  It  is  scarcely  necessary  to 
say  that  anomia  will  be  present  if  a  lesion  destroys  the  tracts  from  the 
retina  to  the  basal  and  cortical  centres  for  sight.  Lateral  hemianopsia 
is  frequently  combined  with  word-dumbness,  because  of  the  juxtaposi- 
tion of  the  optic  radiations  to  the  tracts  and  centres  which  are  concerned 
with  object  recognition  and  the  processes  associated  with  it.  Destruc- 
tion of  the  macular  tracts  and  centres  will,  of  course,  interfere  with  the 
recognition  and  the  naming  of  words,  letters,  and  other  objects. 


CHAPTER    IV. 

GENERAL  EXAMINATION  OF  THE  EXTERIOR  OF  THE  EYE 
AND  THE  REGION  OF  THE  EYE;  PERIMETRY;  COLOR- 
BLINDNESS; PERIPHERAL  BLINDNESS,  AND  SUBJEC- 
TIVE VISUAL  SENSATIONS;    AMBLYOPIA. 

By  CASEY  A.  WOOD,  M.D. 

Inspection  of  the  Eye. — Much  valuable  information  concerning 
the  condition  of  the  eye,  not  only  of  its  external  but  of  its  internal  struc- 
tures, may  be  obtained  by  simple  inspection.  This  is  especially  true  of 
an  examination  made  for  neurologic  purposes;  there  are  many  valua- 
ble signs  of  involvement  of  the  nervous  apparatus  exhibited  by  the 
external  eye  that  may  easily  be  detected  if  the  observer  will  take  a  little 
trouble.  Such  an  examination  must,  of  course,  be  conducted  at  close 
range  and  may  be  aided  by  instruments.  To  be  effective  it  should  be 
methodical  and  a  certain  order  ought  to  be  preserved  in  all  cases.  Great 
gentleness  should  be  observed  and  as  little  handling  of  the  lids  and 
globes  indulged  in  as  possible,  lest  eyes  already  sensitive  be  rendered  so 
irritable  that  further  examination  of  them  is  rendered  difficult,  pain- 
ful or  impossible. 

This  objective  examination  of  the  eye  should  begin  by  seating  the  pa- 
tient in  a  chair  facing  a  window  admitting  indirect  or  diffused  sunlight. 
The  surgeon  should  then  carefully  inspect  the  superficial  structures  and 
parts  surrounding  the  globe.  The  lids  should  first  be  examined  closed. 
Do  their  edges  approximate  readily,  entirely,  and  without  apparent  effort 
on  the  part  of  the  patient  ?  Does  the  dermal  furrow  describe  its  natural 
semicircle  from  one  canthus  to  the  other  ?  Can  the  patient  shut  both  eyes 
tightly,  and  does  the  wrinkled  skin  present  about  the  same  appearance 
on  both  sides  ?  Is  he  able  to  open  both  eyes  to  their  fullest  extent  and 
in  doing  so  is  one  eyebrow  drawn  upward  as  high  as  the  other  ?  Is  there 
contraction,  tonic  or  clonic,  of  the  orbicular  muscle  ?  On  again  assum- 
ing the  primary  position  is  there  drooping  of  either  lid  ?  The  lids  in 
normal  eyes  should  be  in  close  apposition  to  the  globe,  and  the  small 
punda  lachrymalia  should  remain  in  intimate  contact  with  the  eyeball 
in  almost  all  excursions  of  the  latter.  The  margin  of  the  upper  lid 
should  rest  on  the  cornea,  and  this  relation  should  be  preserved  in  the 
downward  movement  of  the  ball;  in  other  words,  there  should  be  no 
152 


EXTERIOK  EXAMIXATIOX  OF  THE  EYE 


153 


exposure  of  the  white  sclera  between  the  upper  lid-edge  and  the  cornea 
proper. 

Incidentally,  one  may  examine  the  lids  for  irregularity  of  their  out- 
line, for  oedema,  distended  vessels,  redness,  tumors,  etc.,  and  for  crusts, 
discharge  or  ulceration  along  their  margins  and  intermarginal  spaces. 


Fig.  1.— Everting  the  lower  lid.    (From  Wood  and  Woodruff.) 


The  eyelashes  also  should  be  inspected,  as  to  whether  they  are  lacking, 
are  abnormally  numerous,  misplaced,  incurved,  etc. 

The  position  and  sufficiency  of  the  lachrymal  drainage  should  be 
determined.  If  there  be  an  overflow  of  tears  its  meaning  should  be 
decided ;  or  as  sometimes  happens,  to  a  paresis  of  the  nerve-supply  to 
the  orbicularis;  whether  it  be  due  to  obstruction  in  some  part  of  the 
lachrymal  passages. 

Both  lids  should  he  everted  and  the  condition  of  the  conjunctivae, 
ocular  and  palpebral,  determined.  To  do  this  thoroughly,  and  without 
hurting  the  patient,  the  tip  of  the  thumb  should  be  placed  on  the  lower 
lid  near  its  margin  and  the  patient  told  to  roll  the  eye  upward.  As  he 
does  this  the  lid  is  pressed  backward,  exposing  the  whole  extent  of  the 


154 


THE    EYE    AND    NERVOUS    SYSTEM 


lining  mucous  membrane.  Complete  eversion  of  the  upper  lid  is  more 
difficult.  The  examiner  stands  behind  the  chair  on  which  the  patient 
sits,  and  the  latter  is  directed  to  look  down  during  the  whole  perform- 
ance. The  forefinger  tip  of  the  left  hand  is  now  placed  upon  the  upper 
lid  just  below  the  eyebrow  and  the  lid  drawn  by  the  finger  up  and  away 


FiQ.  2.— Everting  the  upper  lid,  first  act    (From  Wood  and  Woodruff.) 


from  the  globe  so  that  the  lashes  point  upwards.  The  cilia  and  lid  edge 
are  now  grasped  by  the  forefinger  and  thumb  of  the  right  hand,  care 
being  observed  not  to  touch  or  press  upon  the  eyeball.  The  lid  is  now 
drawn  steadily  down  and  away  from  the  globe  while  the  tip  of  the  left 
forefinger  is  again  slipped  to  the  upper  margin  of  the  tarsus  and  pressed 
doicnuard  and  haclcward.  upon  the  globe.  At  this  moment  the  lid  may 
be  readily  turned  back  over  the  nail  of  the  forefinger,  exposing  the  upper 
sulcus  with  its  covering  of  conjunctiva. 

The  position  of  the  eyeball  in  the  orbit  can  usually  be  determined  at  a 
glance,  although  when  there  is  swelling  of  the  lids  and  periorbital  struc- 
tures the  relations  of  the  globe  to  the  orbital  margins,  or  of  one  eye  to 
the  other,  cannot  always  be  determined  in  this  way. 


EXTERIOR  EXAMINATIOX   OF   THE   EYE         155 

Proptosis,  or  abnormal  protrusion  of  tlie  eyeball,  is  noticeable  only 
when  one  eye  is  more  pi'ominent  than  the  other.  It  is  usually  by  com- 
paring one  orbital  region  with  the  other  that  the  degree  of  exophthalmus 
is  determined.  Where  both  eyes  are  unusually  and  equally  prominent 
it  is  difficult  to  express  such  a  proptosis  in  adequate  terms.  There  are 
so  many  people  who  have  physiologically  prominent  eyes  that  it  is  diffi- 
cult to  do  more  than  express  the  degree  of  protrusion  in  relation  to  sur- 
rounding structures.     Let  the  patient  be  placed  in  the  primarj'^  position, 


Fig.  3.— Everting  the  upper  lid,  second  act.    (From  Wood  and  Woodruff.) 

when  a  straight  edge,  touching  the  cheek  and  supraorbital  ridge,  is  placed 
over  each  globe  parallel  to  and  equidistant  from  the  nasal  crest.  The 
distance  between  the  front  of  the  cornea  and  the  straight-edge  in  each 
eye  can  be  accurately  stated  in  millimetres.  In  some  instances,  not  nec- 
essarily pathologic,  both  cornea  project  beyond  such  a  measuring  stick, 
so  tliat  this  simple  test  cannot  be  applied.  In  these  cases  the  author  has 
found  the  opJithalmometer  of  some  value,  for  the  purpose  of  determining 
whether  one  eye  is  the  subject  of  a  true  exophthalmus  or  not.  The 
patient's  head  being  accurately  and  rigidly  placed  in  the  chin-  and  head- 
rest, the  images  of  the  mires  are  distinctly  reflected  from  one  cornea  and 
the  distance  from  the  external  canthus  of  the  corresponding  eye  to  the 


156 


THE    EYE    AND    NERVOUS    SYSTEM 


central  point  at  the  end  of  the  telescope  carefully  measured.  The 
observation  is  repeated  on  the  other  side.  The  difference  between  the 
two  measurements  expresses  the  amount  of  proptosis  in  the  more  promi- 
nent eye. 

Various  proptometers  have  been  devised,  but  none  of  them  furnishes 
more  useful  information  than  the  methods  just  described. 


P^G.  4. — Oblique  or  focal  illumination.    (From  Posey  and  Wright.) 


Oblique  Illumination.  — The  anterior  segment  of  the  eyeball  is  most 
satisfactorily  and  thoroughly  explored  by  the  aid  of  a  convex  lens,  of 
about  six  centimetres  focus,  and  a  source  of  light  in  a  darkened  room. 
The  rays  from  an  argand  burner  or  an  electric  lamp,  placed  about  a 
metre  to  the  side  and  a  little  to  the  front  and  above  the  patient's  head, 
are  allowed  to  fall  on  the  eye  looking  straight  in  front.  The  lens  is 
held  so  that  the  rays  of  light  fall  at  right  angles  upon  it  and  are 
focused  obliquely  on  the  eyeball.  The  eye  is  now  moved  in  various 
directions  so  that  the  whole  surface  of  the  cornea  and  globe  is  illu- 
minated by  the  pencil  of  light.  In  tliis  way  not  only  the  external  struc- 
tures of  the  globe  but  the  iris,  anterior  chamber,  pupil,  and  lens  are  thor- 
oughly illuminated  and  explored  without  producing  any  of  the  trouble- 
some reflections  that  embarrass  the  observer  when  the  rays  fall  upon  the 
globe  from  a  source  of  light  situated  directly  in  front  of  the  patient.  The 
value  of  this  examination  is  enhanced  by  placing  before  the  observer's 
eye  another  magnifying  loop  of  the  same  focus,  with  which  to  inspect 


EXTERIOR   EXAMINATION   OF   THE   EYE         157 

the  area  lighted  with  the  aid  of  the  other  lens.  This  lens  may  be  held 
in  front  of  the  observer's  eye  with  one  hand  while  the  other  directs  the 
illuminating  rays  upon- the  corneal  surface  (Fig.  4). 

Field  of  Vision. 

There  is  no  examination  of  the  ocular  functions  of  greater  value  to 
the  neurologist  than  the  exploration  of  the  extent  and  other  characters 
of  the  visual  field.  Although  the  test  is  a  subjective  one  and  dependent 
somewhat  upon  the  intelligence  and  cooperation  of  the  patient  it  is, 
when  undertaken  with  proper  care  and  a  due  regard  to  the  requirements 
of  each  case,  difficult  to  overestimate  the  assistance  it  affords  in  diag- 
nosis. The  author  entirely  agrees  with  Wilbrand  ^  that  "  the  measure- 
ment of  the  visual  field  and  the  registration  of  the  results  in  the  form 
of  a  map  have  made  it  possible  to  represent  graphically  the  intensity 
and  extent  of  existing  visual  disturbances  and  to  compare  these  pictures 
with  one  another.  It  can  be  proved  that  certain  defects  in  the  field  of 
vision  correspond  with  certain  disease-processes.  Moreover,  a  more 
precise  diagnosis  has  been  rendered  possible  by  this  method  in  lesions 
of  those  parts  of  the  optic  tract  in  which  the  ophthalmoscope  cannot 
afford  any  assistance.  Perimetry  has  obtained  a  still  greater  diagnostic 
importance,  since  careful  study  of  the  results  of  microscopical  examina- 
tion, with  exact  sketches  of  the  field  of  vision,  has  widened  our  knowl- 
edge about  the  partial  course  of  the  fibres  in  the  optic  tracts,  and  seems 
about  to  fix  the  distinguishing  symptoms  under  which  neuritis,  primary 
atrophy,  and  purely  functional  disturbances  make  their  appearance  in 
the  visual  field,  as  well  as  with  regard  to  their  prognosis  and  treatment." 

On  the  other  hand,  it  must  be  equally  remembered  that  hasty  and 
injudicious  measurements  of  the  visual  field  are  likely  to  be  valueless, 
misleading  and  harmful. 

Various  forms  of  the  instrument  (perimeter)  used  for  determining 
the  extent  and  character  of  peripheral  vision  have  from  time  to  time 
been  devised,  but,  for  all  practical  purposes,  they  may  be  divided  into 
two  classes,  first,  those  intended  for  patients  who  can  sit  upright  during 
the  examination  without  much  general  or  ocular  fatigue ;  second,  those 
by  which  the  measurements  are  taken  while  the  patient  is  in  the  prone 
position.  The  usual  form  of  the  perimeter  employed  in  the  first  class 
of  cases  is  that  devised  by  McHardy,  Landolt,  Skeel,  and  others.  The 
author  prefers  the  first  mentioned.  It  consists  of  a  semicircular  arc  or 
band  which  revolves  upon  its  centre  so  that  it  can  be  placed  in  the  direc- 

*  Norris  and  Oliver.  System  of  Diseases  of  the  Eye.  vol.  ii.  p.  191. 


158  THE    EYE    AND    NERVOUS    SYSTEM 

tion  of  any  meridian.  This  arm  is  divided  into  degrees,  beginning  with 
0  at  the  centre  and  extending  to  90°  at  either  extremity.  At  the  centre 
of  the  semicircle  is  a  small  object  {fixation  point)  for  the  eye  to  look 
at.  The  patient's  head  is  supported  by  a  chin-rest,  one  eye  is  covered, 
and  the  other,  under  examination,  directed  to  look  at  the  fixation  point 
at  the  centre  of  the  semicircle.  A  small  white  or  colored  object,  vary- 
ing from  5  to  20  millimetres  square,  is  then  carried  from  the  periphery 
along  the  inner  surface  of  the  arc.     The  point  at  which  it  first  comes 


Fig.  5.— The  McHardy  Perimeter.    (From  Posey  and  Wright.) 

into  view  in  each  meridian  denotes  the  limit  of  peripheral  vision  for  that 
meridian.  These  points  (marked  on  a  chart)  when  joined  by  lines  give 
the  boundary  of  the  field  of  vision.  If  any  defect  is  found  during  the 
examination  the  movable  objects  will  appear  blurred  or  disappear  en- 
tirely.    This  defect  is  known  as  a  scotoma. 

A  physiological  scotoma  is  present  in  every  field  of  vision.  It  cor- 
responds to  the  optic  nerve  entrance  and  is  situated  about  10°  to  the 
outer  side  of  the  point  of  fixation,  and  is  kno^vn  as  the  blind  spot  of 
Mariotte.  When  a  jierimeter  cannot  be  obtained  the  field  of  vision  may 
be  roughly  measured  by  the  hand  in  the  following  manner:   tho  patient 


EXTERIOK  EXAMINATION   OF   THE   EYE         ir.9 

and  the  examiner  face  one  another,  the  former  with  his  back  to  the  light. 
If  the  left  eye  is  to  be  tested,  the  other  eye  is  covered  with  a  towel.  The 
examiner  closes  his  right  eye  and  each  looks  into  the  other's  eye  at  a 
distance  of  about  a  metre.  The  hand  is  then  carried  to  the  periphery  of 
the  field  at  an  equal  distance  between  the  patient  and  himself.  It  is 
then  moved  slowly  inward  towards  the  visual  axis  and  the  patient  is 
required  to  say  when  he  sees  the  finger-tips.  This  is  repeated  in  the 
various  meridians  of  the  field  of  vision.  If  the  patient  sees  the  fingers 
at  the  moment  they  are  also  seen  by  the  examiner,  his  field  of  vision  is 
normal.  If  the  fingers  are  not  seen  until  they  are  nearer  the  visual  axis 
than  when  seen  by  the  examiner,  there  is  some  defect  in  the  patient's 
field  of  vision.  The  perimeter-arc  and  the  test  objects  are  best  illu- 
minated by  ordinary,  diffuse  daylight  from  a  window,  to  which  the 
patient  presents  his  back.  'Wlien  that  is  not  obtainable  gas  or  electric 
lamps  may  be  employed,  care  being  observed  that  the  illumination  is 
uniform  in  the  various  positions  of  the  arm  of  the  perimeter. 

Myles  Stand ish  employs,  as  a  rough  test  for  absolute  defects  in  the 
visual  field,  a  small  electric  lamp  and  conducts  the  examination  in  a 
dark  room.  One  eye  of  the  patient  is  completely  excluded  by  means  of 
a  folded  towel  held  over  the  orbital  region  by  the  palm  of  the  patient's 
hand  while  he  fixes  the  observer's  nose  with  the  uncovered  eye.  An 
electric  lamp,  of  the  smallest  size,  is  employed  as  the  test-object  instead 
of  the  finger-tips  in  the  preceding  test. 

Sweet  has  devised  a  perimeter  in  which  the  objects  are  white  and 
colored  electric  lamps,  but  the  writer  has  not  used  it. 

On  every  perimeter  chart  used  for  mapping  out  a  field  of  vision,  a 
note  should  be  made  roughly  stating  the  kind  and  amount  of  illumina- 
tion emj)loyed,  such  as  "  bright  day,"  "  dull  daylight,"  "  electric  light," 
etc.,  as  the  size  of  the  field  is,  in  some  instances,  much  affected  tliereby. 

Another  rough  means  of  defining  the  limits  of  the  field  of  vision  is 
the  ordinary  school  blackboard.  Although  theoretically  insufficient  it 
is  a  fairly  good  substitute  for  the  perimeter.  In  such  cases  black  rods 
with  round  or  square  tips,  or  pieces  of  chalk,  white  and  colored,  are 
employed  as  test  objects.  While  the  patient  fixes  a  white  chalk  mark 
on  the  blackboard,  these  objects,  moved  about  as  on  the  arc  of  the  perim- 
eter, map  out  the  visual  field. 

The  portable  perimeter  of  Wilbrand,^  or  one  like  it,  is  valuable  in 
the  examination  of  bedridden  patients.  It  is  used  in  the  wards  of  a 
hospital  or  on  occasions  when  the  stationary  instrument  is  not  accessible. 

'  Norris  and  Oliver,  System  of  Diseases  of  the  Eye,  vol.  ii.  p.  210. 


160 


THE    EYE    AND    NERVOUS    SYSTEM 


The  inventor  terms  it  a  he d- perimeter.  "A  wooden  board  (a),  the  breadth 
of  which  is  equal  to  the  length  of  the  perimeter,  and  the  length  of  which 
equals  the  width  of  the  bed,  is  made  so  that  it  can  be  raised  or  lowered 
like  a  reading  desk.  A  second  board  (6),  which  can  be  placed  at  any 
degree  of  inclination  upon  two  perforated  irons  (c),  is  supported  by 
putting  a  little  iron  pin  {d)  into  a  series  of  holes.  Upon  the  board,  h, 
is  placed  the  perimeter.  The  board,  6,  is  connected  with  the  board,  a, 
on  the  side  turned  towards  the  patient,  by  means  of  two  hinges,  which 
are  placed  near  the  corners.  Both  boards  between  the  hinges  are  hol- 
lowed out  so  as  to  fit  the  regions  of  the  chest  and  abdomen.  The  perim- 
eter itself  is  supported  below  by  a  clamp  (/)  ,which  is  fixed  vertically 


Fig.  6.— VVilbrand's  bed-perimeter.    (From  Norris  and  Oliver.) 


to  {h).  The  board  (a)  is  supported  by  four  wooden  screws  (^),  which 
are  fastened  by  pairs  to  a  rather  narrow  board  (7t)  resting  horizontally  on 
the  lateral  bed-boards.  On  the  board  (/^),  near  the  outer  end  is  placed  a 
clamp  (^'),  so  as  to  prevent  the  whole  apparatus  from  moving  sideways. 
A  head-rest  is  so  arranged  that  the  chin  of  the  patient  can  be  brought  into 
a  comfortable  position  on  the  chin-rest  of  the  instrument." 

The  best  results  in  perimetry  can  be  obtained  only  by  the  exercise  of 
much  patience  and  the  expenditure  of  considerable  time.  Many  neu- 
rologic patients  after  a  short  examination  exhibit  the  so-called  "  retinal 
fatigue"  and  many  others  have  such  dull  peripheral  vision  that  the  limits 
of  their  fields,  especially  for  colors,  are  not  easily  defined.  Again,  it  is 
not  an  easy  task  for  either  the  examiner  or  the  examined  to  locate  and 
measure  scotomata,  particularly  if  they  are  small  or  incomplete.     Fre- 


EXTERIOE  EXAMI:N^ATI0X   OF   THE   EYE         161 

quent  pauses  should  be  made  during  the  examination,  so  that  the  patient 
may  rest  his  eyes,  and  if  fatigue-signs  are  marked  the  completion  of  the 
examination  should  be  deferred  until  another  sitting.  Unfortunately,  it 
is  in  just  such  instances  that  control  experiments  should  be  made,  that 
is  to  say,  it  is  in  cases  exhibiting  a  "  fatigue"  neurosis  that  one  must  be 
most  careful  to  repeat  his  observations  and  where  it  is  desirable  that 
there  should  be  no  "  nerve-tire"  produced  by  the  efforts  of  the  physician 
to  conclude  an  examination.  The  patient  should  be  encouraged  to  close 
his  eyes,  sit  back  in  his  chair,  and  rest  himself  as  often  as  need  be. 

These  difficulties  are  further  increased  when,  for  any  reason,  the 
patient  is  unable  to  see  the  fixation  point  on  the  perimeter.  It  is  then 
advisable  to  have  him  place  the  tip  of  his  forefinger  over  the  point  of 
fixation  and,  by  virtue  of  his  sense  of  locality,  keep  his  eye  in  the 
primary  position.  The  examiner  must  watch  him  closely  to  see  that 
his  gaze  does  not  wander. 

Conditions  that  Determine  the  Limits  and  Other  Characters  of 
the  Visual  Field. — It  is  particularly  desirable  to  remember,  in  examin- 
ing neurologic  cases,  that  a  number  of  factors  enter  into  the  results 
obtained  by  a  perimeter  and  that  many  conditions  may  modify  the  shape 
and  area  of  the  field.  Our  standard  should  be  the  extent  and  boundaries 
of  the  visual  field  in  the  normal,  emmetropic  eye.  Although,  as  before 
stated,  the  examination  is  essentially  a  subjective  one  we  cannot  always 
obtain  the  same  results  in  every  measurement  even  in  the  same  patient 
or  from  observations  of  the  same  eye,  but  the  error  is  generally  so 
slight  that  it  can  be  ignored,  or  at  least  borne  in  mind  when  considering 
the  total  value  of  the  evidence  presented  by  this  and  other  means  of 
diagnosis. 

The  shape  of  the  normal  visual  field  is  an  irregular  oval,  due  to  the  fact 
that  the  retina  extends  farther  forward  on  the  nasal  than  on  the  temporal 
side,  thus  pushing  the  limits  on  the  perimeter-map  correspondingly  out- 
w^ard. 

The  boundaries  of  the  field  of  vision  are  also  affected  by  the  promi- 
nence of  the  eyeball  in  its  socket,  by  the  tip  and  bridge  of  the  nose,  the 
cheek  and  even  by  the  size  of  the  lips  and  moustache.  They  are  often 
measurably  widened  by  opening  the  eyes  to  the  fullest  extent,  and  there 
is  no  doubt  but  that,  other  things  being  equal,  a  larger  field  is  found 
with  a  dilated  pupil  than  with  a  narrow  one.  Owing  to  the  long  antero- 
posterior axis  in  myopia  and  the  shorter  optic  axis  in  Tiypermetropia  the 
field  is  smaller  in  the  former  and  larger  in  the  latter  case.  Bearing  these 
facts  in  mind  one  should  always  have  the  patient  under  examination 
wear  his  correcting  lenses  and  not  hesitate  to  secure  a  full  measurement 
11 


162 


THE    EYE    AND    NERVOUS    SYSTEM 


of  the  field  by  asking  him  to  open  his  eyes  wide,  or  separate  his  lids 
with  the  fingers.  The  other  difiiculties  may  to  some  extent  be  overcome 
by  throwing  the  head  first  backward  and  then  inward  during  the  exam- 
ination of  the  vertical  and  horizontal  meridians  respectively. 

The  extent  of  the  field  for  color  varies  with  the  character  of  the  pig- 
ment used,  the  size  of  the  object,  the  degree  of  illumination,  the  nature 
of  the  background  against  which  it  is  displayed  and  with  individual 
jieculiarities.     For  example,  women,  with  a  more  acute  perception  of 


Eight  Eye 


Fi<j.  ".—Normal  field  of  vision,  right  eye. 


white; blue; 


red ; green. 


(From  Poeey  and  Wright). 


color,  have  larger  color-fields  than  men.  For  these  reasons  the  charts 
found  in  most  text-books  exhibit  considerable  variations  as  to  the  extent 
of  the  color-fields.  Yellow  always  has  the  largest  area  in  the  chart  of  the 
normal  eye ;    then  comes  blue,  then  red,  then  green. 

Although,  in  the  normal  eye,  the  size  of  the  field  for  white  (taken 
with  a  five-millimeter  object)  varies  little  with  the  illumination,  quite 
the  opposite  is  the  case  when  color  tests  are  used.  This  fact  should 
especially  be  remembered  in  testing  for  nervous  disorders. 

Variations  in  the  size  of  the  field  for  white  in  pathologic  states  with 
different  degrees  of  illumination  are  not  infrequent.  We  see  this  espe- 
cially in  the  different  forms  of  hemeralopia  as  encountered  in  retinitis 
pigmentosa,  disseminated  choroiditis,  high  degrees  of  myopia,  secondary 
glaucoma,  luetic  chorio-retinitis  and  tlie  like  (See  Chapter  VIII). 


EXTEKIOR  EXAMINATION  OF  THE  EYE 


163 


Much  has  been  written  upon  the  need  of  standardized  color-objects 
for  the  perimeter  and,  doubtless,  greater  scientific  accuracy  would  be 
attained  if  these  always  exhibited  the  same  color  intensity  or  satura- 
tion. Colored  test-objects  should  be  as  small  as  is  compatible  with  dis- 
tinct vision ;  in  cases  of  amblyopia  they  may  be  as  large  as  20  milli- 
metres square.  It  is  well  to  remember  that  scotomata,  especially  of 
limited  area,  are  best  mapped  out  by  the  use  of  much  smaller  objects, 
whose  sides  measure  only  two  or  three  millimetres. 

The  author  has  long  been  in  the  habit  of  using  a  single  strand  of 
white,  red,  green  and  blue  wool  for  detecting  the  presence  of  central 

Left  Eye 


Fig.  8.— Normal  field  of  vision,  left  eye. 


white; .-  blue; 


.  —  red ; 


(From  Posey  and  Wright. ) 


color  scotomata.  Double  the  thread  between  the  thumb  and  finger  of  the 
right  hand,  holding  it  so  that  only  a  quarter  of  an  inch  of  the  loop  pro- 
jects and  is  seen.  Patient  and  surgeon  should  be  seated  opposite 
one  another.  Cover  the  patient's  left  eye  with  a  closely-fitting  shade, 
and  ask  him  to  fix  steadily  the  observer's  right  pupil  at  a  distance  of 
two-thirds  of  a  metre.  Interpose  the  small  loop  of  wool  in  the  common 
line  of  vision  at  a  point  a  few  centimetres  in  front  of  the  examiner's 
eye.  In  relative  central  scotomata  the  patient  will  not  recognize  the 
color  of  the  small  loop  (or  it  will  appear  less  distinct)  until  it  is  removed 
a  few  millimetres  to  the  right,  left,  up  or  down  from  the  line  of  sight, 
and  the  distance  it  is  necessary  to  carry  the  test  loop  in  the  various 


164 


THE    EYE    AND    NERVOUS    SYSTEM 


directions  before  the  color  is  recognized  will  give  an  idea  of  the  size  and 
shape  of  the  scotoma.  This  rough  test  should  then  be  applied  to  the  left 
eye,  after  which  the  defect  so  detected  can  be  properly  charted  by  means 
of  the  perimeter. 

The  size  of  the  field  to  some  extent  depends  upon  the  manner  in  which 
it  is  measured — another  argument  in  favor  of  always  making  perimetric 
examinations  under  conditions  and  in  a  fashion  as  closely  resembling  one 
another  as  possible.  A  uniform  plan  will  give  definite  information  and 
satisfactory  results,  while  haphazard  and  varied  measurements  will  only 
confuse  and  discourage  the  observer.     For  example,  it  has  long  been 

Left  Eye 
IS.   «L 


90  7* 

Fig.  9.— Central  scotoma  as  seen  In  toxic  amblyopia.    (From  Posey  and  Wright) 


known  that,  in  pathologic  cases  especially,  there  is  a  zone  of  doubtful 
form-  and  color-perception  about  the  boundary  line  of  most  fields. 
Within  this  zonular  region  both  the  observed  and  the  intelligent  patient 
remark  of  the  test-object  that  "  now  he  sees  it  and  then  he  doesn't  see 
it."  This  must  be  borne  in  mind  when  repeated  examinations  are  made ; 
also  that  with  ocular  conditions  unchanged  the  patient  will  (within 
certain  limits)  report  a  gradually  widening  area  of  color-  and  white-per- 
ception the  more  frequently  he  is  examined.  To  a  slight  extent  practice 
increases  the  ability  of  the  patient's  perceptive  elements. 

The  field  is  larger  when  the  test-object  is  moved  from  the  centre  to  the 
periphery  than  when  it  is  carried  in  the  opposite  direction ;    or  when  the 


EXTEKIOK  EXAMINATION   OF   THE   EYE 


165 


object  is  "  wiggled,"  or  kept  in  motion,  about  the  zone  of  doubtful  per- 
ception. Of  course,  it  is  wider  when  a  large,  shiny  object  is  employed 
than  when  a  dull,  small  one  is  used. 

Central  scotomata  result  from  disease  of  the  macular  fibres.  These 
are  termed  absolute  central  scotomata  when  all  light  perception  is  want- 
ing. Defects  for  one  or  more  colors  only  are  called  relative  central 
scotomata.  When  white  is  seen  as  "  grayish,"  or  colors  are  appreciated 
only  as  tints  of  the  test-object,  the  defects  are  known  as  inaccurate  cen- 
tral scotomata.  Finally,  Forster  adds  the  terms  positive  and  negative 
central  scotoma,  defining  the  former  to  be  that  condition  in  which  the 
patient  reports  a  dark  spot  covering  the  fixation-object  and  the  latter 
when  the  point  of  fixation  appears  blurred  or  indistinct.  The  precise 
conditions  of  the  central  field  should  always  be  indicated  on  the  perim- 
eter chart  or  noted  on  its  margin. 


FiQ.  10.— Ring  scotoma.    (From  Norris  and  Oliver.) 

The  foregoing  distinctions  are  now  and  then  valuable  in  diagnosis, 
because  negative  central  scotomata  are  generally  the  result  of  disease  of 
the  papillo-macular  bundle  of  optic  nerve-fibres,  while  positive  defects 
are  generally  due  to  peripheral  alterations  in  the  macular  region,  often 
chorio-retinitic  changes.  Among  the  numerous  forms  of  central  scotoma 
an  important  one  for  the  neurologist  to  remember  is  that  due  to  "  retinal 
fatigue,"  not  infrequently  seen  in  functional  nervous  diseases  (See 
Page  108). 

The  position  of  central  scotomata  with  reference  to  the  point  of 
fixation  is  indicated  by  the  terms  paracentral,  when  the  defect  lies  mostly 
without  the  centre,  and  pericentral,  when  the  fixation-point  forms  the 
centre  of  the  defect. 


IGC) 


THE    EYE    AND    NERVOUS    SYSTEM 


Enlargement  of  the  blind  or  Mariotte's  spot  must  not  be  overlooked  in 
searching  for  defects  in  the  visual  field.  This  may  be  due  to  congenital 
conditions,  such  as  the  presence  of  opaque  nerve-fibres,  but  it  is  also  one 
of  the  early  signs  of  certain  forms  of  optic  atrophy,  of  chorio-retinitis 
of  the  fibres  surrounding  the  nerve-head  and  it  is  seen  in  most  cases  of 
"  choked  disk." 

Ring  Scotoma. — The  area  lying  between  the  fifteenth  and  forty-fifth 
parallels  of  latitude  is  called  the  intermediate  zone.  It  is  occasionally 
subject  to  fairly  well  defined  defects,  which,  enclosing  an  otherw^ise 
normal  central  field  of  zone,  constitute  a  "  ring"  scotoma  (Fig.  10). 


Fig.  11.— Defect  confined  to  the  peripheral  zone  of  the  visual  field.    ( From  Norris  and  Oliver.) 

This  annular  defect  is  sometimes  found,  of  varying  width  and  shape, 
in  syphilitic  lesions  of  the  chorioid  and  retina,  in  multiple  sclerosis  of 
the  optic  nerve  and  a  few  other  conditions. 

The  peripheral  zone  of  the  visual  field  may  be  defined  as  that  lying 
between  the  forty-fifth  parallel  and  tlie  extreme  limits  of  the  field.  Wil- 
brand  says  that  it  generally  begins  in  a  normal  eye  where  the  perception 
of  a  fivc-millinieters  blue  object  ceases.  Isolated  defects  in  this  area  do 
not  disturb  the  vision  of  the  patient  to  any  extent,  and  we  are  mostly 
concerned  with  it  in  the  diagnosis  and  prognosis  of  optic  and  other 
nervous  aifoctions.  When  the  whole  or  most  of  the  peripheral  zone  is 
insensitive  to  light  patients  realize  the  value  of  indirect  peripheral 
vision.  They  act  and  feel  as  if  they  were  seeing  through  a  tube,  and 
when  the  contraction  extends  still  further  towards  the  centre  "  telescopic 
vision"  is  the  result,  obliging  the  one  so  afflicted  to  look  about  him  con- 
tinually to  obtain  satisfactory  sight  (Fig.  11). 


EXTERIOR  EXAMIXATI0:N^  OF  THE  EYE 


1G7 


Hemiopia,  or  (better)  Hemianopsia,  is  characterized  by  loss  of  one 
half  or  portions  of  half  the  field  in  each  eye.  It  may  result  from  disease 
in  any  part  of  one  optic  conducting  path  from  the  calcarine  fissure  to 
and  including  the  chiasma  (Figs.  13,  14).  It  is  almost  invariably 
homonymous, — i.e.,  affects  the  optic  radiations  supplied  to  the  two  eyes 
from  one  side  of  the  brain  without  involvement  of  either  fixation-point. 
Binasal,  bitemporal,  or  heteronymous  hemianopsia  is  quite  rare.   When 

Left  Eye 


Fig.  12. — Scotomata  as  seen  in  disseminated  chorioiditis.    (From  Posey  and  Wright.) 

one  considers  for  a  moment  the  anatomic  arrangement  of  the  crossed 
and  uncrossed  fibres  that  constitute  each  optic  nerve  the  explanation  of 
homonymous  hemianopsia  is  easy  (See  Chapter  I). 

Of  154  cases  of  homonymous  hemianopia  collected  by  Wilbrand  74' 
were  on  the  right  side  and  80  on  the  left.  The  line  dividing  the  blind 
from  the  seeing  half  of  the  retina  does  not,  as  a  rule,  pass  directly 
through  the  fixation-point.  Fortunately,  the  functionating  half  usually 
includes  the  fixation-point, — i.e.,  the  lesion  does  not  affect  the  fovea, 
because  there  are  sufficient  unaffected  fibres  from  the  normal  visual 
centre  supplied  to  each  papillo-macular  bundle  to  permit  of  good  central 
vision.  Inasmuch  as  the  arrangement  and  distribution  of  the  crossed 
and  uncrossed  bundles  varies  greatly  in  individuals  the  position  of  the 
frontier-line  also  varies  accordingly.  As  a  rule,  this  boundary  runs  in 
an  almost  perfectly  vertical  direction  until  it  approaches  within  5°  to 
10°  of  the  fixation-point.     It  then  diverges,  curves  around  the  central 


1G8 


THE    EYE    AND    NERVOFS    SYSTEM 


point  and  at  the  same  distance  below  once  more  resumes  its  vertical 
direction.  The  functionating  area  about  the  point  of  fixation  may  have 
a  considerable  radius — from  3°  to  10°,  or  even  more. 

The  variations  in  the  line  dividing  the  seeing  from  the  non-seeing 
retina  corresjwnd  to  variations  in  the  size  of  the  hemiopic  fields,  only,  as 
a  rule,  the  same  part  of  the  field  is  affected  in  each  eye  even  if  they  vary 
much  in  extent.  Later,  part  of  the  field  may  be  recovered  on  one  side 
■while  undergoing  no  change  in  the  opposite  eye,  rendering  the  contrast 
between  the  two  perimeter  charts  still  more  marked  than  before. 

Right  Eye 


Fig.  13.— Field  In  left-sided  hemianopsia.    (From  Posey  and  Wright) 

Incomplete  hemianopsia  is  due,  in  the  majority  of  instances,  to 
•  destructive  lesions  of  the  occipital  lobe.  Defects  confined  to  either  the 
upper  or  the  lower  quadrants  of  the  field  indicate  destruction  of  the 
lower  or  uj)per  elements  of  the  cuneus  (Figs.  15,  IG). 

Tlie  jx3riphery  of  tlie  unaffected  half  of  the  field  in  hemiopia  may  or 
may  not  be  contracted  and  the  half-field  is  usually  smaller  on  the  side 
opposite  the  lesion  (See  Chapter  TX,  p.  391). 

The  field  of  vision  in  binocular  fixation  is,  of  course,  larger  than  that 

of  each  eye  taken  separately.     Although  it  is  the  field  generally  consid- 

,  ered  by  the  patient  and  ought,  consequently,  to  be  of  particular  interest 

to  the  neurologist,  its  practical  value,  apart  from  the  subject  of  squint,  is 

not  very  great. 

Hemichromatopsia. — In  true  hemianopsia  perception  of  color  is  lost 


EXTEEIOR   EXAMINATIOX   OF   THE   EYE         169 

with  the  field  for  white.  In  rare  cases,  however,  there  is  a  true  kemia- 
chromatopsia,  in  which  the  white  field  is  unaffected  (or  white  objects  are 
seen  as  gray),  the  dividing  line  passing  through  the  fixation-point.  In 
explanation  of  this  curious  condition  Wilbrand  claims  that  there  is  a 
centre  for  colors  distinct  from  that  for  form  or  from  that  for  white. 
Gowers  suggests  that  instances  of  hemichromatopsia  would  be  more 
frequently  noted  if  the  color  fields  were  tested  at  the  bedside  in  all  cases 
of  cerebral  disease  (See  Page  392). 

An  admirable  summary  of  the  diagnostic  values  of  defects  in  the 
visual  field  is  given  by  Gowers.^     Loss  of  the  pupillary  reaction  with 


90  '" 

Fig.  14.— Field  in  left-sided  hemianopsia.    (From  Posey  and  Wright.) 

defective  sight  in  one  eye  without  affection  of  the  opposite  organ  indi- 
cates disease  of  one  optic  nerve.  A  central  scotoma  usually  means  dis- 
ease of  the  macular  bundle  of  optic  fibres,  either  in  the  body  of  the 
nerve  or  at  the  fovea  itself.  Peripheral  limitation  of  the  field  may  be 
due  to  (a)  damage  to  the  peripheral  layer  of  the  nerve  or  (&)  a  lesion 
of  the  whole  nerve,  which  shows  itself  only  in  the  periphery,  where 
vision  is  normally  duller  than  it  is  about  the  macular  region.  A  sector- 
liJce  defect,  confined  to  the  field  of  one  eye  indicates  destruction  of  a 
portion  of  one  nerve.  An  amblyopia,  with  concentric  limitation  of  the 
field,  marked  in  one  eye  but  slight  in  the  other,  is  due  either  to  atrophy 


Diseases  of  the  XerA'ous  System,  vol.  ii.  p.  162. 


170 


THE    EYE    AND    NERVOUS    SYSTEM 


or  to  disease  in  one  hemisphere.  Tn  the  first  instance  the  pupillary 
reaction  is  sluggish  and  the  ophthalmoscope  generally  reveals  the 
atrophic  changes ;  in  the  second  the  nerve  appears  normal,  the  pupil  acts 
promptly  to  light,  the  onset  of  the  visual  disturbance  is  sudden  and  there 
are  usually  other  symptoms  of  cerebral  involvement.  Tn  functional,  or 
hi/stencal,  amblyopia  the  symptoms  are  the  same  as  in  the  second  in- 
stance, but  there  are  other  hysterical  stigmata  present.  In  those  rare 
cases  of  monocular  hysteria  the  perfect  reaction  of  the  pupil  serves  to 
distinguish  the  functional  affection  from  organic  disease  of  the  nerve. 

Bight  Eye 


Fiu.  15.— Homonymous  quadrant  hemianopsia.    (Posey  and  Wright.) 

Temporal  hemianopsia  means  disease  of  the  chiasma.  Complete  blind- 
ness of  one  eye  with  loss  of  the  temporal  field  in  the  other  generally 
points  to  disease  of  the  chiasma  extending  to  the  outer  fibres  of  the  nerve 
or  optic  tract  on  the  side  on  which  there  is  complete  loss  of  vision. 

Wilbrand's  or  Wernicke's  Light-test — the  so-called  "  hemiopic 
pupil-reaction" — is  useful  in  determining  whether  the  lesion  in  a  case 
of  lateral  hemianopsia  lies  in  the  tractus  opticus  or  in  the  cerebral  cen- 
tres behind  it  (Fig.  17).  The  test  depends  upon  the  fact  that  when  light 
is  thrown  upon  the  blind  half  of  the  retina  the  pupil  contracts  just  as 
promptly  as  it  does  when  it  is  projected  upon  the  seeing  half  of  the  dis- 
ease confined  to  the  cortex  or  basal  neurons.  Wlien  the  tract  is  involved 
— i.e.,  when  the  conducting  path  to  the  corpora  quadrigemina  is  diseased 
— it  does  not  contract. 


EXTEKIOR  EXAMINATION   OF   THE   EYE         171 


The  patient  should  sit  (as  for  an  ophthalmoscopic  examination)  in  a 
half-lighted  room  with  one  eye  carefully  covered  and  fixing  with  the  eye 
to  be  tested  an  object  directly  in  front,  at  least  ten  feet  away.  The  rays 
from  a  small  electric  tube-lamp  should  be  first  focused  on  the  forehead 
and  then  allowed  to  strike  only  the  blind  half  of  the  retina.  The  effect 
is  noted  and  compared  with  the  result  obtained  by  illuminating  in  the 
same  way  the  other  retinal  half  (See  Page  393). 

The  ^sthesiometer  is  of  considerable  value  in  the  examination  of  the 
eyes  of  neurologic  patients.  Variations  in  the  tactile  sensibility  of  the 
palpebral  skin  are  best  tested  and  mapped  out  by  either  Carroll's  or 
Sieveking's  instrument,  but  conjunctival  and  corneal  areas  of  anaesthesia 
or  hypersesthesia  cannot,  for  obvious  reasons,  be  so  measured.       The 

Left  Eye 

90 


Fig.  16.— Homonymous  quadrant  hemianopsia.    (Posey  and  Wright.) 

•author  has  found  that  a  small  camel's-hair  brush  answers  every  useful 
purpose.  Employed  with  a  little  care,  particularly  where  it  is  possible 
to  compare  a  normal  with  an  abnormal  eye,  valuable  information  can 
be  obtained,  not  only  in  functional  affections — mostly  hysterical — but 
in  those  organic  lesions — paretic  and  other — that  reduce  or  increase  the 
conjunctival  and  corneal  sensibility.  In  examining  the  patient  with  this 
end  in  view,  it  is  well  to  have  him  rotate  the  eye  upward  as  much  as 
possible,  while  very  gently  touching  the  lower  conjunctival  surfaces  and 
lower  half  of  the  cornea  with  the  tip  of  the  brush.  Then,  the  upper  lid 
should  be  everted  and  the  exposed  conjunctivae  and  upper  hemisphere  of 


172 


THE    EYE    AND    NERVOUS    SYSTEM 


the  cornea  are  similarly  tested.  In  this  way  the  nervous  patient  does 
not  see  the  approach  of  the  brush-tip  and  is  obliged  to  regulate  his 
answers  by  touch-sensations  only. 

Color-Blindness. 

Color-blindness  is  either  congenital  or  is  due  to  some  organic  change 
in  the  optic  nerve-fibres  or  the  visual  centres  from  which  they  spring. 
The  latter  class  is  best  illustrated  in  toxic  (tobacco  or  alcohol)  ambly- 
opia, optic  atrophy,  diabetes,  hysteria,  etc.  Congenital  anomalies  of  the 
color-sense,  due  to  organic  defects  in  some  element  of  the  optic  radia- 
tions, are  more  common  in  men  (four  per  cent.)  than  in  women  (one 
per  cent.).  (See  Page  92).  It  is  well  to  remember  that  while  this  con- 
genital defect  cannot  be  remedied  and  that  the  person  born  color-blind  is 


Fig.  17 Illastrating  the  test  for  hemianopsic  pupillary  inaction  ;  the  lines  represent  a  horizontal 

plane  through  the  left  eye  and  its  visual  field  :  F  O,  fundus  oculi ;  .V,  macula  lutca ;  X,  nasal  half  of  the 
field  which  is  ancesthetic  in  temporal  hemianopsia;  T,  temporal  half  of  the  retina;  TF,  temporal 
field ;  /',  pupillary  aperture,  180°  to  0°,  the  equatorial  arc  or  semicircle ;  90°,  vertical  point  and  line 
passing  through  centre  of  eye  to  M;  70°  and  40°,  rays  of  light  striking  the  Insensitive  nasal  half  of  the 
retina,  producing  the  pupillary-  reflex.    (Seguin. ) 

always  color-blind,  the  intelligent  individual  establishes  a  standard  of 
color  for  himself  and  so  frequently  discounts  some  of  the  drawbacks  that 
reside  in  a  dyschromatopsia.  Occupations  like  that  of  railroad  fireman 
or  engineer,  recpiiring  acute  perception  of  colors,  should  not,  of  course, 
be  undertaken  l)y  color-blind  individuals. 

Color-blindness  is,  for  clinical  purposes,  readily  detected  by  the  use 
of  various  colored  skeins  of  wool  after  the  plan  devised  by  Holmgren. 
Thomson's  "  color-stick"  (Plate  I)  constitutes  a  handy  arrangement 
of  the  skeins,  which  are  numbered  to  correspond  witli  a  private  key 
for  the  use  of  the  color-blind  examiner.  The  patient  is  seated  before  a 
table  with,  preferably,   a  sufficient  natural  illumination  from  a  side 


EXTEKIOR  EXAMI:N^ATI0X   OF   THE   EYE         173 

window,  on  which  are  placed  skeins  of  colored  wools  in  different  shades 
of  red,  green,  gray,  bro^^^l,  etc.  In  front  of  him  is  a  large  skein  or  knot 
of  pure  green  wool.  This  is  the  "  test"  skein,  and  he  is  required  to  select 
from  the  others  in  front  of  him  the  skeins  of  the  same  color,  but  not 
necessarily  of  the  same  shade.  If  there  is  any  defect  in  his  color  vision 
the  patient  will  select  a  number  of  the  confusion  colors  (chiefly  grays), 
with  perhaps  some  green  tints.  If  he  accurately  chooses  all  greens  the 
investigation  need  proceed  no  further;  he  has  normal  color-sense — is 
not  color-blind.  If,  however,  he  fails,  he  is  given  a  rose-colored  skein 
and  again  told  to  choose  wools  of  that  color.  He  is  now  likely  to  match 
the  rose  with  browns,  purples,  dark  grays  or  even  greens.  Finally,  to 
find  out  what  variety  of  color-blindness  he  has,  he  is  given  a  deep  red 
skein,  to  match  which  he  will  probably  choose  the  dark  bro%vns  and 
greens.  The  great  majority  of  color-blind  people  are  defective  in  red 
and  green  and,  consequently,  are  said  to  have  red-green-blindness, 
although  all  varieties,  from  simple  green  to  total  color-blindness,  exist. 

With  Holmgren's  test  it  is  almost  impossible  for  one  who  is  color- 
blind to  escape  detection.  Thomson's  modification  of  this  test  is  prob- 
ably the  most  convenient.  The  person  under  examination  should  not 
be  allowed  to  name  the  colors ;  only  to  select  those  colors  resembling  the 
test-skein  in  his  hand. 

Peripheral  Blindness. — Generally  speaking,  blindness  is  regarded 
as  that  loss  of  sight  that  incapacitates  the  individual  from  following  any 
occupation  requiring  the  use  of  the  eyes.  The  proportion  of  blindness 
due  to  perij)heral  causes — i.e.,  those  affecting  the  external  organs  of 
vision — is  large.  The  tables  of  Magnus  show  that  at  least  67  per  cent,  of 
2528  cases  occurring  in  Germany  were  due  to  idiopathic  eye  affections. 
On  the  other  hand  as  high  as  two  per  cent,  resulted  from  purely  cerebral 
diseases ;  injury,  congenital  defects  and  general  affections  being  account- 
able for  the  remainder.  Gonorrhceal  infections  of  the  conjunctiva  (oph- 
thalmia neonatorum,  gonorrhceal  ophthalmia  of  the  adult),  trachoma  and 
diseases  of  tlie  cornea  induce  blindness  chiefly  by  producing  scars  in  the 
clear  corneal  tissue.  These  infections  were  alone  responsible  for  blind- 
ness in  27  per  cent,  of  Magnus's  cases.  Spinal  optic  atrophy  as  well  as 
irido-chorioiditis  from  meningitis  (particularly  the  epidemic  cerebro- 
spinal variety)  are  common  causes  of  lost  vision. 

When  sight  is  lost  from  destruction  of  the  cornea  the  necrosed  tissue 
is  replaced  by  cicatrices  (leucomata  especially)  that  are  readily  detected 
by  the  naked  eye.  More  deeply  seated  opacities  of  the  media,  such  as 
cataract,  are  better  seen  by  the  aid  of  the  lens  and  the  oblique  illumina- 
tion, already  described  on  page  156.     The  use  of  the  ophthalmoscope  is 


174  THE    EYE    AND    NERVOUS    SYSTEM 

required  for  the  identificatiou  of  many  lesions  of  the  jx)sterior  structures 
of  the  globe  that  with  equal  certainty  produce  blindness.  Diseases  of  the 
chorioid,  retina,  and  optic  nerve,  changes  in  the  vitreous  body,  intra- 
ocular growths,  glaucoma, — all  these  generally  require  an  exploration  of 
the  jwsterior  chamber  and  fundus  oculi  through  the  pupil  for  a  positive 
diagnosis.  The  employment  of  the  ophthalmoscope  in  the  determina- 
tion of  sources  of  blindness  is  all  the  more  imperative  since  the  uveal 
tract,  optic  nerve,  and  retina  constitute  the  anatomical  seats  of  more 
than  half  the  blindness  one  meets  with. 

Subjective  Visual  Sensations. 

Peripheral  Subjective  Visual  Sensations. — Muscae  Volitantes;  Phos- 
phenes. — The  appearance  of  specks,  spots,  or  other  minute  objects  float- 
ing in  front  of  the  eye  may  or  may  not  depend  upon  organic  disease  of 
that  organ.  In  most  instances  musca?  are  due  to  a  hypersensitive  per- 
cipient apparatus  which  takes  notice  of  the  normal  opacities  in  the  vitre- 
ous, but  it  is  not  uncommon  to  discover  exudates  and  other  floating 
deposits  within  the  vitreous  whose  presence  easily  accounts  for  these 
appearances. 

Phosphenes  are  colored  rings  or  flashes  of  light  which  may  at  any 
time  be  induced  by  pressing  on  the  inner  aspect  of  the  closed  eye.  These 
bright  color  sensations  may  also  be  caused  by  cerebral  diseases,  by  optic 
nerve  affections,  by  glaucoma,  and  by  other  conditions. 

Hallucinations  of  Vision.* — Although  in  a  restricted  sense  most 
phosphenes  and  muscaj  volitantes  are  examples  of  visual  hallucination 
they  are  not  usually  regarded  as  such.  The  sensations  of  this  order  due 
to  cerebral  diseases  are  on  the  other  hand  mostly  genuine  delusions,  just 
as  we  know  them  to  be  in  acute  alcoholic  mania.  Ilansell  gives  three 
varieties  of  visual  hallucinations ;  first,  those  due  to  ocular  disease, 
whether  organic  or  peripheral ;  second,  those  that  arise  from  localized 
disturbance  of  the  cerebral  circulation,  including  functional  nervous 
affections,  and,  third,  those  resulting  from  organic  disease  of  the  cortical 
centres.  Purely  ocular  hallucinations,  as  Ilansell  points  out,  are  de- 
pendent upon  a  number  of  causes.  These  he  enumerates  as  follows :  (1) 
errors  of  refraction,  (2)  oculo-mnscular  defects,  (3)  opacities  of  the 
dioptric  media,  and  (4)  change  in  the  choroid,  retina  and  optic  nerve. 
Thus  myopes  have  an  inaccurate  impression  of  size,  distance,  color,  and 
detail,  and  err  in  their  judgments  of  matters  concerning  which  their  only 


*  For  more  extended  information  on  this  subject  the  reader  is  referred  to  the 
reeent  papers  of  Drs.  Wm.  Campbell  Posey  and  Howard  F.  Hansell. 


SUBJECTIVE  VISUAL  SENSATIONS  175 

or  chief  information  comes  through  their  organs  of  vision.  So  much  is 
left  to  the  imagination  in  the  make-up  of  a  landscape,  a  picture,  a 
human  face,  that  they  suffer  a  rude  awakening  when  made  to  realize  by 
glasses  the  real  appearance  of  things.  The  hypermetrope  sees  the  detail, 
but  misses  the  harmonious  whole.  The  astigmatic  individual  sees  the 
square  an  oblong,  the  circle  an  oval,  and  fails  to  perceive  with  equal 
clearness  simultaneously  all  parts  of  an  object  (Compare  Page  388.). 

The  patient  with  a  paralyzed  ocular  muscle  receives  two  impressions 
of  every  object,  neither  of  them  correct,  because  the  mental  conception 
must  be  formed  without  the  association  of  the  binocular  co-ordination 
and  physiologic  double  retina  image.  The  mental  impression  of  opaci- 
ties of  the  cornea  is  that  of  cloudiness ;  of  opacities  of  the  lens,  that  of 
a  fog;  of  opacities  of  the  vitreous,  that  of  smoke.  Minute  scattered 
vitreous  opacities  are  extremely  deceptive,  and  lead  the  patient  to  believe 
he  sees  flies,  spiders,  webs,  that  elude  his  grasp  as  he  reaches  to  brush 
them  aside. 

Irritation  of  the  rods  and  cones  of  the  retina  and  the  axis  cylinders 
in  the  nerve  and  retina  by  the  pressure  of  blood  or  inflammatory  material 
accentuates  the  normal  activity  of  the  tissues  and  the  field  of  vision  is 
occupied  by  brilliant  meteoric  displays  of  flashes  and  circles  of  light, 
bright  or  colored  rings  or  waves  of  light  rapidly  passing  from  one  side 
to  the  other,  or  emanating  from  the  centre  and  spreading  toward  the 
periphery,  startling  and  real  while  they  last,  and  their  disappearance  is 
followed  by  a  feeling  of  intense  relief. 

The  author  has  notes  of  a  number  of  cases  in  which  patients  had, 
without  any  evidence  of  nervous  disease  and  exhibiting  only  faint  opaci- 
ties of  the  vitreous  or  slight  choroidal  lesions,  distinct  visions  of  rats  or 
mice  running  across  the  room.  In  every  instance  these  phenomena  were 
visible  towards  one  or  other  temporal  side  of  the  patient  and  at  a  dis- 
tance of  from  two  to  three  metres.  As  they  all  wore  glasses  the  hallu- 
cination was  attributed  to  images  projected  into  the  over-sensitive  eye 
from  the  inner  surface  of  one  lens. 

The  scintillating  scotoma,  or  "  fortification  spectrum,"  of  migraine 
is  sometimes  seen  without  the  occurrence  of  headache,  vertigo  or  nausea. 
Occasionally  it  appears  as  a  visual  aura  of  epilepsy.  In  the  first  instance 
it  may  mislead  the  surgeon  into  the  fear  of  an  approaching  optic  atrophy, 
but  an  examination  of  the  visual  field  and  the  use  of  the  ophthalmoscope 
will  clear  the  diagnosis. 

Hemeralopia  (sometimes  called  nyctalopia),  or  day  blindness,  consti- 
tutes a  peculiar  hypersesthesia  of  the  retina,  in  which  vision  is  decidedly 
worse  when  the  patient  is  exposed  to  bright  daylight,  but  improves  wuth 


176  THE    EYE    AND    NERVOUS    SYSTEM 

a  dim  illumination.  It  is  sometimes  associated  with  true  photophobia, 
phosphenes  and  with  various  other  visual  hallucinations.  It  is  seen,  now 
and  then,  after  prolonged  exposure  to  intense  reflections  from  large 
areas  of  water,  snow,  ice,  etc.,  and  is  not  uncommon  in  albinism  and  in 
those  suffering  from  coloboma  of  the  iris  and  choroid. 

Nyctalopia,  on  the  other  hand,  occurs  in  a  sensitive  retina.  The 
person  so  affected  sees  fairly  well  in  ordinary  daylight,  but  the  moment 
the  intensity  of  the  illumination  is  reduced  vision  is  much  worse.  This 
condition  is  occasionally  functional,  but  is  often  due  to  organic  disease 
of  the  choroid  and  retina,  as  seen  in  retinitis  pigmentosa,  syphilitic 
chorio-retinitis  and  in  some  degenerations  of  the  retina  not  due  to  lues. 

Erythropsia,  or  red  vision,  is  one  of  the  most  frequent  forms  of  chro- 
matopsia.  Colored  vision  is  generally  due  to  the  absorption  of  pigments 
that  either  tinge  the  dioptric  media  or  produce  some  unknown  effect 
upon  the  percipient  elements  of  the  retina.  Probably  this  explains  the 
yellow  vision  (xanthopsia)  of  jaundice,  as  well  as  that  following  poi- 
soning from  santonin,  amyl  nitrite,  cannabis  indica,  picric  acid,  etc.  The 
complaint  of  aphakic  patients  that  objects  seen  through- the  correcting 
lenses  are  blue,  or  deeply  edged  with  that  color,  is  not  explained  by 
the  possible  prismatic  effect  of  the  glasses.  Probably  the  eye,  accus- 
tomed to  perceive  objects  through  the  yellowish  medium  of  the  lens,  per- 
ceives them  as  blue  (the  complementary  color  of  yellow)  when  the  dis- 
eased crystalline  is  removed.  Red  vision  follows  exposure  to  extremely 
bright  white  or  violet  light,  for  instance,  to  the  rays  of  the  sun  or  to  the 
light  from  the  electric  furdace. 

Amblyopia. 

Amblyopia  is  a  term  indicating  poor  vision  without  apparent  cause, 
particularly  when  the  condition  is  not  explained  by  an  ophthalmoscopic 
examination. 

Toxic  Amblyopia. — The  commonest  forms  of  defective  vision  pro- 
duced by  poisoning  are  those  from  the  habitual  abuse  of  grain  (ethyl) 
alcohol,  tobacco  in  any  of  its  forms,  or  from  the  use  of  both  together. 
The  disease  is  usually  found  in  men  over  forty  years  of  age  who  suffer 
from  intestinal  catarrh  and  who  have  an  idiosyncrasy  against  stimulants. 
The  patient  complains  of  foggy  or  misty  vision  and  generally  applies  to 
the  ophthalmic  surgeon  for  glasses  to  assist  his  failing  sight.  Central 
vision  often  falls  to  t<s  of  normal,  and  the  reading  of  ordinary  print  is 
impossible  with  any  lens.  Such  a  patient  has  a  faulty  digestion,  an 
"  old  pipe"  odor  to  his  breath,  anorexia  and  morning  nausea,  insomnia 
and  dull  headache.     The  ocular  condition  is  mainly  due  to  a  central 


AMBLYOPIA  177 

scotoma  for  red  and  green,  the  perimetric  examination  showing  an  oval 
defect  about  the  fixation-point.  The  prognosis  in  this  form  of  toxic 
amblyopia  is  generally  very  favorable,  particularly  if  the  patient  can  be 
persuaded  to  abandon  his  toxic  habits. 

Rarer  forms  of  amblyopia  from  poisons  are  those  produced  by  drink- 
ing methyl  or  wood  alcohol  ("  Columbian  Spirits/'  "  Cologne  Spirits," 
adulterated  ''  Jamaica  Ginger,"  lemon  extract,  bay  rum,  etc.),  by  the 
•  ingestion  of  quinine  and  by  the  absorption  of  lead  salts.     In  all  these 
cases  complete  blindness  from  optic  atrophy  may  occur. 

Reflex  Amblyopia  is  a  form  of  blindness  due  to  irritation  (generally 
peripheral)  of  the  nerves  in  more  or  less  close  relation  to  the  optic  cen- 
tres. Intestinal  worms,  especially  in  children,  diseases  of  the  teeth  in 
adults  and,  in  rare  instances,  pathologic  changes  in  the  reproductive 
organs  have  been  held  responsible  for  visual  defects  that  have  disap- 
peared with  the  removal  of  the  cause. 

Amblyopia  ex  anopsia  is  a  term  of  rather  ill-defined  meaning.  In 
many  cases  of  strabisnms  due  to  congenital  conditions,  or  associated 
with  them,  the  squinting  eye,  although  amblyopic,  presents  no  organic 
defect.  However,  after  exercise  of  the  weaker  eye  (following  a  removal 
of  the  barrier  to  binocular  sight)  not  only  central  but  peripheral  vision 
of  the  eye  may  very  greatly  improve.  The  same  statement  is  occasionally 
true  of  long-standing  early  cataract.  It  is  not  improper,  then,  to  believe 
that  the  defective  sight  of  such  eyes  was  due  to  non-use  of  the  organ — 
just  as  temporarily  paretic  muscles  may  undergo  a  functional  atrophy 
and  yet  regain  their  original  size  on  the  return  of  normal  innervation. 
It  is  not  yet  decided  how  one  may  unerringly  differentiate  between  eyes 
capable  of  visual  improvement  and  those  that  continue  functionally  in- 
competent during  life  in  spite  of  all  treatment. 

Amblyopia  from  Toxins.  Uraemia. — Loss  of  vision  from  drug  poisoning 
has  its  counterpart  in  certain  toxic  conditions  of  the  blood,  particularly 
uraemia.  The  blindness,  as  in  quinine  and  methyl  alcohol  amaurosis, 
comes  on  suddenly  and  the  pupils  are  often  dilated,  although  they  gen- 
erally react  to  light.  There  are  no  ophthalmoscopic  changes  due  to  the 
toxaemia  proper,  and  sight  returns  when  the  state  of  the  blood  improves. 
Convulsions  and  other  evidence  of  uraemia  usually  accompany  the  visual 
defect. 


12 


CHAPTER    V. 

THE  EXTEA-OCULAE  MUSCLES 

By  ALEXANDER  DUANE,  M.D. 

Section  I. 
PHYSIOLOGY    OF    THE    EYE-MUSCLES    AND    EYE-MOVEMENTS. 
TESTS.       NOMENCLATURE    AND    CLASSIFICATION    OF    THE 
OCULAR    DEVIATIONS. 

Fixation  and  its  Derangements. — Monocular  Fixation. — When  we 
direct  one  of  our  eyes  towards  an  object  so  as  to  see  it  as  distinctly  as 
possible  we  are  said  to  fixate  or  jix  that  object. 

The  fovea  centralis  is  the  most  sensitive  portion  of  the  retina. 
Hence,  in  performing  fixation  we  normally  direct  the  eye  so  that  the 
image  of  the  object  looked  at  falls  upon  the  fovea.  This  is  called 
central  fixation.  It  may  happen,  however,  that  owing  to  disease  of 
the  retina  or  to  other  reasons  vision  with  the  fovea  is  less  acute  than 
with  some  outlying  portion  of  the  retina,  and  in  that  case  the  eye,  in 
monocular  fixation  at  least,  will  be  directed  so  that  the  image  shall 
fall  upon  this  outlying  portion  {eccentric  fixation). 

Monocular  Projection. — When  we  are  looking  with  one  eye  at  an 
object  and  that  object  forms  its  image  on  the  retina,  we  not  only  see  the 
object,  but  get  an  idea  that  it  occupies  a  certain  definite  position  in  space. 
This  process  by  which  we  infer  the  position  of  an  object  in  space  from 
the  visual  impression  that  the  object  produces  we  call  projection. 

An  object  towards  which  the  eye  is  pointing  forms  its  image  on  the 
fovea.  Conversely,  an  object  which  thus  forms  its  image  on  the  fovea 
we  judge  to  be  situated  in  that  part  of  space  towards  which  we  think 
the  eye  is  pointing.  If  our  judgments  are  correct  as  to  the  direction 
of  our  eye,  we  project — i.e.,  locate — objects  correctly ;  but  if,  as  in 
paralysis,  our  notions  of  the  positions  of  the  eye  are  erroneous,  our 
projection  will  be  faulty  and  we  will  see  objects  in  the  wrong  place. 

Not  only  do  we  get  an  idea  of  the  location  of  an  object,  A  (Fig.  1), 
towards  which  the  eye  is  pointing,  but  also  a  notion,  more  or  less  pre- 
cise, of  the  location  of  all  objects  surrounding  A.  Thus,  an  object,  B, 
that  forms  its  image  below  the  fovea,  we  regard  as  situated  in  space 
correspondingly  far  above  A,  for,  objects  that  are  actually  above  the 
178 


THE  EXTRA-OCULAR  MUSCLES 


179 


point  we  are  looking  at  do  regularly,  in  our  experience,  form  their  image 
below  the  fovea.  So  also  the  object,  C  (Fig.  2),  which  forms  its  image 
to  the  left  of  the  fovea,  we  judge  to  be  situated  correspondingly  far  to 
to  the  right  of  A, 

We  thus  arrive  at  the  law  of  projection,  which  may  be  stated  thus : 
An  object  ivhicJi  forms  its  image  on  any  point  in  the  retina  is  projected 
to — i.e.,  mentally  located  in — the  point  in  space  directly  opposite. 

Fig.  1. 


Binocular  Fixation. — In  the  vast  majority  of  cases,  if  one  eye  fixates 
an  object  with  its  fovea  the  other  will  do  so  too  (binocular  fixation). 
In  fact,  the  whole  extremely  complicated  mechanism  of  muscles  and 
nerves  with  which  the  two  eyes  are  supplied  is  designed  primarily  to 
accomplish  this  single  end — i.e.,  to  so  move  the  eyes  that  they  shall 
both  be  directed  accurately  to  the  object  we  wish  to  see  and  each  eye 

Fig.  2. 


shall  receive  the  image  of  that  object  precisely  upon  the  fovea.  The 
movements  of  the  two  eyes  are  hence  almost  invariably  so  co-ordinated 
as  to  secure  binocular  fixation  under  all  conditions. 

Derangements  of  Binocular  Fixation;  Deviations  of  the  Eye. — !N^ormally, 
as  we  have  seen,  a  person  looking  with  both  eyes  open  fixates  the  same 
object  with  both, — i.e.,  both  eyes  are  "  straight." 


180  THE    EYE    AND    NERVOUS    SYSTEM 

Xot  only  is  this  so,  but  in  the  majority  of  cases  both  eyes  will  still 
fixate  the  same  object, — i.e.,  both  will  be  "  straight"  even  when  one 
eye  is  covered.  This  condition  is  called  orthophoria,  and  must  be 
regarded  as  the  ideal  or  natural  condition. 

In  other  instances  both  eyes  will  look  straight  at  the  same  object 
when  both  are  uncovered,  but  either  eye  as  soon  as  it*  is  covered  will 
deviate — turning  out,  in,  up,  or  down.  This  condition  is  called  hetero- 
phoria,  or,  less  properly,  insufficiency. 

In  a  third  class  of  cases,  comparatively  few  in  number,  only  one 
eye  is  straight  at  a  time,  the  other  deviating  even  when  both  eyes  are 
open.     This  condition  is  called  squint,  strabismus,  or  heterotropia. 
Squint  is  further  called — 
I.    (a)  Alternating  when  sometimes  one  eye  deviates,  sometimes  the 
other. 
(&)  Monocular  (right  or  left)  when  the  deviation . always  affects 
the  same  eye. 
Again,  the  deviation^  whether  squint  or  heterophoria,  is  called — 
II.    (a)   Intermittent  when  under  the  same  conditions  it  is  sometimes 
present,  sometimes  not. 
(b)   Constant  when  it  is  present  all  the  time. 

III.  (a)  Periodic  when  it  is  present  when  the  eyes  are  directed  at  a 

near  object  and  absent  in  distant  vision,  or  vice  versa, 
(b)   Continuous  when  it  is  present  both  in  distant  and  near  vision. 

IV.  (a)   Comitant  or  concomitant  when  the  amount  of  deviation  re- 

mains the  same,  and 
(b)  Non-comitant  when  the  deviation  changes  notably,  as  the  eyes 
are  carried  in  different  directions. 
A  non-comitant  deviation  is  usually  of  paralytic  origin  (paralytic 
squint  or  heterophoria).     A  comitant  deviation  is  not  usually  due  to 
paralysis,  at  least  paralysis  of  the  individual  muscles,  although  it  may 
develop  out  of  such  a  paralysis. 

Deviations  are  also  named  according  to  the  direction  of  the  deviating 
eye.    Thus — 

HETEROPHORIA. 

Both  eyes  fix  when  both  arc  uncovered,  but  behind  screen — 

Either  eye  turns  in Esophoria. 

Either  eye  turns  out Exophoria. 

R.  eye  turns  up'  and  L.  down  * R.  Hyperphoria. 

L.  eye  turns  up  and  R.  down  * L.  Hyperphoria. 

*  In  some  anomalous  cases  (see  Section  VI.),  instead  of  one  eye  going  up  and  the 
otlicr  down  when  screened,  each  eye  goes  up  or  each  down. 


THE  EXTRA-OCULAR  MUSCLES  181 

SQUINT. 

Wlien  both  eyes  are  uncovered  one  fixes  and  the  other  turns — 

In Esotropia  (convergent  squint,  strabismus  con- 

vergens),  R.  or  L, 

Out Exotropia    (divergent   squint,    strabismus   di- 

vergens),  R.  or  L. 

Up Hjpertropia  (upward  squint,  strabismus  sur- 

sumvergens),  R.  or  L. 
Down.  .  .  .Hypotropia  (downward  squint,  strabismus  de- 
orsumvergens),  R.  or  L. 
Behind  screen — 

Either  eye  deviates  in Esotropia  (R.  or  L.). 

Either  eye  deviates  out Exotropia  (R.  or  L.). 

R.  eye  turns  up  and  L.  eye  down  * .  .  .  R.  Hypertropia  or 

L.  Hypotropia. 
L.  eye  turns  up  and  R.  eye  down  * .  .  .  L.  Hypertropia  or 

R.  Hypotropia. 
Esophoria,  exophoria,  esotropia,  and  exotropia  are  also  known  as 
lateral  deviations    (heterophoria,   squint) ;    hyperphoria,  hypertropia, 
and  hypotropia  as  vertical  deviations. 

Mixed  conditions, — e.g.,  combinations  of  a  vertical  with  a  lateral 
deviation, — are  quite  frequent.  Especially  common  are  the  conditions 
prevailing  in  a  developing  squint  (see  Section  IV.),  in  which  hetero- 
phoria or  orthophoria  alternate  with  a  true  strabismus,  or  a  squint  for 
near  is  associated  with  heterophoria  for  distance,  etc.  Lastly,  a  patient, 
especially  in  paralytic  cases,  may  show  an  esotropia  if  he  looks  in  one 
direction  and  an  exotropia  if  he  looks  in  another ;  or,  he  may  have  an 
esotropia  for  distance  and  an  exotropia  for  near,  as  in  a  combined 
divergence-  and  convergence-paralysis  (page  257)  ;  or  he  may  have 
an  esotropia  if  he  fixes  with  one  eye  and  exotropia  if  he  fixes  with  the 
other.  In  the  case  last  mentioned,  one  eye  will  deviate  out  when  cov- 
ered, the  other  eye  will  deviate  in  (Graefe).     (See  Page  251,  note.) 

Binocular  Single  Vision:  Fusion. — As  we  have  seen,  in  all  but  a 
small  minority  of  cases  binocular  fixation  is  present  whenever  we  look 
with  both  eyes.  The  image  of  the  object  looked  at  then  is  formed  on 
each  fovea.  Yet,  though  there  are  the  two  images,  we  see,  not  two 
objects,  but  one  (binocular  single  vision).  For  the  mind  by  a  peculiar 
process  which  we  denominate  fusion  combines  the  two  retinal  images  into 

*  In  some  anomalous  cases  (see  Section  VI.))  instead  of  one  eye  going  up  and  the 
other  down  when  screened,  each  eye  goes  up  or  each  down. 


182  THE    EYE    AND    NERVOUS    SYSTEM 

a  single  visual  impression.  This  binocular  visual  image  differs  some- 
Avhat  in  character  from  the  monocular  visual  image  gained  when  we 
look  with  either  eye  by  itself,  being  rather  more  distinct  and  also 
more  solid-looking.  In  fact,  vision  with  both  eyes  differs  from  vision 
with  one  eye  in  that  with  the  former  we  get  clear,  and  with  the  latter 
but  imperfect,  ideas  of  distance,  depth,  and  relief.  In  other  words, 
when  we  look  with  both  eyes  we  have  solid  or  stereoscopic  vision. 

The  mental  faculty  by  which  fusion  is  effected  is  called  the  fusion 
faculty.  It  acts,  presumably,  by  means  of  a  cerebral  nerve-centre  {fusion 
centre),  the  site  of  which,  however,  is  altogether  undetermined.  Absence 
or  non-development  of  tlie  fusion  faculty  is  thought  to  be  an  important 
factor — according  to  some,  the  prime  factor — in  the  production  of 
squint. 

Binocular  Projection. — The  visual  impression  produced  by  this  fusion 
of  the  two  retinal  images  is  by  the  mind  projected,  or  referred  to  a  por- 
tion of  space  outside  of  the  body,  being  thus  regarded  as  a  something 
apart  from  ourselves  and  occupying  a  certain  place  in  the  exterior 
world.  The  point  to  which  we  thus  refer  it  is  the  point  of  intersection 
of  our  two  visual  lines.  Unless  the  eyes  abnormally  deviate,  this  point 
corresponds  to  the  actual  situation  of  the  object  itself  as  determined  by 
our  other  senses.  Thus,  when  we  look  at  an  object  one  foot  off,  the  eyes 
turn  until  their  visual  lines  are  both  directed  at  the  object,  and  hence 
intersect  at  the  point  where  it  is  situated.  Then  each  foveal  image  is 
projected  out  to  this  point  of  intersection, — i.e.,  to  the  place  that  the 
object  itself  actually  occupies. 

Corresponding  Points. — Not  only  does  the  mind  fuse  the  two  retinal 
images  of  any  object  looked  at  directly,  so  that  that  object  appears 
single,  but  it  fuses  the  images  of  a  good  many  other  objects  as  well. 
Thus,  if  we  are  fixing  an  object  A  with  both  eyes.  A,  which  forms  an 
image  on  each  fovea,  will  appear  single  and  will  look  to  be  straight  in 
front  of  the  eyes.  Another  object,  B,  situated  to  the  right  of  A,  will 
form  its  retinal  image  a  certain  distance,  say  10°  to  the  left  of  the 
fovea  in  the  right  eye.  If  now  it  is  so  situated  as  to  form  its  retinal 
image  10°  to  the  left  of  the  fovea  in  the  left  eye  too,  it  will  appear 
single,  the  two  outlying  retinal  images  being  fused  in  this  case  just 
as  the  two  foveal  images  were.  In  general,  any  object  whose  retinal 
images  are  formed  equally  far  and  in  the  same  direction  from  the 
fovea  in  each  eye — are  formed  as  we  say,  on  the  corresponding  points 
of  the  two  retinas — will  appear  single. 

Diplopia. — As  we  have  just  seen,  when  the  images  of  the  same  object 
fall  upon  corresponding  points,  the  object  appears  single.     It  is  other- 


THE  EXTRA-OCULAR  MUSCLES  183 

wise  when  the  images  fall  upon  points  not  corresponding, — e.g.,  on  the 
fovea  in  one  eye  and  on  any  outlying  portion  of  the  retina  in  the  other. 
Then,  in  general,  we  have  double  vision  (diplopia),  the  mind  receiving 
two  visual  impressions  of  the  same  object.  In  this  case  each  eye  pro- 
jects the  image  differently, — i.e.,  locates  it  in  a  different  point  in  space. 
Physiological  Diplopia. — In  many  cases  this  diplopia  is  physiological. 
Thus,  in  Fig.  3  the  object  A,  at  which  both  eyes  are  looking  and  which, 
therefore,  forms  its  retinal  images  on  the  two  foveas  M  and  M'  respec- 
tively, appears  single,  being  projected  by  both  eyes  to  its  true  place,  A ; 
but  B,  an  object  more  remote  than  A  and  situated  in  the  prolongation 
of  the  line  MA,  will  appear  double,  for  while  it  forms  its  image  in  the 
right  eye  on  the  fovea,  M,  it  will  form  its  image  in  the  left  eye  on  a 
point,  X,  to  the  right  of  the  fovea.     To  the  right  eye  it  will  appear 

Fig.  3. 


directly  in  line  with  A  (behind  it)  ;  to  the  left  eye  it  will,  according 
to  the  law  of  projection,  appear  to  be  to  the  left  of  A,  and  the  more  so 
the  further  X  is  to  the  right  of  M'.  We  shall  have  thus  a  condition  of 
homonymous  diplopia, — i.e.,  a  condition  in  which  the  image  seen  by 
the  left  eye  is  to  the  left  of  that  seen  by  the  right  eye. 

The  object,  C,  lying  nearer  to  the  eyes  than  A  and  in  the  line,  AM, 
will,  to  the  right  eye,  seem  to  be  in  line  with  A  (just  in  front  of  it) 
and  to  the  left  eye  wiU  seem  to  lie  to  the  right  of  A,  since  C  forms  its 
image  in  the  left  eye  at  O  to  the  left  of  the  fovea.  Such  diplopia,  in 
which  the  image  seen  by  the  left  eye  appears  to  the  right  of  that  seen 
by  the  right  eye,  is  said  to  be  heteronymous  or  crossed.  This  physio- 
logical diplopia,  crossed  for  objects  nearer  than  tiie  object  looked  at  and 


184  THE    EYE    AND    NERVOUS    SYSTEM 

homonymous  for  more  distant  objects,  although  readily  demonstrable, 
rarely,  if  ever,  appears  spontaneously  and  hardly  ever  occasions  any 
confusion. 

Artificial  Diplopia  produced  by  Prisms. — Diplopia  may  also  be  pro- 
duced by  prisms.  Thus,  suppose  both  eyes  are  looking  at  an  object,  A 
(Fig.  4)  so  that  the  image  of  that  object  falls  on  the  fovea  in  each  eye, 
and  then  a  prism  with  the  base  directed  inwards  (towards  the  nose)  is 
placed  before  the  left  eye.  The  prism  will  deflect  the  rays  of  light 
towards  its  base,  so  that  the  retinal  image  in  the  left  eye  will  no  longer 
fall  on  the  fovea,  M',  but  to  the  right  of  it,  N.  The  eye,  according  to 
the  law  of  projection,  will  regard  this  image  as  situated  to  the  left  of 
A, — i.e.,  the  right  eye  fixing  A  with  its  fovea  will  see  A  in  its  true 
place,  while  the  left  eye  will  see  a  similar  image,  but  dislocated  to  the 
left  (at  A').    There  will  then  be  a  condition  of  homonymous  diplopia. 

Fig.  4. 


So  also,  a  prism  placed  base  out  before  one  eye  will  produce  crossed 
diplopia.  A  prism  placed  feose  up  or  base  down  will  make  the  corre- 
sponding image  appear  below  or  above  the  other, — i.e.,  will  cause  a 
vertical  diplopia. 

Pathological  Diplopia. — Diplopia  is  also  caused  by  a  deviation  of  the 
eyes.  That  is,  if  the  right  eye  (R.  Fig.  5)  is  fixing  the  object  A,  but 
the  left  eye,  instead  of  fixing  it,  is  turned  too  far  inwards,  the  retinal 
image  of  A  in  this  eye  will  fall  not  on  the  fovea  M',  but  to  the  inner  or 
right  side  of  it  (N).  Now,  the  mind  really  does  not  know  how  the  left 
eye  is  pointing;  in  fact,  it  seems  to  regard  both  eyes  as  looking  in  the 
same  direction ;  hence,  an  object  whose  image  is  on  the  fovea  of  the 
left  eye  will  be  projected  out  to  A,  the  direction  in  which  the  mind  con- 


THE  EXTRA-OCULAK  MUSCLES  185 

ceives  this  eye  to  be  pointing  (page  178),  and  an  object  whose  reti- 
nal image  is  at  X,  to  the  right  of  the  fovea,  will  be  regarded  as  located 
correspondingly  far  to  the  left  of  A.  Such  a  patient,  therefore,  sees 
two  images  of  A — one  seen  by  the  right  eye,  situated  in  its  true  place 
straight  ahead ;  the  other  seen  by  the  left  eye,  situated  to  the  left  (A') — 
i.e.,  there  is  here  an  homonymous  diplopia.  If  the  left  eye  had  been 
turned  out  there  would  have  been  a  crossed  diplopia;  if  up  or  down, 
a  vertical  diplopia. 

Overcoming  Diplopia. — Diplopia  produced  by  prisms,  or  diplopia  pro- 
duced by  pathological  deviation,  may  be  overcome  if  the  eye  is  turned 
so  that  the  image  in  either  case  falls  upon  the  fovea.  Thus,  the  homony- 
mous diplopia  shown  in  Fig.  4  and  in  Fig.  5  will  be  abolished  if  the  left 
eye  turns  out  as  shown  by  the  arrow,  for,  turning  the  eye  out  will  turn 

Fig.  5. 


the  fovea  M'  in,  and  when  M'  has  gone  in  far  enough  the  retinal  image 
of  A  will  fall  upon  it;  then  the  retinal  image  will  fall  on  the  fovea 
in  each  eye,  and  there  will  be  single  vision  (See  Page  181). 

The  ability  to  overcome  diplopia  depends  partly  upon  the  readiness 
with  which  the  various  movements  required  for  the  purpose  can  be  exe- 
cuted, partly  upon  the  development  of  the  fusion  faculty  (See  Page  182). 
A  man,  however  well  developed  and  well  regulated  his  eye  muscles  and 
the  nerve-centres  that  co-ordinate  them  may  be,  will  not  unite  double 
images  well  if  his  fusion  faculty  is  imperfect.  Ill  development  or 
temporary  abeyance  of  the  fusion  faculty  probably  accounts  in  part  for 
the  fact  that  in  some  cases  of  neurasthenia,  hysteria,  and  organic  brain 
disease,  diplopia  is  so  easily  produced  and  when  product  is  so  difficult 


186 


THE    EYE    AI^D    NERVOUS    SYSTEM 


to  overcome.  In  such  cases  the  mere  act  of  placing  a  red  glass  or  a 
1°  prism  before  one  eye  suffices  to  evoke  an  insuperable  diplopia. 
Again,  there  are  some  persons  who  never  by  any  adjustment  of  the  eyes 
or  with  any  prism  get  fusion  of  the  double  images, — in  whom,  for 
instance,  a  prism  of  6°,  base  out,  produces  a  crossed,  and  a  prism  of 
5°,  base  out,  a  homonymous  diplopia,  but  no  prism  produces  single 
vision.  This  condition  of  so-called  antipathy  to  single  vision  occurs  in 
squint,  particularly  after  operation,  and  is  probably  attributable  to  non- 
development  of  the  fusion  faculty. 

Varieties  of  Diplopia. — The  foregoing  facts,  together  with  the  nomen- 
clature of  the  different  varieties  of  diplopia  are  succinctly  shown  in  the 
following  table : 

TABLE   OF   DIPLOPIA.* 


NAME  or  DIPLOPIA 

CHARACTER 

CAUSED  BY 

CORRECTED  BY 

Image  of  R. 
eye  as  com- 
pared    with 
that  of  L.  is 

(1)  A  natural 
deviation  of 

(2)   Artifici- 
al ly     by    a 
prism     placed 
base. 

( 1 )  Turning, 

(2)  Prism 
placed  with 
base. 

"< 
M 
H  ' 

< 

-< 

U 

> 

H  o  m  o  n  y- 

mous. 

Heterony- 
mous. 
.(Crossed.) 

rRight. 
Left. 

Ontheright. 
On  the  left. 

Below. 
Above. 

Either  eye  in- 
ward (esopho- 
ria,  esotropia). 

Ei  ther  eye 
outward    ( ex- 
ophoria,    exo- 
tropia). 

R.  eye  up  or 
L.    eye    down 
( R.  hyperpho- 
ria, R.  hyper- 
tropia,  L.  hy- 
potropia. 

R.  eye  down  or 
L.  eye  up  (L. 
hyperphoria, 
L.     hypertro- 
pia,  R    hypo- 
tropis). 

In,    before 
either  eye. 

Out,    before 
either  eye. 

Up   before   R. 
eye,     do  ton 
before  L.  eye. 

Down     before 
R.    eye,    up 
before  L.  eye. 

Both  eyes  out- 
ward.    ( D  i- 
vergence. ) 

Both  eyes  in- 
ward.    ( Con- 
vergence. ) 

R.    eye    down 
and  L.  eye  up 
(L.  supra ver- 
gence). 

R.  eye  up  and 
L.    eye   down 
(R.   supraver- 
gence). 

Out  before 
either  eye. 

In  before 
either  eye. 

Down  be- 
fore R.  eye 
or  up  be- 
fore L.  eye. 

Up  before 
R.  eye  or 
down  before 
L.  eye. 

In  conditions  producing  vertical  diplopia,  especially  when  the  eyes 
are  directed  down,  one  image  (usually  the  one  that  should  be  higher) 
often  seems  to  be  on  a  level  with  the  other,  but  back  of  it.  This  is  due 
partly  to  the  character  of  the  background  upon  which  the  images  are 
projected,  partly  to  other  factors  (M.  Sachs,  Frolich). 


*  Modified  from  the  author's  article  in  De  Schweinitz  and  Randall's 
Text-Book  of  the  £ye,  Ear,  Nose,  and  Throat." 


■  American 


THE  EXTRA-OCULAE  MUSCLES  187 

Character  of  the  Images  in  Diplopia. — In  diplopia  the  image  seen  by 
the  fixing  eye  is  usually  sharp  and  clear  because  that  eye  sees  it  with 
the  fovea,  the  portion  of  the  retina  having  the  most  distinct  vision. 
This  image  is  hence  called  the  real  image.  The  other,  or  false,  image, 
projected  by  the  non-fixing  eye,  looks  shadowy  and  indistinct,  because 
it  is  seen  by  an  extrafoveal  portion  of  the  retina. 

Binocular  Fixation  and  Binocular  Single  Vision  compared. — De- 
rangements of  binocular  fixation  cause  the  various  deviations  of  the  eye 
(squint  or  heterophoria).  Such  a  derangement  is  proved  to  exist  if 
the  deviation  can  be  made  out  objectively, — i.e.,  if  there  is  a  deflection 
of  the  eyes  which  the  observer  can  discover  on  inspection  of  the  patient. 

On  the  other  hand,  disturbance  of  binocular  single  vision,  is  proved 
to  exist  if  the  patient  himself  has  the  subjective  sensation  of  diplopia. 

Upon  these  two  facts  depend  all  of  our  tests,  which  are  hence  classed 
as  objective  when  they  show  disturbance  in  binocular  fixation  which  the 
observer  can  detect,  and  subjective  when  they  depend  directly  or  indi- 
rectly upon  the  demonstration  of  diplopia. 

The  two  conditions — disturbance  of  binocular  fixation  and  disturb- 
ance of  binocular  single  vision — usually  occur  together  and  are  equally 
marked.  In  this  case  the  objective  and  subjective  tests  can  both  be 
employed  and  will,  moreover,  agree  with  each  other.  In  two  classes  of 
cases,  however,  this  is  not  so : 

I.  In  most  cases  of  old  squint  binocular  vision  is  absent — that  is, 
when  both  eyes  are  open  the  image  formed  on  the  retina  of  the  squint- 
ing eye  is  mentally  suppressed  so  that  the  patient  sees  with  the  other 
eye  only.  When  the  squinting  eye  alone  is  used  for  seeing,  this  sup- 
pression does  not  occur ;  hence  if  a  red  glass  is  placed  before  the  squint- 
ing eye  and  the  patient  looks  at  a  light  with  his  right  eye  shut  he  will 
see  a  red  light,  while  if  he  either  has  both  eyes  open  or  has  the  left  eye 
shut  he  will  see  simply  a  white  light.  In  these  cases,  therefore,  the 
patient  cannot  be  made  to  recognize  diplopia;  hence,  all  the  subjective 
tests  which  in  one  way  or  another  depend  upon  the  recognition  of  diplo- 
pia, fail,  and  we  have  to -depend  entirely  on  the  objective  tests  (those 
for  binocular  fixation). 

II.  In  many  cases  of  squint,  and  particularly  of  cured  squint,  both 
subjective  and  objective  tests  are  applicable,  but  are  discrepant  because 
the  patient  projects  or  locates  objects  in  space  erroneously  with  his 
squinting  eye.  Thus,  we  may  have  a  condition  in  which  the  eye  is 
obviously  turned  in,  and  yet  there  is  either  a  crossed  diplopia,  or,  if 
there  is  a  homonymous  diplopia  it  does  not  correspond  in  amount  to 
the  degree  of  deviation  (incongruity  of  the  double  images). 


188  THE    EYE    AND    NERVOUS    SYSTEM 

Muscles  of  the  Eye. — The  muscles  moving  the  eyeball  and  eyelids 
are  the  internal,  external,  superior,  and  inferior  recti,  the  superior  and 
inferior  obliques,  and  the  levator  palpebra?. 

The  four  recti  and  the  levator  arise  from  the  lesser  and  greater  wing 
of  the  sphenoid  and  from  the  dense  fibrous  membrane  filling  in  the 
sphenoidal  fissure.  Their  combined  origins  form  a  tendinous  ring 
which  surrounds  the  optic  foramen  and  that  portion  of  the  sphenoidal 
fissure  giving  passage  to  the  oculomotor  and  abducens  nerves.  These 
five  muscles  pass  forward,  diverging  as  tliey  go,  so  as  to  form  a  hollow 
cone  (muscle  cone),  which  includes  the  second,  third,  and  sixth  nerves, 
and  the  ciliary  ganglion. 

The  superior  rectus  lies  underneath  the  levator,  to  which  it  is  con- 
nected quite  firmly  by  bands  of  fascia.  The  other  muscles  at  first  run 
quite  close  to  the  walls  of  the  orbit,  but  somewhat  in  front  of  the  middle 
of  their  course  the  four  recti  turn  in  toward  the  eyeball,  and  piercing 
the  orbital  fat  and  Tenon's  capsule,  are  inserted  into  the  sclera  around 
the  cornea.  The  internal  rectus  is  inserted  5.5  millimetres,  the  inferior 
6.5  millimetres,  the  external  7  millimetres,  and  the  superior  7.5  to  8 
millimetres  from  the  corneal  margin.  The  levator  passes  on  to  be 
inserted  into  the  tarsus  of  the  upper  lid. 

The  superior  oblique  arises  from  the  body  of  the  sphenoid,  runs  for- 
ward, keeping  close  to  the  upper  wall  of  the  orbit,  and  at  the  upper 
inner  margin  of  the  orbit  passes  through  a  tendinous  ring  (the  tro- 
chlea), from  which  it  is  reflected  obliquely  backward  and  outward  so  as 
to  pass  beneath  the  tendon  of  the  superior  rectus.  It  pierces  Tenon's 
capsule,  and  is  inserted  into  the  upper  outer  portion  of  the  eye  back  of 
the  equator. 

The  inferior  oblique  arises  from  the  inner  lower  wall  of  the  orbit  just 
back  of  the  nasal  duct,  passes  obliquely  up  and  out  beneath  the  inferior 
rectus,  and  is  inserted  into  the  lower  outer  part  of  the  eyeball  behind 
the  equator. 

Mention  has  been  made  of  Tenon's  capsule.  This  is  a  spherical 
capsule  of  connective  tissue  surrounding  the  whole  eye  except  the  cornea 
and  a  little  rim  of  sclera  aroimd  it  in  front,  and  the  optic  nerve  and  a 
little  annular  area  of  sclera  about  it  behind.  It  is  thus  like  a  broad 
convex  ring  surrounding  the  eye  like  a  sling.  The  anterior  margin  of 
this  ring  is  fused  with  the  conjunctiva,  and  both  conjunctiva  and  cap- 
sule are  attached  firmly  to  the  sclera  round  the  cornea.  The  posterior 
margin  of  the  capsular  ring  is  also  attached  to  the  sclera,  so  that  the 
space  between  the  eyeball  and  Tenon's  capsule  is  closed  in,  both  anteri- 
orly and  posteriorly.     This  space,  called  Tenants  space,  is  traversed  by 


THE  EXTExV-OCULAR  MUSCLES  189 

connective  tissue  bands  joining  tlie  capsule  to  the  eyeball.  Tenon's 
space  is  held  to  be  the  analogue  of  a  joint  cavity,  although  it  is  filled 
neither  with  synovia  nor  with  lymph.  This  analogy  must  not  be  pushed 
too  far,  for  while  the  eye  does,  in  fact,  turn  in  the  orbital  tissue  as  in  a 
socket,  it  is  probable  that  as  Tenon's  capsule  must,  to  a  certain  extent, 
move  Avith  the  eye  to  which  it  is  attached,  the  real  socket  of  the  eyeball 
lies  in  the  orbital  fat  outside  of  the  capsule  (Motais,  cited  by  Maddox). 

In  considering  the  action  of  the  muscles,  we  have  to  take  into  ac- 
count not  only  the  primary  insertion  (to  the  eyeball),  but  also  the 
secondary  insertion  (to  the  orbital  walls  and  eyelids). 

The  primary  insertion  of  each  muscle  is  by  a  broad,  flat  tendon, 
implanted  into  the  sclera  beneath  the  conjunctiva  and  Tenon's  cap- 
sule. This  insertion  is  reinforced  by  a  fibrous  band  on  each  side  con- 
nected with  the  sclera  and  extending  about  one  to  one  and  one-half  milli- 
metres in  front  of  the  insertion  proper.  These  adminicular  as  they  are 
called,  like  the  insertion  itself,  lie  beneath  Tenon's  capsule, — i.e.,  within 
the  capsular  cavity.  Other  bands  are  given  off  from  the  tendon  just 
before  it  enters  the  capsule  which  attach  the  tendon  to  the  outer  surface 
of  the  latter. 

The  secondary  insertions  of  the  tendons  are : 

I.  Check  ligaments  or  firm  bands  of  fascia  extending  from  the 
anterior  portion  of  the  muscles  to  the  orbital  walls. 

II.  The  palpebral  bands  extending  to  the  septum  orbitale  and  the 
lids. 

The  check  ligament  of  the  internal  rectus  unites  with  that  of  the 
inferior  rectus  and  is  inserted  into  the  lachrymal  bone  behind  the  lach- 
rymal sac. 

The  external  rectus  has  two  check  ligaments, — one  below,  the  other 
above, — both  attached  to  the  outer  ^vall  of  the  orbit  near  its  margin. 

The  superior  rectus  and  levator  palpebrae  have  two  check  ligaments. 
The  outer,  broad  and  firm,  fuses  with  the  upper  check  ligament  of  the 
externus  and  forms  a  firm  septum  supporting  the  lachrymal  gland,  and 
is  attached  to  the  upper  wall  of  the  orbit  near  its  margin.  The  inner 
check  ligament  is  attached  to  the  upper,  inner  part  of  the  orbit  as  far 
forward  as  the  trochlea. 

The  superior  oblique  has  two  check  ligaments — one,  the  posterior, 
running  from  the  belly  of  the  muscle  to  the  trochlea;  the  other,  the 
anterior,  running  from  the  trochlea  backward  upon  the  reflected  tendon 
to  which  it  is  attached. 

The  inferior  oblique  has  no  proper  check  ligament.  It  is  attached 
by  very  strong  fibrous  bands  to  the  inferior  rectus,  the  fascia  here  being 


190  THE    EYE    AND    NERVOUS    SYSTEM 

so  dense  and  strong  as  to  act  like  a  sling  supporting  the  eyeball  (hence, 
called  by  some  tlie  suspensory  ligament  of  the  eye). 

The  action  of  a  check  ligament  is  twofold :  first,  it  restricts  the  action 
of  the  corresponding  muscle  so  as  to  limit  very  considerably  the  rota- 
tion the  latter  would  otherwise  produce;  second,  it  prevents  the  recti 
muscles  from  retracting  the  eyeball  into  the  orbit.  In  fact,  the  eyeball, 
because  it  is  held  by  the  various  check  ligaments,  and  also  because  it 
is  pulled  in  opposite  directions  by  the  obliques  (which  tend  to  draw  it 
forward),  and  by  the  recti  (which  tend  to  draw  it  backward),  is  kept 
slung  in  one  position,  so  that,  instead  of  advancing  or  receding,  it  simply 
rotates  in  its  socket. 

The  place  of  a  check  ligament  for  the  inferior  oblique  is  taken  by 
tlie  anterior  ligament  of  the  superior  oblique  which  becomes  tense  in 
proportion  as  the  inferior  oblique  contracts  and  the  superior  oblique 
relaxes. 

The  palpebral  bands  are  the  superior,  running  to  the  tarsus  of  the 
upper  lid  from  the  fascia  connecting  the  superior  rectus  with  the  levator 
palpebral,  and  the  inferior,  running  to  the  tarsus  of  the  lower  lid  from 
the  fascia  between  the  inferior  rectus  and  the  inferior  oblique.  When 
the  superior  rectus  contracts,  the  superior  palpebral  band  is  pulled  upon 
and  the  upper  lid  is  elevated  so  that  lid  and  eye  rise  together.  So  also 
when  the  inferior  rectus  contracts,  the  lower  lid  is  pulled  downwards 
by  the  inferior  palpebral  band. 

Actions  of  the  Ocular  Muscles. — The  actions  of  the  eye  muscles  may  be 
divided  into  main  and  subsidiary. 

1.  The  main  actions  are  as  follows :  The  external  rectus  abducts 
the  eye, — i.e.,  moves  it  straight  outwards  or  towards  the  temple. 

The  internal  rectus  adducts  the  eye, — i.e.,  moves  it  straight  in  or 
towards  the  nose. 

The  superior  rectus  elevates  the  eye.  As  the  muscle  runs  from 
within  outward,  its  elevating  action  will  be  greatest  with  the  eye  ab- 
ducted, because  then  it  will  be  in  line  with  the  anteroposterior  axis  of 
the  eye  and  will  thus  work  to  the  greatest  advantage.  On  the  other 
hand,  when  the  eye  is  adducted  the  muscle  will  be  nearly  at  right  angles 
to  the  anteroposterior  axis  of  the  eye,  and  will  hence  have  little  or  no 
action  as  an  elevator. 

The  inferior  oblique  acts  to  pull  the  back  part  of  the  eye  down,  and 
hence  throws  the  front  part  up.  Like  the  superior  rectus,  therefore,  it 
is  an  elevator  of  the  eye.  As  it  runs  quite  obliquely  backward  and  out- 
ward its  elevating  action  will  be  greatest  when  the  eye  is  adducted,  for 
then  the  muscle  will  be  nearly  in  line  with  the  anteroposterior  axis  of 


THE  EXTRA-OCULAK  MUSCLES  191 

the  eye.  On  the  other  hand,  when  the  eye  is  abducted  its  elevating  action 
will  be  practically  nil. 

The  inferior  rectus  depresses  the  eye,  and,  like  the  superior  rectus 
to  which  it  is  nearly  parallel,  it  will  depress  the  eye  most  energetically 
when  the  latter  is  abducted,  and  little  or  not  at  all  when  it  is  adducted. 

The  superior  oblique  elevates  the  back  part  of  the  eye,  and  hence 
throws  the  front  part  down  like  the  inferior  rectus;  therefore,  it  is  a 
depressor,  but,  owing  to  the  oblique  way  in  which  it  runs,  it  depresses 
the  eye  the  most  when  the  latter  is  adducted,  and  little  or  not  at  all 
when  it  is  abducted. 

The  levator  lifts  the  upper  lid. 

2.   The  subsidiary  actions  of  the  eye  are  as  follows : 

I.  Lateral  Action  of  Vertical  Muscles. — The  superior  and  inferior 
recti,  owing  to  the  obliquity  of  their  course,  adduct  the  eye,  and  the 
more  so  when  the  eye  is  already  adducted. 

The  two  obliques,  for  a  similar  reason,  abduct  the  eye,  and  the  more 
so  the  more  the  eye  is  already  abducted. 

II.  Torsion  Movements. — The  superior  rectus  and  the  superior 
oblique  intort  the  eye,- — i.e.,  rotate  the  top  of  its  vertical  meridian 
inward,  or  towards  the  nose. 

The  inferior  oblique  and  inferior  rectus  extort  the  eye, — i.e.,  rotate 
its  vertical  meridian  outward. 

The  intorting  action  of  the  superior  rectus  and  the  extorting  action  of 
the  inferior  rectus  are  most  pronounced  when  the  eye  is  adducted ;  the 
intorting  and  extorting  action  of  the  obliques  is  most  pronounced  when 
the  eye  is  abducted. 

III.  Action  of  Eye-Muscles  on  Lids. — The  superior  rectus  by  virtue 
of  its  palpebral  band  elevates  the  upper  lid,  and  the  inferior  rectus 
depresses  the  lower  lid. 

Monocular  Movements. — From  what  has  just  been  said  it  is  evi- 
dent that  the  eye  is  adducted  mainly  by  the  internal  rectus,  assisted 
towards  the  end  of  its  course  by  the  superior  and  inferior  recti. 

It  is  abducted  by  the  external  rectus,  reinforced  towards  the  end  of 
its  course  by  the  two  obliques. 

It  is  elevated  by  the  superior  rectus  and  inferior  oblique.  The  supe- 
rior rectus  acting  alone  would  carry  it  up  and  a  little  in ;  the  inferior 
oblique  acting  alone  would  carry  it  up  and  a  little  out ;  the  two  muscles 
acting  together  would  carry  it  straight  up.  It  is  quite  certain  from 
the  results,  both  of  physiological  experiment  and  the  data  derived  from 
cases  of  paralysis,  that  when  we  look  up  both  muscles  do  always  act 
together.    "Wlien  we  look  straight  up,  both  act  quite  strongly  as  elevators, 


192  THE    EYE    AND    KERVOUS    SYSTEM 

although  the  superior  rectus,  having  the  less  oblique  course,  is  rather 
more  efficient  than  tlie  inferior  oblique.  \Vhen  we  look  up  and  out, 
although  both  muscles  still  act,  the  superior  rectus  is  the  only  one  that 
lifts  the  eye,  the  superior  oblique  simply  tending  to  abduct  it  still  more 
and  thus  reinforce  the  action  of  the  external  rectus.  Per  contra,  when 
we  look  up  and  in,  the  inferior  oblique  is  the  main  agent  in  lifting 
the  eye,  the  superior  rectus  simply  acting  to  carry  the  eye  inward  and 
so  reinforce  the  action  of  the  internal  rectus. 

In  all  movements  of  elevation  of  tlie  eye,  whether  straight  up,  up 
and  in,  or  up  and  out,  the  upper  lid  is  simultaneously  elevated  by  the 
levator  and  the  superior  rectus. 

The  eye  is  depressed  by  the  inferior  rectus  and  superior  oblique. 
The  inferior  rectus  acting  alone  would  carry  it  do%vn  and  a  little  in ; 
the  superior  oblique  down  and  a  little  out  As  a  matter  of  fact,  both 
muscles  always  act  together  and  the  eye  is  carried  straight  down  through 
their  combined  action,  the  inferior  rectus  being  rather  the  more  effective 
of  the  two.  WTien  we  look  down  and  out  the  inferior  rectus  carries  the 
eye  down  and  the  superior  oblique  carries  it  out,  reinforcing  the  action 
of  the  externus.  \Mien  we  look  down  and  in  the  superior  oblique  carries 
the  eye  down,  the  inferior  rectus  acting  simply  to  carry  the  eye  in  and 
reinforce  the  action  of  the  internal  rectus. 

In  all  movements  of  depression  of  the  eye,  whether  straight  do^vn, 
or  do^\^l  and  in,  or  dowTi  and  out,  the  lower  lid  is  simultaneously  de- 
pressed by  the  inferior  rectus. 

Movements  of  the  Lids. — The  upper  lid  is  raised  by  the  levator,  as- 
sisted by  an  unstriated  muscle,  the  superior  muscle  of  Miiller,  which  is 
supplied  by  the  sympathetic.  Moreover,  when  the  eye  is  elevated,  so 
that  the  superior  rectus  is  brought  into  play,  this  muscle  also  takes  part 
in  raising  the  lid. 

The  lower  lid  is  depressed,  partly  by  its  ovm  weight,  partly  by  the 
contraction  of  the  unstriated  inferior  muscle  of  Miiller,  which  is  sup- 
plied by  the  sympathetic.  [Moreover,  when  the  eye  is  depressed,  so  that 
the  inferior  rectus  is  brought  into  action,  this  muscle  also  takes  part  in 
depressing  tlie  lid  (See  Chapter  VII). 

Field  of  Fixation. — By  the  action  of  the  several  muscles,  either  single 
or  combined,  the  eye  is  able  to  make  excursions  in  every  direction — 
up,  down,  in,  out,  and  obliquely.  In  this  way,  without  moving  his  head, 
one  can  fix  in  succession  all  the  objects  occupying  a  large  portion  of 
the  space  about  him.  By  thus  moving  the  eye  as  far  as  possible  in 
every  direction,  we  delimit  what  is  called  the  field  of  fixation.  (See 
Section  on  Tests.) 


THE  EXTRA-OCULAR  MUSCLES  193 

Position  of  Equilibrium,  or  Primary  Position. — When  all  the  muscles 
that  move  the  eye  are  at  rest,  or  at  least  when  no  one  of  them  is  con- 
tracting more  than  any  of  its  fellows,  the  eye  assumes  its  position  of 
equilibrium,  or  what  is  called  the  primary  position.  This  position,  in 
the  great  majority  of  cases,  is  such  that  the  line  of  sight  is  perpendicular 
to  the  line  connecting  the  centres  of  rotation  of  the  two  eyes,  and  lies 
in  or  slightly  below  the  horizontal  plane, — i.e.,  the  eye  looks  right  ahead 
and  on  the  level  or  a  little  below  it. 

Field  of  Action  of  the  Muscles. — As  we  have  just  seen,  the  external 
rectus  acts  to  carry  the  eye  directly  outward.  "When  the  eye  is  turned 
in,  this  muscle  relaxes,  and  the  only  effect  it  produces  is  that  due  to  the 
passive  traction  exerted  by  its  tendon.  Thus,  the  external  rectus  acta 
only  in  the  outer  half  of  the  field  of  fixation.  This  outer  half  of  the 
field  of  fixation  we  may  then  denominate  the  field  of  action  of  the 
external  rectus.  Similarly  the  field  of  action  of  the  superior  rectus 
will  be  the  upper  half  of  the  field  of  fixation,  and  if  we  have  regard 
only  to  the  elevating  action  of  the  superior  rectus,  its  field  of  action 
will  be  confined  mainly  to  the  upper  outer  quadrant  of  the  field  of 
fixation. 

From  a  similar  consideration  it  is  evident  that  each  one  of  the  six 
muscles  moving  the  eye  has  its  special  field  of  action,  as  shown  in  the 
following  table: 

Muscle.  Special  Field  of  Action. 

External  rectus.  Outer  half  of  field  of  fixation. 

Internal  rectus.  Inner  half  of  field  of  fixation. 

Superior  rectus  (as  an  elevator).  Upper   and   outer  quadrant   of   field   of 

fixation. 
Inferior  oblique    (as  an  elevator).  Upper   and    inner   quadrant   of   field   of 

fixation. 
Inferior  rectus   (as  a  depressor).  Lower   and   outer   quadrant  of   field   of 

fixation. 
Superior  oblique   (as  a  depressor).  Lower   and   inner   quadrant  of   field   of 

fixation. 

Binocular  Movements. — The  two  eyes  when  acting  together  can- 
not move  independently  of  each  other.  The  whole  purpose  of  the  neuro- 
muscular apparatus  of  the  eyes  is  to  secure  binocular  fijxation.  Hence, 
movements  which  will  interfere  with  binocular  fixation  are  not  ordi- 
narily performed,  and,  more  than  that,  cannot  be  performed  except  to 
a  very  limited  degree.  The  movements  actually  possible  to  the  two  eyes 
working  together  are  as  follows : 

I.  Parallel  Movements. — This  class  of  movements  is  employed  when 
we  carry  the  eye  from  one  distant  object  to  another.  In  this  case  the 
13 


194  THE    EYE    AND    NERVOUS    SYSTEM 

two  visual  lines  remain  parallel  with  each  other,  both  moving  equally 
far  and  equally  fast  to  the  right,  to  the  left,  up,  down,  and  obliquely. 

An  analysis  of  the  statements  made  regarding  the  actions  of  the 
muscles  for  moving  each  eye  individually  (see  Section  on  Monocular 
Movements)  will  show  that  the  twelve  muscles  moving  both  eyes  may 
be  classed  as  follows : 

A.  LATERAL    ROTATORS. 

Producing  lateral  rotation  of  both  eyes — lateri version* 

(o)   Right    rotators    (acting    when    the        (6)  Left  rotators  (acting  when  the  eyes 

eyes  are  directed  to  the  right).  are  directed  to  the  left). 

Producing  rotation  to  the  right  (dewtro-       Producing    rotation    to    the    left     {levo- 
version ) .  version ) . 

R.  External  rectus.  R.  Internal  rectus. 

L.  Internal  rectus.  L.  External  rectus. 

B.  ELEVATORS. 

Producing  elevation  of  both  eyes   (sursumversion) . 
(a)   Right-handed      elevators       (acting        (b)   Left-handed        elevators         (acting 
mainly    when    the    eyes    are    di-  mainly    when    the    eyes    are    di- 

rected to  the  right).  rected    to    the    left). 

R.  Superior  rectus.  R.  Inferior  oblique. 

L.  Inferior  oblique.  L.  Superior  rectus. 

C.  DEPRESSORS. 

Producing  depression  of  both  eyes   (deorsumversion) . 
(a)   Right-handed     depressors      (acting        (6)   Left-handed       depressors       (acting 
mainly  when  the  eyes  are  directed  mainly  when  the  eyes  are  directed 

to  the  right).  to  the   left). 

R.  Inferior  rectus.  R.  Superior  oblique. 

L.  Superior  oblique.  L.  Inferior  rectus. 

It  will  be  seen  that  the  twelve  muscles  are  divided  into  three  groups, 
and  each  group  comprises  two  pairs,  the  muscles  of  one  pair  acting  when 
the  eyes  are  directed  towards  the  right,  the  muscles  of  the  other  pair 
acting  when  tlie  eyes  are  directed  to  the  left. 

This  makes  six  pairs  of  muscles — one  muscle  of  each  pair  being  in 
the  right  eye,  the  other  in  the  left.  The  two  muscles  constituting  a 
pair  are  called  associates.  Two  associates  acting  together  will  move 
the  right  eye  and  the  left  eye  respectively  in  the  same  direction  and  to 
the  same  extent  Thus,  the  right  inferior  rectus  moves  the  right  eye 
down,  to  the  left,  and  rotates  its  vertical  meridian  to  the  right.     Its 


•  In  lateri  version  the  movements  of  abduction  made  by  one  eye  and  of  adduction 
made  by  the  other  are  called  latero-abduction  and  latero-adduction,  respectively 
( Schapringer ) .  They  are  to  be  distinguished  from  divergence-abduction  and  con- 
vergence-adduction (pages  195,  196). 


THE  EXTRA-OCULAR  MUSCLES  195 

associate,  the  left  superior  oblique,  moves  the  left  eye  down,  to  the  left, 
and  rotates  its  vertical  meridian  to  the  right. 

The  precision  with  which  parallel  movements  are  performed  is  re- 
markable. Under  ordinary  circumstances,  parallelism  is  maintained 
up  to  the  limits  of  excursion  of  the  eyes  in  any  given  direction.  If  this 
is  not  the  case — if  one  eye  for  any  reason  fails  to  keep  pace  with  the 
other  in  advancing  in  any  particular  direction — diplopia  ensues.  The 
point  where  such  diplopia  begins  indicates  the  limits  of  binocular  single 
vision  in  that  special  direction  of  the  gaze,  and  by  determining  these 
limits  for  all  directions  of  the  gaze — right,  left,  itp,  down,  and  obliquely 
— we  can  map  out  what  is  called  the  field  of  binocular  vision. 

As  we  shall  see  later,  any  marked  restriction  of  the  field  of  binocular 
single  vision  in  some  one  direction  of  the  gaze,  indicates  an  insufficiency 
or  paralysis  of  one  of  the  ocular  muscles. 

There  appears  to  be  a  distinct  nerve-centre  or  tract  governing  each 
one  of  the  different  parallel  movements  of  the  eyes, — i.e.,  there  is  one 
centre  governing  the  movements  to  the  right,  one  governing  the  move- 
ments to  the  left,  one  governing  upward  movements,  etc.  The  centres 
for  the  initiation  of  such  movements  {centres  of  voluntary  movement) 
are  apparently  in  the  frontal  lobe  (Russell,  Mott  and  Schafer,  Sherring- 
ton, Topolanski).  In  addition  to  these  there  are  posterior  cortical  cen- 
tres which  are  undoubtedly  situated  in  the  angular  gyrus  (Bernheimer). 
These  are  reflex  centres, — i.e.,  designed  for  movements  set  up  in  a  reflex 
way  by  visual  impressions.  Stimulation  of  either  the  anterior  or  the 
posterior  cortical  centres  on  the  right  side  produces  a  movement  of  both 
eyes  to  the  left  (or  up  and  to  the  left  or  down  and  to  the  left),  and,  if 
very  strong,  also  produces  inhibition  of  the  movement  to  the  right.  If  the 
stimulus  starts  from  the  angular  gyrus,  it  crosses  over  just  beneath  the 
aqueduct  to  reach  the  subcortical  centres  which  are  found  in  and  about 
the  nerve  nuclei  of  the  opposite  side.  The  thalami  and  corpora  quadri- 
gemina  take  no  part  in  the  production  of  parallel  movements  (Topo- 
lanski). 

11.  Movements  of  Convergence.^ — ^When  we  pass  from  a  distant  object 
to  one  nearer  we  have  to  converge  the  eyes.  We  do  this  by  means  of 
a  simultaneous  and  equal  contraction  of  the  two  internal  recti,  produc- 
ing an  equal  movement  inward  {convergence-adduction)  of  each  eye. 
This  movement  of  convergence  may  be  combined  with  a  lateral  or  verti- 
cal parallel  movement,  as  when  we  look  at  a  near  object  that  is  situated 
to  the  right  or  left,  or  is  situated  above  or  below  the  horizontal  plane. 

The  centre  for  the  initiation  of  convergence-movements  (voluntary 
movements  of  convergence)  is  probably  in  the  frontal  lobe  (according 


196  THE    EYE    AND    NEKVOUS    SYSTEM 

to  two  experiments  of  Sherrington's,  near  the  angle  of  the  preeentral 
fissure).  Sherrington  also  found  that  inJiibition  of  convergence  could 
be  produced  by  cortical  stimulation.  The  posterior  cortical  {reflex) 
centres  are  said  by  Bechterew  to  be  in  the  occipital  lobe  behind  the 
junction  of  the  Sylvian  with  the  first  temporal  fissure  and  in  the  angu- 
lar gyrus.  The  subcortical  centres,  according  to  Bechterew,  are,  first, 
one  in  the  third-nerve  nucleus,  probably  connected  with  the  frontal 
centre;  and,  second,  one  in  the  corpora  quadrigemina,  probably  con- 
nected with  the  posterior  cortical  centres.  According  to  Bernheimer, 
the  first  of  these  subcortical  centres  would  comprise  the  partial  nuclei 
for  the  internal  rectus  on  either  side,  the  commissural  fibres  connect- 
ing the  right  and  left  partial  nuclei,  and  the  fibres  running  from  each 
partial  nucleus  to  the  internus  of  the  same  side  (the  crossed  fibres 
running  to  the  internus  of  the  other  side  subserving  not  convergence 
but  latero-adduction).     (Compare  Page  13). 

III.  Movements  of  Divergence. — When  our  eyes  are  converged  on  a 
near  object  and  we  then  look  at  an  object  more  remote,  the  lines  of 
sight  of  the  two  eyes  separate,  or,  as  we  say,  the  eyes  diverge.  This 
movement  of  divergence  is  equal  and  simultaneous  in  the  two  eyes,  each 
one  moving  out  equally  far  and  fast.  According  to  some,  the  movement 
is  produced  by  the  relaxation  of  the  two  internal  recti  (passive  diver- 
gence) ;  according  to  others,  by  the  contraction  of  the  two  external  recti 
(active  divergence,  divergence-abduction).  In  all  probability,  it  is 
really  due  to  these  two  actions  combined.  According  to  Bechterew,  the 
centre  for  the  initiation  of  divergence  (voluntary  divergence  move- 
ments) lies  in  the  frontal  lobe  in  front  of  the  middle  of  the  preeentral 
fissure.  The  posterior  (reflex)  centre  is  in  the  angular  gyrus.  The 
subcortical  centre  is  in  the  abducens  nuclei. 

IV.  Divergence  in  the  Vertical  Plane. — To  a  very  slight  extent  the 
lines  of  sight  of  the  two  eyes  can  separate  in  a  vertical  direction.  The 
movement  is  doubtless  equal  and  simultaneous  in  the  two  eyes,  one  eye 
rising  while  the  other  descends.  It  is  called  supravergence,  and  is 
denoted  as  right  or  left,  according  as  the  right  or  left  eye  is  the  higher. 

Tests. — In  testing  for  derangements  of  the  ocular  muscles  and  ocular 
movements  we  have  to  determine: 

I.'  \Miether  each  eye  by  itself  can  perform  fixation.  (Tests  of  mon- 
ocular fixation.) 

II.  WTiether,  when  in  the  primary  position,  both  eyes  always  or 
usually  perform  binocular  fixation  or  whether  they  deviate.  (Tests 
of  binocular  fixation.) 

III.  Whether,  the  eyes  being  in  the  primary  position,  the  patient 


THE  EXTRA-OCULAR  MUSCLES  197 

has  or  has  not  binocular  vision,  or,  if  he  has,  whether  he  has  binocular 
single  vision,  or  else  either  diplopia  or  a  tendency  to  diplopia.  (Tests 
for  binocular  vision ;   diplopia  tests.) 

IV.  Whether  each  eye  by  itself  can  move  freely  in  all  directions. 
(Tests  of  monocular  movement;   determination  of  field  of  fixation.) 

V.  Whether  the  two  eyes  working  together  can  perform  their  regu- 
lar co-ordinated  movements,  and  particularly  whether  they  can  perform : 

(a)  Parallel  movements,  (Determination  of  binocular  excursions 
and  of  the  field  of  binocular  single  vision.) 

(&)   Movements  of  convergence.     (Tests  of  convergence.) 

(c)  Movements  of  divergence.     (Tests  of  divergence.) 

A  great  many  tests  are  utilized,  but  only  the  more  important  will 
be  mentioned  here;  those  given,  however,  being  sufficient  in  all  ordi- 
nary cases  to  make  an  adequate  diagnosis. 

In  making  all  the  tests  the  patient's  head  must  be  kept  erect  and 
motionless,  and  the  face  *  must  not  be  turned  either  to  the  right  or  to  the 
left. 

The  test-object  used  should  be  sharply  defined,  be  just  large  enough, 
be  distinctly  seen,  offer  a  marked  contrast  to  its  surroundings,  and  be 
in  the  same  plane  as  the  latter.  An  excellent  test-object  is  a  white  target 
20  to  30  centimetres  in  diameter,  wdth  a  black  bull's-eye.  The  latter, 
if  the  tests  are  made  for  distance  (fifteen  or  twenty  feet),  should  be 
8  millimetres  in  width,  and  the  target  itself  should  then  be  set  flat  on  a 
large  dead-black  surface.  If  the  tests  are  made  at  near  points  the  bull's- 
eye  should  be  1  or  at  most  2  millimetres  wide.  Another  good  test- 
object,  indispensable  for  some  of  the  tests,  is  a  small  brilliant  light  set 
against  a  large,  flat,  dead-black  background.  For  near  tests  this  may 
conveniently  take  the  form  of  a  minute  electric  light  set  in  the  centre  of 
a  metal  disk  which  is  covered  with  black  cloth. 

In  most  of  our  tests  we  measure  the  amount  of  deviation  present  by 
means  of  prisms.  In  so  doing  we  must  recollect  that  prisms  are  usually 
numbered  according  to  their  refracting  angle,  and  that  the  amount  of 
deviation  that  they  produce — and  consequently  the  amount  of  deviation 
that  they  measure — is  only  one-half  or  a  little  more  than  one-half  of 
this  angle.  Thus  a  deviation  that  is  neutralized  by  a  prism  of  10°  is 
actually  one  of  only  5°  of  arc,  a  deviation  neutralized  by  a  prism  of  20° 
is  11°  of  arc,  etc. 

I.  Tests  of  Monocular  Fixation. — We  make  the  patient  cover  one  eye 
and  then  direct  his  other  eye  sharply  at  some  well  defined  object,  like 


Except  when  determining  the  field  of  binocular  single  vision  for  distance. 


198  THE    EYE    AND    NERVOUS    SYSTEM 

a  pencil  or  a  light,  held  straight  in  front  of  him.  Inspection  then  will 
show,  approximately  at  least,  whether  his  fixation  is  central  or  eccentric, 
or  whether,  as  in  cases  of  a  high  grade  amblyopia,  he  cannot  fix  at  all, 
but  allows  his  eye  to  wander  aimlessly,  getting  mere  glimpses  of  the 
object  shown  him. 

II.  Tests  of  Binocular  Fixation. — Of  the  several  tests  for  binocular 
fixation,  by  far  the  best  and  most  reliable  is  the  screen  test,  and  this 
alone  will  be  described  here.  In  performing  this  we  have  the  patient 
look  at  an  object  fifteen  feet  or  more  from  the*  eyes. 

The  patient  is  directed  to  look  sharply  at  the  object  (bull's-eye  or 
light),  and  then  the  card  is  placed  before  the  right  eye  and  passed 
quickly  to  and  fro  from  one  eye  to  the  other. 

If  there  is  orthophoria,  neither  eye  will  deviate  when  covered,  and 
each  consequently  will  remain  steady  when  the  screen  is  removed.  If 
there  is  squint  or  heterophoria  of  more  than  a  degree  or  two,  each  eye 
when  covered  will  deviate  perceptibly,  and  when  uncovered  will  turn 
back  into  the  fixing  position.  Thus,  if  there  is  either  an  esophoria  or 
convergent  squint  each  eye  in  turn  will  deviate  in  or  towards  the  nose 
when  covered,  and  will  swing  out  again  when  uncovered. 

Whether  the  two  eyes  deviate  to  the  same  amount  or  not  behind  the 
screen  will  depend  upon  which  has  the  stronger  muscles.  Equally,  in 
the  case  of  paralysis  of  the  right  eye  with  normal  musculature  in  the 
left,  and  in  the  case  of  spasm  of  the  left  eye  with  normal  musculature  in 
the  right,  the  deviation  of  tlie  right  eye  behind  the  screen  will  be  less 
than  that  of  the  left.  (Compare  remarks  under  Diagnosis  of  Paralysis, 
Section  II.,  and  Symptoms  of  Spasm,  Section  III.)  The  same  thing 
occurs  in  certain  cases  of  squint.  (See  Note  to  Table  of  Periodic 
Heterophoria  and  Squint,  Section  IV.) 

The  amount  of  the  deviation  can  be  measured  accurately  by  means 
of  the  prism  that  abolishes  it  Thus,  if  the  eye  is  deviated  out  behind 
the  screen,  showing  that  there  is  an  exophoria  or  a  divergent  squint, 
the  amount  of  the  deviation  may  be  measured  by  the  prism,  placed  base 
in  before  the  eye,  that  stops  the  movement  altogether.  Usually,  in 
measuriiig  the  deviation  in  this  way  we  add  prisms  until  the  movement 
is  not  simply  abolished,  but  is  converted  into  a  movement  in  the  opposite 
direction.  The  first  prism  that  causes  this  over-correction,  less  2°,  will 
represent  pretty  closely  the  amount  of  the  deviation. 

Having  ascertained  in  this  way  that  a  deviation  is  present  and  how 
much  it  is,  we  next  inquire  whether  this  deviation  is  a  squint  or  a 
heterophoria. 

To  determine  this  we  make  the  patient  look  fixedly  at  the  test-object 


THE  EXTRA-OCULAR  MUSCLES  199 

with  both  ejes  open,  and  then  alternately  cover  and  uncover  one  eye, — 
say  the  right, — leaving  the  other  all  the  time  uncovered  (method  of 
binocular  uncovering).  By  thus  covering  the  right  eye  we  compel  the 
left  eye  to  fix,  if  it  is  not  already  doing  so,  and  we  allow  the  right  eye  to 
deviate  if  it  has  any  tendency  to  do  so  if  it  is  not  already  deviating. 

In  this  case  three  conditions  may  obtain : 

(a)  There  may  be  lieterophoria.  In  this  event  the  left  eye  which 
is  fixing  already  will  continue  fixing  when  the  right  eye  is  covered, 
and  the  latter  which  was  fixing  before  being  covered  will  not  deviate. 
Then,  when  the  cover  is  taken  off  again,  the  right  eye  will  swing  back 
into  place  and  the  left  will  still  remain  fixing, — i.e.,  both  on  applying 
and  on  removing  the  screen  but  one  eye  will  move,  and  that  the  right. 

(6)  There  may  be  a  squint  of  the  right  eye.  In  this  case  the  left 
eye  which  was  fixing  before  the  right  was  covered  will  continue  fixing 
afterwards,  and  the  right  eye  which  was  deviating  before  it  was  cov- 
ered will  remain  deviating  afterwards.  Then,  on  uncovering,  the  left 
eye  will  still  remain  fixing  and  the  right  will  still  remain  deviating, — 
i.e.,  both  on  applying  and  on  removing  the  screen  neither  eye  moves,  pro- 
vided the  screen  is  put  over  the  squinting  eye. 

(c)  There  may  be  a  squint  of  the  left  eye.  Then  the  left  eye,  which 
was  deviating  before  the  right  eye  was  covered,  will  now  have  to  move 
into  place  in  order  to  fix.  As  it  moves  into  place,  the  right  eye,  which 
is  behind  the  screen,  will  move  out  of  place.  Then,  when  the  screen  is 
removed,  the  right  eye  which  has  thus  become  deviated,  will  move  back 
into  the  fixing  position,  since  it  is  the  right  eye  which  ordinarily  fixes 
when  both  eyes  are  open,  and  the  left  eye  will  move  out  of  the  fixing 
position  and  back  to  its  ordinary  position  of  deviation, — i.e.,  both  on 
applying  and  on  removing  the  screen  both  eyes  will  move,  provided  in 
the  case  of  a  squint  the  screen  is  put  before  the  fixing  eye. 

If  repeated  applications  of  the  test  show  that  sometimes  the  right 
eye  squints,  sometimes  the  left,  then  we  are  evidently  dealing  with 
an  alternating  strabismus. 

III.  Tests  of  Binocular  Vision. — To  determine  whether  the  patient 
has  binocular  vision  or  not,  and,  in  case  he  has  binocular  vision,  whether 
there  is  diplopia  or  a  tendency  to  diplopia,  we  employ  a  large  number 
of  tests. 

As  has  already  been  said,  the  tests  of  binocular  vision  do  not  agree 
ivith  those  for  binocular  fixation, — e.g.,  with  the  screen  test, — if  there 
is  any  incongruity  of  the  double  images  (See  Page  187). 

Again,  if  there  is  suppression  of  the  image  in  one  eye,  such  as 
regularly  takes  place  in  squint,  all  the  tests  for  binocular  vision  will 


200  THE    EYE    AND    NERVOUS    SYSTEM 

fail.    Per  contra,  the  failure  of  these  tests  in  any  given  case  argues  the 
existence  of  suppression. 

Only  the  more  important  tests  will  be  given  here : 

1.  Parallax  Test. — As  we  are  passing  the  card  from  eye  to  eye  in 
making  the  screen  test,  we  ask  the  patient  whether  the  object  he  is 
looking  at  seems  to  move  or  not.  If  it  does,  it  means  that  he  actually 
sees  the  object  double,  only  instead  of  seeing  the  two  images  simultane- 
ously he  sees  one  after  the  other.  Thus,  if  on  uncovering  the  right  eye 
the  object  seems  to  move  to  the  right  it  means  that  the  right-eye  image 
is  to  the  right  of  the  left, — i.e.,  he  has  homonymous  diplopia.  If,  under 
the  same  conditions,  the  object  had  seemed  to  move  to  the  left  he  would 
really  have  had  crossed  diplopia.  If  it  had  moved  down  he  would  have 
had  a  right  diplopia ;   if  up,  a  left  diplopia. 

In  order  to  be  sure  that  the  patient  is  actually  fixing  the  object  and 
not  looking  beyond  it,  so  that  he  sees  it  projected  against  a  background 
more  remote,  the  test-object  should  be  on  the  same  plane  as  the  objects 
immediately  surrounding  it  Hence,  as  already  stated,  we  make  the 
patient  look  at  a  small  spot  or  light  set  on  a  large  blank  surface. 

The  amount  of  the  parallax,  whether  homonymous,  crossed,  right, 
or  left,  as  the  case  may  be,  may  be  measured  accurately  by  the  prism 
which,  placed  before  the  eyes,  will  cause  its  abolition.  Thus,  an  homony- 
mous parallax  which  is  completely  abolished  by  a  prism  of  15°  base 
out,  indicates  an  esophoria  of  15°,  etc. 

2.  Red  Glass. — If  there  is  much  tendency  to  diplopia  the  mere  inter- 
position of  a  red  glass  before  one  eye  will  evoke  it  In  this  case  the 
test  object  should  be  a  light,  of  which,  then,  two  images  will  be  seen — 
one  red,  the  other  white.  This  proves  that  the  patient  has  binocular 
vision  with  diplopia.  If  the  red  glass  is  placed  before  the  right  eye 
the  red  image  will  be  on  the  right  hand  if  the  diplopia  is  homonymous ; 
on  the  left  hand  if  the  diplopia  is  crossed ;  below  if  there  is  right  diplo- 
pia, and  above  if  there  is  left  diplopia. 

If,  with  the  red  glass  before  one  eye,  the  patient  sees  a  single  light 
which  is  white  tinged  with  red,  he  surely  has  no  diplopia,  and  has  binoc- 
ular single  vision. 

If  he  sees  a  single  light  which  is  pure  white  or  pure  red,  he  is  sup- 
pressing the  image  in  one  eye  (page  178).  In  this  case  he  has  no 
diplopia,  and  has  only  monocular  vision. 

The  amount  of  deflection,  and  hence  the  amount  of  deviation,  may 
be  determined  either  by  ascertaining  the  linear  distance  between  the 
double  images  (allowance  being  made  for  the  distance  of  the  object 
from  the  patient),  or  by  finding  out  what  prism  will  be  required  to  over- 


THE  EXTEA-OCULAR  MUSCLES  201 

come  the  diplopia.  The  latter  method,  however,  usually  gives  an  insuffi- 
cient measure  of  the  amount  of  the  diplopia.  The  real  diplopia,  for 
example,  may  be  20°  and  yet  be  apparently  nullified  by  a  prism  of 
18°,  because  such  a  prism  brings  the  images  so  close  together  that  the 
patient  then  unites  them  himself  without  further  aid. 

In  testing  for  diplopia  in  this  way  we  ask  the  patient  not  only  how 
the  double  images  are  placed  with  regard  to  each  other  and  how  far 
apart  they  are,  but  also  whether  both  are  upright  or  not.  If  one  of 
the  images  is  tilted,  either  to  the  right  or  to  the  left,  it  indicates  that 
the  corresponding  eye  is  tilted  and  has  its  vertical  meridian  rotated  in 
the  opposite  direction.  Thus,  if  the  candle  seen  by  the  right  eye  tilts 
to  the  right,  it  means  that  the  right  eye  itself  is  tilted  to  the  left  or  has  a 
pathological  intorsion. 

3.  Maddox  Rod. — The  Maddox  rod  consists  of  one  or  more  little 
cylinders  of  glass  set  in  a  frame  (Fig.  6).     If  the  instrument  is  held 

Fig.  6. 


as  shown  in  the  figure  and  placed  before  the  right  eye,  the  patient, 
looking  at  the  light,  will  see  with  this  eye  the  light  transformed  into  a 
luminous  vertical  streak.  If  he  has  neither  esophoria  nor  exophoria 
this  streak  will  appear  to  run  through  the  flame  which  in  its  unaltered 
form  he  sees  with  the  left  eye.  If  he  has  esophoria  it  will  appear  to  the 
right  of  the  flame ;   if  he  has  exophoria,  to  the  left  of  it. 

If  now  the  rod  is  turned  at  right  angles  to  the  position  shown  in  the 
cut  and  again  placed  before  the  right  eye,  the  patient,  if  he  has  no 
hyperphoria,  will  see  a  horizontal  line  bisecting  the  light.  If,  how- 
ever, he  has  right  hyperphoria  the  horizontal  line  will  appear  below, 
and  if  he  has  left  hyperphoria  it  will  appear  above  the  light. 

The  amount  of  deviation  may  be  measured  by  the  linear  distance 
of  the  streak  from  the  light  (measured  on  a  tangent  scale)  or  by  the 
amount  of  prism  that  will  be  required  in  order  to  make  the  streak  coin- 
cide with  the  light. 


202  THE    EYE    AND    NERVOUS    SYSTEM 

The  Maddox  rod  can  also  be  used  to  indicate  ichether  there  is  any 
torsion  of  the  eye, — i.e.,  any  tilting  of  its  vertical  meridian  to  the  right 
or  left.  If  there  is,  the  streak  of  light  will  appear  tilted  when  the  rod 
is  set  directly  vertical,  and  the  number  of  degrees  through  which  the 
rod  has  to  be  rotated  in  order  to  make  the  line  of  light  appear  vertical, 
will  indicate  the  amount  of  the  torsion.  Thus,  if  the  vertical  meridian 
of  the  right  eye  is  rotated  10°  towards  the  temple,  we  shall  have  to  turn 
the  Maddox  rod  out  10°  in  order  to  make  the  line  of  light  appear 
vertical. 

4.  Phorometer. — The  phorometer  is  an  instrument  for  producing 
artificial  diplopia  by  means  of  prisms,  and  from  the  relation  of  the 
double  images  deducing  whether  there  is  any  tendency  to  deviation  or 
not. 

Stevens's  phorometer  consists  essentially  of  two  6°  prisms,  geared  so 
that  they  can  be  rotated  simultaneously  but  in  opposite  directions.  The 
prisms  are  first  set  so  that  their  bases  both  look  inward ;  the  patient 
then  looking  through  the  prisms  will  see  two  images  of  the  test  object, 
that  of  the  right  eye  being  on  the  right  side.     If  he  has  no  hyperphoria 


the  images  will  be  on  the  same  level.  If  he  has  right  hyperphoria  the 
right  image  will  be  lower ;    if  he  has  left  hyperphoria  it  will  be  higher. 

In  case  the  images  are  not  on  a  level  the  prisms  are  rotated  until 
they  are;  then  the  amount  of  hyperphoria  can  be  read  off  from  the 
index. 

To  measure  for  lateral  deviations  the  prisms  are  rotated  until  the 
right-hand  one  points  down,  the  left  up.     Then,  if  there  is  no  esophoria 


THE  EXTRA-OCULAR  MUSCLES 


203 


or  exophoria,  the  images  will  appear  directly  over  each  other.  If, 
however,  there  is  esophoria  the  upper  image  will  appear  on  the  left; 
if  there  is  exophoria  it  will  appear  on  the  right.  In  either  of  the  latter 
events  the  prisms  are  rotated  until  the  images  do  stand  over  each  other, 
when  the  amount  of  the  esophoria  or  exophoria,  as  the  case  may  be,  can 
be  read  off  from  the  index. 

5.  Bar-7-eading. — If  the  patient  is  directed  to  read  and  holds  a 
pencil  some  three  inches  in  front  of  the  page,  between  it  and  his  eyes, 
he  will  still,  if  he  has  binocular  vision,  be  able  to  read  every  word. 


FiG.8.— Worth's  Amblyoscope,  modified  by  Black.— The  amblyoscope  consists  of  two  tubes, bent 
and  joined  so  as  to  admit  of  being  converged  and  diverged  through  a  considerable  angle.  In  the  Black 
modification  here  shown,  one  of  the  tubes  can  also  be  raised  or  lowered,  an  arrangement  advantageous 
when  the  two  eyes  are  not  on  the  same  level.  My  own  model  is  also  fitted  with  a  graduated  arc 
which  shows  the  amount  of  the  deviation  or  of  the  convergence  and  divergence  employed.  The  fur- 
ther end  of  each  tube  carries  a  picture  mounted  on  a  translucent  plate  of  celluloid.  A  mirror  at  the 
bent  portion  of  the  tube  enables  the  patient  looking  through  the  eyepiece  to  see  the  picture  with  the 
■corresponding  eye.  If  he  has  binocular  vision  he  will  see  two  pictures,  one  with  each  eye,  and  by 
winging  the  tubes  in  or  out  he  will  get  these  two  pictures  together  and  fuse  them. 


because  the  letters  that  are  hidden  from  the  left  eye  will  be  seen  by  the 
right,  and  vice  versa.  If  he  has  not  binocular  vision,  the  pencil  ob- 
viously will  cut  out  part  of  what  he  is  reading.  Hence,  this  simple  test 
enables  us  to  tell  whether  or  not  he  is  using  one  or  both  eyes. 

6.  Stereoscope  and  Amblyoscope. — A  patient  who  has  binocular 
vision  will  be  able  to  see  two  dissimilar  images  placed  in  the  stereoscope 
and  to  fuse  them  into  a  single  image.  This,  of  course,  is  impossible 
for  one  who  has  only  monocular  vision.  Different  forms  of  the  stereo- 
.scopc  have  been  devised,  both  for  determining  the  presence  or  absence 


204 


THE    EYE    AND    NERVOUS    SYSTEM 


of  binocular  vision  and  also  for  training  binocular  vision  and  the  fusion 
faculty. 

A  similar  office  is  performed  much  more  effectively  by  the  amblyo- 
scope  (Fig.  8). 

IV.  Tests  of  Monocular  Movements:  Field  of  Fixation. — Roughly,  we 
can  ascertain  how  far  each  eye  can  move  in  any  given  direction  by 
making  it  follow  the  moving  finger  or  pencil.  When  we  make  the  test 
in  this  way  we  may  have  to  screen  the  eye  not  under  examination,  in 
order  to  make  sure  that  the  one  we  are  testing  fixes  the  moving  object 
accurately.     Otherwise,  the  test  is  performed  with  both  eyes  open,  and 


Fig.  9.— Stevens's  Tropometer. 

we  watch  the  movements  of  both  at  once, — i.e.,  the  test  is  really  one  of 
binocular  fixation  (V.  1,  Page  205). 

More  precisely,  the  range  of  excursion  of  each  eye  separately,  and 
consequently  the  field  of  fixation  of  each  eye,  can  be  determined  by  the 
tropometer  (Fig,  9),  and  the  perimeter.  With  the  latter  the  patient 
is  placed  so  that  the  eye  in  the  primary  position  is  fixing  the  zero  mark 
on  the  scale ;  then  a  fine  object,  preferably  a  pair  of  dots  set  very  close 
together  on  a  card,  is  passed  along  the  perimeter  arc  and  the  patient  di- 
rected to  follow  it,  moving  his  eyes,  but  not  his  head.  The  moment  he 
ceases  to  follow  it, — i.e.,  the  moment  that  the  eye  has  reached  the  limit 
of  its  excursion  in  that  special  direction, — the  two  dots  will  blur  and 
appear  to  him  to  run  into  one.     Then  the  point  to  which  the  test-object 


THE  EXTRA-OCULAR  MUSCLES  205 

has  been  carried  on  the  perimeter  arc  shows  the  amount  in  degrees  that 
the  eye  has  rotated  in  the  given  direction. 

By  ascertaining  tlie  limits  of  the  excursion  in  different  meridians 
the  field  of  fixation  may  be  plotted  out  on  a  perimetric  chart  in  the  same 
way  as  is  done  for  the  field  of  vision. 

V.  Tests  of  Binocular  Movements. — 1.  Tests  of  Parallel  Movements. 
— There  are  two  ways  in  which  the  ability  of  the  eyes  to  perform  paral- 
lel movements  can  be  tested. 

First,  we  may  ascertain  the  field  of  hinocular  fixation, — that  is,  we 
may  find  how  far  the  two  eyes  can  move  in  any  given  direction  and  keep 
pace  with  each  other  in  their  movements,  so  that  they  both  fix  the  same 
object.  The  usual  way  of  doing  this  is  to  make  the  two  eyes  follow  a 
pencil  or  similar  object  in  the  different  directions  of  the  gaze  and  note 
whether  either  eye  lags  or  falls  behind  in  making  the  movement.  This 
lagging  can  usually  be  detected  pretty  readily  when  there  is  a  deficiency 
of  either  inward  or  outward  rotation.  It  is  well  marked,  therefore,  in 
insufficiency  or  paresis  of  the  external  and  internal  recti.  It  is  much 
more  difficult  to  tell  whether  either  eye  lags  behind  the  other  when  the 
two  are  moving  up  or  down.  This  is  especially  the  case  when  the  eyes 
are  directed  up  and  to  the  right  or  up  and  to  the  left,  for  then  the  out- 
ward eye  usually  appears  to  be  the  lower,  even  when  it  can  be  readily 
proved  that  both  are  accurately  fixing  the  same  object. 

A  more  accurate  means  of  determining  the  field  of  binocular  fixa- 
tion is  by  making  the  screen  test  in  the  various  directions  of  the  gaze. 
A  noticeable  deviation  behind  the  screen  would  show  that  there  was  at 
least  a  tendency  to  deflection  of  the  eyes.  If  this  deviation  was  greater 
than  in  the  primary  position  it  would  show  the  deflection  was  increasing 
as  the  eyes  were  carried  away  from  the  latter;  and  if  the  method  of 
binocular  uncovering  (page  199)  should  show  that  there  was  a  squint 
in  one  of  the  outlying  positions  which  did  not  exist  in  the  primary 
position,  it  would  indicate  that  the  deflection  had  become  so  great  that 
one  eye  could  no  longer  keep  pace  with  the  other  and  perform  fixation 
with  it. 

Second,  we  may  determine  the  field  of  binocular  single  vision.  In 
doing  this  we  place  a  red  glass  before  the  patient's  right  eye  and  stand 
three  feet  in  front  of  him  with  a  light  which  we  pass  to  the  right,  to 
the  left,  up  and  to  the  right,  up  and  to  the  left,  down  and  to  the  right, 
and  down  and  to  the  left.  We  direct  him  to  follow  the  light  with  his 
eyes  without  moving  his  head,  and  if  in  any  direction  of  the  gaze  he 
gets  double  vision,  we  ask  him  how  the  images  are  related  to  each  other, 
how  far  they  are  apart,  and  whether  one  or  the  other  is  tilted  or  not. 


206  THE    EYE    AXD    NERVOUS    SYSTEM 

Normally,  the  patient  should  be  able  to  carry  the  gaze  through  a 
range  of  40°  at  least  in  any  given  direction  without  seeing  double,  and 
most  people,  indeed,  will  not  see  double  at  all,  no  matter  how  far  the 
test-object  is  carried. 

Diplopia  developing  within  30°  of  the  primary  position  is  distinctly 
pathological,  and  indicates  insufficiency  of  some  of  the  ocular  muscles. 

It  is  sometimes  important  to  determine  the  field  of  binocular  single 
vision  for  distance.  In  this  case  the  patient  is  made  to  keep  his  eyes 
fixed  on  a  small  brilliant  light  fifteen  or  twenty  feet  off  while  he  turns 
his  head  in  various  directions.  A  diplopia  developed  when  the  head  is 
turned  to  the  left,  etc.,  is  really  a  diplopia  produced  when  the  eyes  are 
turned  to  the  right,  etc.  (The  method  of  taking  and  recording  is  de- 
scribed more  fully  in  Section  II.  under  Diagnosis  of  Paralysis.) 

In  taking  the  field  of  binocular  single  vision  we  may  at  the  same 
time  take  the  field  of  binocular  fixation,  for,  as  the  patient  follows  the 
light  with  his  eyes,  we  can,  by  watching  closely,  ascertain  whether 
either  eye  seems  to  lag  when  the  light  is  carried  in  any  direction. 

2.  T'ests  of  Convergence. — These  are  threefold:  (a)  By  the  screen 
and  diplopia  tests  (Maddox  rod,  phorometer)  we  measure  the  amount 
of  any  deviation  that  may  be  present  when  the  eyes  are  converged  on 
a  point  some  ten  inches  distant.  If  this  deviation  is  much  greater  than 
that  found  for  distance  by  the  same  tests,  it  argues  some  abnormality 
of  tlie  converging  power,  either  in  the  way  of  excess  or  defect.  Such 
a  determination  is  of  great  importance  and  should  form  a  regular  part 
of  the  examination  for  muscular  anomalies.         ' 

(b)  We  may  determine  the  convergence  near-point,  or  the  nearest 
point  to  which  the  patient  can  converge  his  eyes  by  the  utmost  effort. 
This  should  be  not  more  than  two  inches  from  the  root  of  the  nose.  If 
more  than  this  it  argues  an  insufficiency  of  convergence. 

Even  when,  as  in  divergent  squint,  the  patient  has  no  absolute  con- 
vergence near-point,  since  he  cannot  produce  binocular  fixation  at  any 
distance,  far  or  near,  he  yet  often  makes  strong  converging  efforts, 
swinging  the  eyes  nearly  but  not  quite  to  meet  the  object  of  fixation 
as  it  is  brought  towards  him.  The  point  nearest  to  the  eyes  at  which  he 
gives  up  this  effort,  and  allows  the  eyes  to  diverge  widely,  is  called  his 
relative  convergence  near-point. 

(c)  We  may  determine  the  prism-convergence  (wrongly  called  the 
adduction).  This  is  measured  by  the  ability  to  overcome, — i.e.,  see 
single  with — prisms  held,  base  out,  before  the  eyes. 

The  amount  of  prism-convergence  that  a  patient  can  do  at  the  first 
examination  will  vary  from  10°  to  20°  or  more,  but  by  a  little  practice 


THE  EXTRA-OCULAR  MUSCLES  207 

most  people  can  overcome  prisms  of  50°,  60°,  or  more,  and  this  either 
wlien  looking  at  a  distant  object  or  when  looking  at  an  object  a  foot  away. 

3.  Tests  of  Divergence. — The  ability  of  the  eyes  to  diverge  is  meas- 
ured by  the  prism,  base  in,  that  they  can  overcome.  When  looking 
at  a  distant  object  most  eyes  can  overcome  from  4°  to  8°  of  prism, 
base  in,  this  constituting  the  prism-divergence  or  so-called  abduction 
for  distance. 

A  prism-divergence  of  less  than  4°  in  general  indicates  weak,  and 
of  over  8°  or  9°  an  excessive,  divergence  power. 

How  the  Tests  are  made  in  Practice. — In  examining  a  case  for  a  motor 
anomaly  of  the  eye  we  proceed,  as  follows: 

A.  We  first  determine  the  relations  for  distance  by  placing  the 
test-object  far  off, — if  possible,  twenty  feet  or  more, — from  the  eyes 
and  then  measuring  the  amount  of  deviation  by 

(1)  The  screen  and  (2)  the  parallax  simultaneously.  At  the 
same  time  we  determine  by  the  method  described  on 
page  21  whether  the  deviation  is  a  squint  or  a  hetero- 
phoria. 

(3)  The  Maddox  rod,  testing  both  for  hyperphoria  and   for 

lateral  deviation. , 

(4)  The  phorometer,  testing  both  for  hyperphoria  and  for  lateral 

deviation. 

B.  We  measure  the  deviation  for  near  points  (with  the  test-object 
held  at  about  ten  inches)  by 

(5)  The  phorometer;     (6)    the  screen  and    (7)    the  parallax 

simultaneously ;    (2)  the  Maddox  rod. 
In  this  case,  again,  we  ascertain  by  the  screen  test  whether 
the  deviation  is  a  squint  or  a  heterophoria. 

C.  We  determine : 

(9)   The  convergence  near-point. 

(10)  The  prism-divergence,  with  prisms  held,  base  in,  before  the 

eyes,  the  test  object  in  this  case  being  at  twenty  feet. 

(11)  The  prism-convergence  by  means  of  prisms  held,  base  out, 

before  the  eyes,  the  test  object  again  being  at  twenty 
feet. 

(12)  The  field  of  binocular  single  vision,  by  using  as  the  test 

object  a  candle  held  at  three  feet  from  the  eye.  In 
making  this  test* we  note  at  the  same  time  Avhether 
the  two  eyes  follow  the  candle  in  all  directions,  or 
w^hether  one  eye  lags  behind  the  other  in  performing 
some  particular  movement, — that  is,  we  determine 
(13)   The  field  of  binocular  fixation. 


208  THE    EYE    AND    NERVOUS    SYSTEM 

Other  tests  may  be  employed,  but  these  are  sufficient  for  all  practi- 
cal purposes.  Indeed,  in  all  ordinary  cases  we  may  omit  the  use 
of  the  phorometer,  and,  provided  the  convergence  near-point  is  nor- 
mal, we  may  also  dispense  with  the  determination  of  the  prism- 
convergence. 

What  the  Test  shows  in  Normal  Cases. — With  the  test  made  in  tho 
way  indicated  above,  the  eye-muscles  may  be  regarded  as  normal  if 

(a)  Tests  1  and  6  show  that  there  is  no  squint,  and  Tests  1  to  8 
show  that  there  is  only  a  minimal  amount  of  heterophoria  (less  than 
2°  of  esophoria  or  exophoria  *  and  less  than  ^°  of  hyperphoria)  for 
both  distance  and  near. 

(6)  The  convergence  near-point  is  less  than  two  inches  from  the  root 
of  the  nose. 

(c)  Prism-divergence  ia  not  less  than  4°  and  not  over  8°  (prism  = 
2°  to  4°  of  actual  deviation). 

(d)  Prism-convergence  after  a  few  trials  can  be  run  up  to  15°  or 
20°  or  more.f 

(e)  Tests  12  and  13  show  that  the  fields  of  binocular  fixation  and 
binocular  single  vision  are  normal, — i.e.,  the  patient  having  a  red  glass 
before  one  eye  and  following  a  light  that  is  carried  in  all  possible  direc- 
tions sees  everywhere  a  single  particolored  light,  and  each  eye  moves 
equally  with  its  fellow  without  lagging  any\vhere. 

Anomalies  of  Eye-Muscles  and  Eye  Movements ;  Etiological  Clas- 
sification of  Deviations. — Tlie  various  tests  just  outlined  enable  us  to 
make  a  diagnosis  of  the  ordinary  motor  anomalies  of  the  eye  commonly 
met  with.  In  considering  these  anomalies  we  must  distinguish  be- 
tween— 

A.  Deviations  due  to  anomalies  of  one  or  more  of  the  ocular 
muscles;  deviations,  that  is,  which  are  produced  by  some  affection 
of  the  muscles  themselves,  of  the  nerves  supplying  them,  or  of  the  nerve 
nuclei. 

B.  Deviations  due  to  derangement  of  one  of  the  co-ordinating  move- 
ments of  the  eyes.     The  muscles  themselves  in  this  case  and  their  cen- 

*  The  [Maddox  rod  often  shows  an  excess  of  esophoria  for  distance  ( 1-2"  more 
than  the  other  tests),  and  the  phorometer  sometimes  shows  2-3"  of  exophoria  for 
near  when  the  other  tests  prove  that  there  is  orthophoria. 

f  There  is  no  absolute  or  fixed  relation  between  the  amount  of  prism-divergence 
(abduction)  and  prism-convergence  (adduction).  The  former  amounting  to  from  5° 
to  8",  cannot,  as  a  rule,  be  altered  in  any  given  case  by  exercise.  Prism-convergence, 
on  the  other  hand,  may  range  anywhere  from  12°  to  30°  at  the  first  trial,  and,  how- 
ever low  at  the  outset,  can  almost  always  by  practice  be  brought  up  to  60°  or  70° 
or  even  more. 


THE  EXTEA-OCULAR  MUSCLES  209 

tral  connections  up  to  and  including  the  nuclei  are  intact,  but  the  supra- 
nuclear or  internuclear  nervous  mechanism  presiding  over  the  functions 
of  convergence,  divergence,  and  associated  parallel  movements  is  in 
some  way  out  of  gear. 

We  have,  therefore,  to  consider  in  succession — 

A.  Anomalies  of  the  ocular  muscles: 

(1)  Paralysis  of  the  ocular  muscles. 

(2)  Spasm  of  the  ocular  muscles. 

B.  Anomalies  of  the  co-ordinate  movements: 

(1)  Anomalies  of  convergence: 

Convergence-excess. 

Convergence-insufficiency  and  convergence-paralysis. 

(2)  Anomalies  of  divergence: 

Divergence-excess. 

Divergence-insufficiency  and  divergence-paralysis. 

(3)  Anomalies  of  parallel  movements: 

Conjugate  paralysis. 
Conjugate  spasm. 
Nystagmus. 

Section  II. 
PARALYSIS  OF  THE  INDIVIDUAL  MUSCLES. 

Symptoms. — A.  Diplopia. — All  the  subjective  symptoms  of  paralysis 
are  due  chiefly  to  the  diplopia  that  the  paralysis  causes.  Since  this 
diplopia  itself  is  due  to  the  fact  that  the  eye,  weakened  by  paralysis, 
fails  to  keep  pace  with  its  fellow,  and  since  this  diplopia  will  become 
most  apparent  as  soon  as  the  affected  muscle  is  called  into  action,  it  is 
evident  that  the  diplopia,  and  with  it  the  other  symptoms,  will  increase 
when  the  eyes  are  carried  into  the  field  of  action  of  the  paralyzed 
muscle.  For  example,  in  paralysis  of  the  right  abducens,  diplopia  will 
increase  fast  when  the  eyes  are  directed  to  the  right ;  whereas,  when  the 
eyes  are  directed  to  the  left,  the  diplopia  will  diminish  and  ultimately 
disappear,  because  in  that  direction  of  the  gaze  the  affected  muscle  takes 
no  part  in  moving  the  eye. 

The  characteristic  mark  of  paralysis,  therefore,  is  a  diplopia  which 
increases  progressively  and  fast  as  the  eyes  are  carried  in  some  definite 
direction  and  diminishes  when  the  eyes  are  carried  in  the  contrary  way. 

The  kind  of  diplopia  present  in  the  various  fonns  of  paralysis  will 
be  given  below  under  the  head  of  diagnosis. 

The  other  symptoms  beside  diplopia  which  are  found  in  paralysis 
are  as  follows : 
14 


210  THE    EYE    AND    NERVOUS    SYSTEM 

B.  False  Projection, — In  a  fresh  case  of  paralysis  of  the  right  ab- 
ducens,  the  patient  when  told  to  close  his  left  eye  and  quickly  put  his 
finger  on  an  object  situated  to  his  right,  will  in  general  shoot  too  far  to 
the  right  of  the  object ;  in  other  words,  the  object  appears  to  him  to  be 
further  to  the  right  than  it  really  is.  This  is  due  to  the  fact  that  in 
order  to  look  at  the  object  with  the  paretic  eye  he  has  to  exert  an  ex- 
cessive amount  of  nerve  energy,  such  as  corresponds  in  his  former 
experience  to  a  marked  movement  of  the  eye  to  the  right — that  is,  he 
has  to  work  hard  to  move  his  eye  a  little  and  he  thinks,  therefore,  that 
he  really  moved  it  a  great  deal. 

So,  too,  in  paralysis  of  a  left  rotator,  the  patient  will  project  the 
object  too  far  to  the  left ;  in  paralysis  of  an  elevator  he  will  project  it 
too  high  up,  and  in  paralysis  of  a  depressor  too  low  down. 

C.  Apparent  Movement  of  Objects  pseudokinesis) . — ^Yhen  a  patient 
with  paralysis  of  the  right  abducens  looks  with  his  paralyzed  eye  at  an 
object  situated  to  the  left,  the  latter  will  appear  in  its  proper  place ;  if, 
then,  he  suddenly  turns  his  head  to  the  left,  so  that  the  object  is  now 
somewhat  to  the  right,  it  will  no  longer  appear  in  its  proper  place,  but 
somewhat  to  the  right  of  it.  In  other  words,  it  will  seem  to  make  a  sud- 
den jump  from  left  to  right.  And,  in  general,  when  the  eye  is  carried 
in  a  direction  corresponding  to  the  field  of  action  of  a  paralyzed  muscle, 
objects  at  which  it  is  looking  will  suddenly  move,  the  direction  of  the 
movement  being  always  that  in  which  the  muscle  itself  would  naturally 
turn  the  eye.  That  is,  in  paralysis  of  a  right  rotator  objects  will  appear 
to  go  to  the  right,  in  paralysis  of  a  left  rotator  to  the  left,  in  paralysis  of 
an  elevator  they  will  rise  up  and  in  paralysis  of  a  depressor  they  will  go 
down. 

D.  Vertigo. — The  changing  diplopia  and  the  apparent  movement  of 
objects  seen  with  the  paralyzed  eye  produce  vertigo,  which  is  often  a 
very  marked  and  distressing  symptom  of  paralysis. 

E.  Attitude  of  Head. — All  the  symptoms  above  described  increase  in 
proportion  as  the  eyes  are  carried  in  the  special  direction  in  which  the 
muscle,  if  it  were  not  paralyzed,  would  move  the  eye.  To  avoid  them, 
the  patient  instinctively  turns  his  head  in  this  direction  so  that  his  eyes 
shall  be  turned  in  the  opposite  way.  Thus,  in  paralysis  of  a  right 
rotator  (right  externus,  left  internus),  he  will  turn  his  head  to  the  right, 
so  that  even  when  looking  at  objects  straight  in  front  of  him,  the  eyes 
themselves  will  Jdc  directed  to  the  left.  Similarly  in  paralysis  of  a  left 
rotator,  he  will  turn  his  head  to  the  left.  In  paralysis  of  an  elevator,  he 
may  throw  his  head  up  and  back,  and  in  paralysis  of  a  depressor,  he 
may  throw  it  forward  and  dowm.     In  paralysis  of  an  elevator  or  de- 


THE  EXTKA-OCULAR  MUSCLES  211 

pressor,  however,  the  easiest  and  most  usual  way  of  obviating  the 
diplopia  is  for  the  patient  to  tilt  the  head  towards  one  shoulder.  This 
depresses  the  corresponding  image,*  and  thus  enables  the  patient  to 
bring  the  images  on  a  level  in  case  there  is  vertical  diplopia.  The  head 
at  the  same  time  is  usually  turned  somewhat  to  right  or  left  (Fig.  13). 
This  constant  maintenance  of  the  head  in  a  constrained  position,  to 
obviate  the  diplopia  due  to  a  paralysis,  has,  in  some  instances,  produced 
torticollis.  (See  cases  cited  by  Posey,  Section  on  Ophthalmology, 
Eifty-third  Annual  Meeting  of  the  American  Medical  Association.)    * 


Fig.  13. — Showing  torticollis  from  vertical  deviation  of 
one  eye.    Case  of  Dr.  Posey 

F.  Other  Symptoms.- — In  paralysis  we  often  meet  with  other  symp- 
toms, such  as  headache,  various  forms  of  ana?sthesia,  paresthesia,  etc. 
The  headache  may  occasionally  be  a  reflex  symptom,  due  to  the  con- 
fusion and  strain  imposed  by  the  paralysis,  but  this  seems  to  be  very 
rarely  the  case.  Generally  speaking,  the  headache  and  other  symptoms 
are  the  result  of  the  cause  producing  the  paralysis  and  not  of  the 
paralysis  itself. 

Diagnosis  of  Paralysis. — In  diagnosticating  paralysis  we  apply 
the  various  tests  already  described : 

A.  Deviation  in  Primary  Position. — The  screen-test  and  the  different 
diplopia  tests  (parallax,  Maddox  rod,  and  phorometer)  show  the  pres- 

*  That    is,   if   he    tilts   the   head   to   the    right  shoulder,    the   right-hand   image, 
whether  belonging  to  the  right  or  to  the  left  eye,  is  carried  down. 


212  THE   EYE    AND    NERVOUS    SYSTEM 

ence  of  a  deviation  even  in  the  primary  position.  Thus,  in  paralysis  of 
the  abducens,  the  eyes  will  be  unduly  converged  so  that  all  the  tests 
will  show  a  pronounced  esophoria ;  in  paralysis  of  the  internus  there 
will  be  exophoria;  and  in  paralysis  of  the  elevators  or  depressors, 
hyperphoria. 

In  applying  the  screen-test,  we  will  generally  notice  that  the  devia- 
tion of  the  paralyzed  eye  behind  the  screen  {primary  deviation)  is  less 
than  that  of  the  sound  eye  {secondary  deviation).  The  reason  for  this 
is  as  follows:  Suppose  that  the  right  abducens  is  paralyzed  and  that 
this  eye,  in  consequence  when  screened,  deviates  inward  1  millimetre; 
when  the  screen  is  transferred  to  the  left  eye  the  right  will  have  to  turn 
out  1  millimetre  in  order  to  fix.  To  do  this,  the  eye  must  employ  its 
paralyzed  externus  and,  as  this  is  an  inefficient  instrument,  must  exert  a 
great  deal  of  nerve  power.  This  same  nervous  force  is  necessarily  con- 
veyed to  the  left  eye  at  the  same  time,  impelling  it  to  turn  very  strongly 
to  the  right.  As  the  muscles  of  the  left  eye  are  intact,  it  is  able  to  obey 
this  impulse  fully,  and  hence  moves  to  the  right,  not  one  but  several 
millimetres. 

The  difference  between  the  primary  and  the  secondary  deviation  is 
best  measured  with  prisms.  If,  for  example,  a  prism  of  10°,  before  the 
right  eye  will  exactly  abrogate  the  screen  deviation,  but  it  takes  a  prism 
of  20°  before  the  left  eye  to  do  the  same  thing,  we  know  that  the  sec- 
ondary deviation  of  the  left  eye  is  about  twice  as  great  as  the  primary 
deviation  of  the  right. 

B.  Limitation  of  Movement. — The  tests  for  the  monocular  field  of 
fixation  made  with  the  tropometer  or  perimeter  show  the  limitation 
of  movement  of  the  affected  eye  in  some  particular  direction  of  the  gaze. 
This  test,  however,  is  often  unsatisfactory  and  is  better  replaced  by  the 
following  tests  for  determining  the  field  of  binocular  fixation  and  bin- 
ocular single  vision. 

C.  Lagging  of  One  Eye  behind  the  Other. — The  field  of  binocular  fixa- 
tion is  determined  in  the  Way  already  described  (page  205).  In  a 
case  of  paralysis  we  find  that  as  the  two  eyes  follow  a  moving  object,  one 
lags  markedly  behind  the  other  as  soon  as  the  eye  is  carried  in  the  direc- 
tion of  the  field  of  action  of  the  paralyzed  muscle.  Thus,  in  paralysis 
of  the  right  abducens  the  right  eye  will  begin  to  falter  and  fall  behind 
as  soon  as  the  eyes  are  turned  to  the  right ;  in  paralysis  of  an  elevator, 
the  affected  eye  will  fail  to  rise  with  its  fellow,  and  in  paralysis  of  a 
depressor,  it  will  not  go  down  as  well. 

By  applying  the  screen-test  in  the  different  directions  of  the  gaze, 
it  may  be  possible  to  show  that  there  is  binocular  fixation  when  the  eyes 


THE  EXTRA-OCULAR  MUSCLES  213 

are  turned  in  one  way,  say  the  right,  and  that  there  is  a  marked  devia- 
tion from  binocular  fixation  when  the  eyes  are  turned  in  the  opposite 
way,  to  the  left.    This  will  be  evidence  of  the  paresis  of  a  left  rotator. 

D.  Diplopia. — By  far  the  most  satisfactory  evidence  of  paralysis  is 
that  afforded  by  the  determination  of  the  field  of  hinocular  single  vision, 
— i.e.,  by  ascertaining  the  amount  and  kind  of  diplopia  present  in  the 
different  directions  of  the  gaze. 

The  characteristic  diplopia  found  in  paralysis  of  each  muscle  is  as 
follows : 

In  paralysis  of  the  right  abducens  there  is  homonymous  diplopia, 
increasing  fast  to  the  right.  In  paralysis  of  the  left  abducens  there 
is  homonymous  diplopia  which  increases  fast  to  the  left.  In  both  cases 
the  diplopia  will  be  slightly  less  when  the  eyes  are  directed  up  and 
slightly  greater  when  the  eyes  are  directed  down. 

In  paralysis  of  the  right  internal  rectus  there  will  be  crossed  di- 
plopia increasing  fast  to  the  left.  In  paralysis  of  the  left  intemus  there 
will  be  crossed  diplopia  increasing  fast  to  the  right.  In  both  cases  the 
crossed  diplopia  will  increase  somewhat  when  the  eyes  are  turned  up 
and  diminish  somewhat  when  they  are  turned  down. 

In  paralysis  of  the  right  superior  rectus  there  will  be  vertical  di- 
plopia, with  the  image  of  the  right  eye  higher  (left  diplopia).  This  wall 
increase  as  the  eyes  are  carried  up,  and  particularly  as  they  are  carried 
up  and  to  the  right.  In  general,  but  not  invariably,  there  will  be  also  a 
moderate  crossed  diplopia,  w^hich  will  increase  in  looking  to  the  left. 
The  image  of  the  right  eye  will  be  tilted  to  the  left. 

In  paralysis  of  the  left  superior  rectus  there  will  be  a  vertical  di- 
plopia (right  diplopia),  increasing  up  and  to  the  left,  and  a  crossed 
diplopia,  increasing  up  and  to  the  right.  The  image  of  the  left  eye 
will  be  tilted  to  the  right. 

In  paralysis  of  the  right  inferior  rectus  there  will  be  a  vertical 
(right)  diplopia,  increasing  as  the  eyes  are  carried  down  and  to  the 
right,  and  usually,  but  not  always,  a  crossed  diplopia,  which  will  increase 
in  looking  down  and  to  the  left.  The  image  of  the  right  eye  will  be 
tilted  to  the  right. 

In  paralysis  of  the  left  inferior  rectus  there  will  be  a  vertical  (left) 
diplopia,  increasing  down  and  to  the  left,  while  the  crossed  diplopia,  if 
present,  will  increase  in  looking  down  and  to  the  right.  The  image  of 
the  left  eye  will  be  tilted  to  the  left. 

In  paralysis  of  the  right  superior  oblique  there  will  be  a  vertical 
(right)  diplopia,  which  will  increase  as  the  eyes  are  directed  down  and  to 
the  left,  and,  usually,  also  an  homon^TUOus  diplopia,  which  will  increase 


214  THE    EYE    AI^D    NERVOUS    SYSTEM 

down  and  to  the  right.  The  image  of  the  right  eye  will  be  tilted  to 
the  left. 

In  paralysis  of  the  left  superior  oblique  there  will  be  a  left  diplopia, 
increasing  down  and  to  the  right  and  an  homonymous  diplopia,  in- 
creasing down  and  to  the  left.  The  image  of  the  left  eye  will  be  tilted 
to  the  right. 

In  paralysis  of  the  right  inferior  oblique  there  will  be  a  left  di- 
plopia, which  will  increase  as  the  eyes  are  carried  up  and  to  the  left, 
and  sometimes,  but  not  always,  an  homonymous  diplopia,  which  will 
increase  as  the  eyes  are  carried  up  and  to  the  right.  The  image  of  the 
right  eye  will  be  .tilted  to  the  right. 

In  paralysis  of  the  left  inferior  oblique  there  will  be  a  right  di- 
plopia, increasing  up  and  to  the  right,  and  an  homonymous  diplopia, 
increasing  up  and  to  the  left.  The  image  of  the  left  eyewiU  be  tilted 
to  the  left. 

We  meet  with  various  combined  paralyses  producing  more  or  less 
complicated  forms  of  diplopia.  Chief  among  these  is  the  oculo-motor 
paralysis,  or  paralysis  of  all  the  muscles  supplied  by  the  third  nerve. 
In  this  case  the  eye  can  be  moved  outward  by  the  abducens,  but  it  can- 
not be  moved  in  any  other  way,  for,  although  the  superior  oblique  is 
still  acting,  it  cannot  move  the  eye  downward  since  it  is  not  efficient  as 
a  depressor  when  the  eye  is  in  a  position  of  abduction,  as  it  is  in  paral- 
ysis of  this  sort.  The  most  that  the  superior  oblique  can  do  under  these 
conditions  is  to  give  the  eye  a  slight  inward  rotation  upon  its  antero- 
posterior axis  when  the  patient  attempts  to  turn  the  eye  down. 

In  a  right  oculo-motor  paralysis  there  will  be  a  crossed  diplopia,  due 
to  the  relative  divergence  of  the  paralyzed  eye,  and  usually  also  a  slight 
left  diplopia,  because  the  eye  sags  somewhat  below  the  level  of  the 
other.  This  left  diplopia  will  increase  fast  as  the  eyes  are  carried  up. 
On  the  other  hand,  when  the  eyes  are  carried  down,  the  left  diplopia 
will  be  transformed  into  a  vertical  diplopia  of  the  opposite  kind  (right), 
which  will  increase  fast  as  the  eyes  are  depressed. 

The  diagnosis  of  a  paralysis  from  the  behavior  of  the  double  images 
will  be  facilitated  by  the  following  table : 

TABLE  SHOWING  FROM  DIPLOPIA  WHICH  MUSCLE  IS  PARALYZED. 

DH=homonymous  diplopia. 
DX=cro88ed  diplopia. 

DR=right  diplopia  (vertical  diplopia  with  image  of  right  eye  below). 
DL=:left  diplopia  (vertical  diplopia  with  image  of  left  eye  below). 
Er,  El,  Eu,  Ed,  Eu  and  r,  etc.=respectively  "  when  both  eyes  are  directed  to  the 
"right"  "to  the  left,"  "up,"  " dovm,"  " up  and  to  the  right,"  etc. 
»=increasing  fast  and  progressively. 


THE  EXTKA-OCULAR  MUSCLES  215 

A.  The  diplopia  is  lateral  (DX  or  DH)  »  in  Er  or  El.     (=  paralysis  of  a  lateral 

rotator.) 

Diplopia  »  in  Er.     (^paralysis  of        Diplopia  »  in  El.     (=paralysis  of  a 

a  RIGHT  ROTATOR.  )  LEFT  ROTATOR.  ) 

DH  »  in  Er.     R.  extemus.  DH  »  in  El.     L.  extemus. 

DX  »  in  Er.     L.  intemus.  DX  »  in  El.     R.  intemus. 

B.  The  diplopia  is  vertical  (DR  or  DL)  »  in  Eu.     (=paralysis  of  an  elevator.) 

Diplopia   »   in   Er    (Eu   and   r).        Diplopia  »  in  El  (Eu  and  1).     (=pa- 
(=:paraly8is  of  a  right-hand  ele-  ralysia  of  a  left-hand  elevator). 

VATOR.  ) 

DL  »  in  Eu  and  r.  R.  superior        DR    »  in   Eu   and    1.     L.      superior 

rectus.  rectus. 

DR  »  in   Eu  and  r.  L.  inferior        DL  »  in  Eu  and  1.     R.  inferior  ob- 

oblique.  lique. 

C.  The  diplopia  is  vertical  (DR  or  DL)  »  in  Ed.     (=paralysis  of  a  depressor.) 

Diplopia  »    in   Er    (Ed   and   r).  Diplopia  »  in  El  (Ed  and  1).     (=pa- 

(=paralysis  of  a  right-hand  de-  ralysis  of  a  left-hand  depressor.) 

pressor.  ) 

DR  »  in  Ed  and  r.     R.  inferior  DL  »  in  Ed  and  1.      L.  inferior  rectus. 

rectus. 

DL  »  in  Ed  and  r.     L.  superior  DR  »  in  Ed  and  1.     R.  superior  ob- 

oblique.  lique. 

A  good  rule  to  enable  us  to  determine  which  is  the  paralyzed  eye  is 
the  following: 

When,  in  moving  the  eyes  in  a  given  direction,  the  double  images 
begin  to  separate,  that  image  which  moves  the  faster  and  the  further 
belongs  to  the  paralyzed  eye. 

And  a  rule  to  determine  the  kind  of  double  vision  produced  by  a 
given  paralysis  is, — the  image  belonging  to  the  paralyzed  eye  is  dis- 
placed in  just  that  direction  in  which  the  affected  muscle,  if  intact, 
would  naturally  move  the  eye.  Thus,  in  paralysis  of  the  right  superior 
rectus,  which  moves  the  right  eye  up  and  to  the  left  and  tilts  the  verti- 
cal meridian  to  the  left,  the  image  of  the  right  eye  is  up  (higher),  de- 
flected to  the  left  (crossed  diplopia),  and  tilted  to  the  left. 

The  double  images  may  be  conveniently  plotted  on  a  diagram,  as 
shown  in  Fig.  14. 

Fixation  with  the  Paralyzed  Eye. — A  marked  change  in  the  ap- 
pearances presented,  although  not  in  the  actual  conditions  present,  is 
produced  by  fixation  with  the  paralyzed  eye.  The  paretic  eye,  in  this 
case,  is  forced  into  position,  and  the  sound  eye  deviates.  !N^ow  the  force 
applied  to  the  sound  eye  to  make  it  deviate  is  equal  to  the  force  applied 
to  the  paretic  eye  to  make  it  get  into  position.  This  latter  force,  how- 
ever, must  be  very  considerable,  and  the  more  so  the  greater  the  degree 


216 


THE    EYE    AND    NERVOUS    SYSTEM 


of  paresis.  Hence,  as  we  have  seen  (p.  212),  the  deviation  of  the  sound 
eye  when  the  paretic  eye  fixes  (secondary  deviation)  is  much  greater 
than  the  deviation  of  the  paretic  eye  when  the  sound  eye  fixes  (primary 


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Fig.  14.— Diagram  for  plotting  Double  Images  in  Paralysis.— The  diagram  is  made  on  the 
assumption  that  the  field  of  binocular  single  vision  is  taken  at  a  distance  of  thirty  inches  and  pro- 
jected on  a  tangent  plane  at  that  distance  (represented  by  the  large  square).  The  fine  cross  lines 
denote  intervals  of  2  inches ;  the  heavier  cross  lines,  intervals  of  one  foot.  The  concentric  circles 
denote  intervals  of  5°  plotted  on  the  tangent  plane.  The  diagram  Is  used  as  follows :  The  light  is 
held  thirty  inches  from  the  patient  and  carried  into  the  six  cardinal  positions  "Eyes  right"  (Er), 
"Eyes  up  and  right"  (Eu  and  r),  etc.,  and  the  amount  of  diplopia,  vertical  and  lateral,  in  inches  is 
laid  off  on  the  diagram.  In  the  case  shown  (one  of  paralysis  of  the  left  sujierior  rectus),  in  which  the 
image  of  the  right  eye  is  denoted  by  a  circle  and  that  of  the  left  by  a  cross,  the  vertical  distance  be- 
tween the  two  images  when  the  eyes  are  directed  two  feet  up  and  two  feet  to  the  left  is  six  inches 
and  the  lateral  distance  is  four  inches.  The  diagram  shows  that  this  means  that  when  the  eyes  are 
elevated  3<s°  and  also  carried  38°  to  the  left,  the  vertical  diplopia  is  over  5°  and  the  lateral  diplopia 
about  3°  of  arc.  When  the  eyes  are  directed  the  same  distance  up  and  to  the  right  there  is  simple 
crossed  diplopia  of  5°,  and  if  directed  two  feet  straight  to  the  left  a  simple  vertical  diplopia  of  2.5°. 
Below  the  oblique  line  AB  (that  is  in  the  positions  Er,  Ed  and  r,  and  Ed  and  1)  there  is  single  vision. 
The  diagram  can  be  used  for  any  other  distance  than  thirty  inches,  provided  the  proper  allowance  is 
made.  Thus  if  the  light  were  held  five  feet  from  the  patient,  the  fine  cross  lines  would  represent 
intervals  of  four  inches,  and  the  heavy  cross  lines  intervals  of  two  feet. 


deviation).  Accordingly,  the  effect  of  fixation  with  the  paralyzed  eye 
is  to  increase  the  deviation  sometimes  to  double  or  treble  of  its  original 
amount.     The  result  is  that  the  double  images  are  put  much  further 


THE  EXTEA-OCULAR  MUSCLES  217 

apart,  and  hence  interfere  less  with  each  other,  cause  less  confusion,  and 
are  more  readily  ignored.  This,  in  fact,  as  Graefe  says,  is  evidently  the 
reason  why  the  patient  adopts  this  manoeuvre  at  all. 

This  secondary  deviation  of  the  sound  eye,  when  the  paretic  eye 
fixes,  always  appears  under  the  guise  of  a  spasm  of  the  associate  (page 
17)  of  the  paralyzed  muscle.  Thus,  if  the  right  superior  rectus  is 
paralyzed,  and  yet  this  eye  is  used  for  fixation,  the  appearances  pro- 
duced will  be  those  of  a  spasm  of  the  left  inferior  oblique.  For,  in  this 
case  the  right  eye  tends  all  the  time  to  drop  and  can  be  kept  in  place 
only  by  an  excessive  nervous  effort.  This  effort  will  be  very  great  when 
the  eyes  are  directed  to  the  right,  because  in  this  position  its  paretic 
superior  rectus  is  the  only  muscle  that  lifts  the  right  eye.  On  the  other 
hand,  when  the  eyes  are  directed  to  the  left,  the  right  superior  rectus 
acts  very  little  as  an  elevator,  and,  as  the  muscle  (the  inferior  oblique) 
which  here  does  the  main  part  of  the  work  is  normal,  but  little  extra 
effort  will  be  required  to  keep  the  eye  in  place.  Now  the  extra  effort 
that  is  put  forth  to  keep  the  right  eye  in  place  is  exerted  equally  upon 
the  left  eye.  Hence,  when  looking  to  the  right  the  left  eye  will  rise 
unduly  (shoot  obliquely  up),  while  in  looking  to  the  left,  it  will  rise 
little  or  not  at  all  above  the  proper  level.  This  is  just  the  effect 
that  would  be  produced  by  a  spasm  of  the  right-hand  elevator 
(inferior  oblique)  of  the  left  eye.  Similarly,  as  shown  in  the  table 
on  page  246,  a  paralysis  of  the  right  superior  oblique  will,  if  the  right 
eye  is  fixing,  give  the  appearance  of  a  spasm  of  the  left  inferior 
rectus,  etc. 

Fixation  with  the  paralyzed  eye  is  not  infrequent.  It  is  especially 
apt  to  occur  when  the  paretic  eye  has  the  better  vision,  although  it  may 
also  occur  when  this  is  the  poorer  eye  of  the  two.  It  seems  to  be  com- 
paratively frequent  in  paralysis  of  the  superior  rectus,  and  not  very 
frequent  in  abducens  paralysis. 

Sometimes  the  patient  fixes  alternately  with  the  paretic  and  the 
sound  eye.  Thus,  in  one  case  that  I  saw,  a  boy  with  a  paresis  of  the 
left  superior  rectus,  either  fixed  with  the  right  eye  so  as  to  have  a  left 
hypotropia,  or  with  the  left  eye  so  as  to  have  a  right  hypertropia  (alter- 
nating vertical  squint).  In  the  same  way  an  alternating  convergent 
squint  may  be  produced  by  alternate  fixation  in  a  case  of  paralysis  of 
one  abducens. 

Varieties  of  Paralysis. — Isolated  and  Combined  Paralyses. — Paralysis 
may  affect  one  or  several  ocular  muscles. 

Isolated  paralysis  of  the  abducens  is  very  common  and  such  paraly- 
sis is  occasionally  bilateral. 


218  THE    EYE    AND    NERVOUS    SYSTEM 

Oculomotor  paralysis,  complete  or  partial,  is  the  next  most  frequent 
form,  and  all  sorts  of  combinations  occur.  In  complete  oculomotor 
paralysis,  not  only  are  the  motions  of  the  eye  affected,  as  stated  (page 
39),  but  there  are  also  ptosis  (or  drooping  of  the  lid  from  paral- 
ysis of  the  levator),  paretic  mydriasis,  and  paralysis  of  accommodation. 
Frequently  the  accommodation  and  the  iris  alone  are  paralyzed  {oph- 
thalmoplegia interior  or  interna)  (see  page  312)  ;  in  other  cases  just 
these  muscles  are  exempt.  In  other  cases  still,  ptosis  alone  is  present 
and  is  often  bilateral.  In  ptosis  not  only  is  there  complete  or  partial 
drooping  of  the  lid,  but  there  is  also  considerable  wrinkling  of  the 
brow,  because  the  patient  endeavors  by  compensatory  contraction  of  the 
frontalis  to  make  up  for  the  deficient  action  of  the  levator. 

The  next  most  frequent  form  of  paralysis  in  the  author's  experience 
(differing  therein  from  that  of  other  observers)  is  isolated  paralysis  of 
the  superior  rectus.    It  also  is  sometimes  bilateral. 

Next  in  order  comes  paralysis  of  the  superior  oblique  (trochlear 
paralysis).  This  is  fairly  common,  but  it  is  very  rarely  indeed  bi- 
lateral, only  two  or  three  such  cases  being  recorded,  one  of  which 
occurred  in  the  author's  own  experience. 

Isolated  paralysis  of  the  inferior  rectus  is  fairly  frequent,  occurring 
in  the  author's  experience  more  often  than  that  of  the  internal  rectus. 

Isolated  paralysis  of  the  inferior  oblique  is  very  uncommon.  Some 
thirty  cases  have  been  recorded,  but  less  than  half  of  these  are  authen- 
tic. Yet  it  is  probably  more  frequent  than  has  been  supposed,  since  the 
author  has  seen  no  less  than  four  cases,  all  incomplete.  One  of  these 
was  congenital.  In  a  single  instance,  reported  by  Bielschowsky,  the 
paralysis  was  bilateral. 

In  some  cases  there  is  total  paralysis  of  all  the  muscles  moving  the 
eyeball  and  of  the  interior  muscles  as  well  (total  ophthalmoplegia). 
The  eyeball  is  then  perfectly  motionless,  the  pupil  widely  dilated  and 
the  accommodation  entirely  absent.  More  or  less  complete  paralysis  of 
all  the  exterior  muscles  with  exemption  of  the  sphincter  and  ciliary 
muscles,  is  called  ophthalmoplegia  exterior  or  externa. 

Etiology. — Etiologically,  we  have  to  distinguish  three  varieties  of 
paralysis, — structural,  insertional,  and  innervational. 

Structural  paralysis  is  due  to  some  abnormality  of  the  muscle  itself. 
Such  abnormality  may  be  the  result  of  congenital  defect,  solution  of  con- 
tinuity, inflammation  of  the  muscle  or  its  surroundings,  atrophy,  etc. 
(For  fuller  details  see  section  on  Orbital  Paralysis.) 

Insertional  paralysis  is  the  form  in  which  the  muscle  and  its  supply- 
ing nerve  are  normal,  but  the  muscle  is  improperly  inserted,  either  as 


THE  EXTRA-OCULAR  MUSCLES  219 

the  result  of  congenital  misplacement,  accident,  or  operation.  (See 
for  fuller  details  Orbital  Paralysis.) 

Innervational  paralysis  is  the  form  in  which  the  muscle  and  tendon 
are  normal,  but  there  is  some  abnormality  of  the  nerve  or  its  central 
connections.  Such  abnormality  may  consist  in  (a)  congenital  defi- 
ciency; (&)  solution  of  continuity;  (c)  anjcmia  or  hyperaemia,  due 
either  to  general  disturbance  of  the  cerebral  circulation,  or  to  pressure 
upon  the  vessels  supplying  the  nerve  or  its  nucleus;  {d)  atrophy  of  the 
nerve  or  its  nucleus,  due  to  pressure  of  exudates,  inflammatory  swell- 
ings, callus,  liemorrhages,  aneurismal,  atheromatous  or  thrombotic 
vessels,  or  neoplasms;  (e)  softening  or  degeneration  of  the  nerve  or  its 
nucleus. 

With  regard  to  site  we  divide  paralysis  into  extracranial  and  intra- 
cranial. 

Extracranial  (Orbital  or  Peripheral)  Paralysis. — In  a  certain  number 
of  cases  orbital  paralysis  is  congenital.  (For  varieties  see  below  under 
Exciting  Cause,  Congenital  Conditions.) 

Orbital  paralysis  (or  insufficiency)  may  be  due  to  developmental 
changes  in  the  muscles  or  nerves.  One  variety  of  this  is  the  relative 
insufficiency  of  the  interni  and  preponderance  of  the  externi,  caused 
by  the  gradual  separation  of  the  orbits,  which  takes  place  in  the 
development  of  the  skull  in  childhood.  This  separation,  as  Weiss 
showed,  by  giving  the  externi  a  larger  and  the  interni  a  smaller  arc 
of  contact  with  the  eyeball,  tends  to  produce  a  relative  divergence  of 
the  eyes. 

Some  orbital  paralyses  are  due  to  traumatism,  either  from  the 
direct  effect  of  the  injury  upon  the  muscle  or  tendon,  or  the  remote 
effects  produced  by  the  pressure  of  effused  blood  and  callus  in  the  case 
of  fractures,  or  to  cicatricial  contraction  in  the  tissues  of  the  orbit. 
(See  for  further  particulars  Exciting  Causes,  Traumatism.) 

Orbital  paralysis  may,  very  rarely,  be  due  to  disease  of  the  muscles 
themselves,  as  to  myositis,  simple  (Gleason)  or  syphilitic  (Hochheim)  ; 
to  hyaline  degeneration  (Xuel)  ;  to  degenerative  changes  occurring  in 
progressive  muscular  dystrophy  (Groenouw,  Uhthoff)  and  in  acute 
ascending  paralysis  (Goebel)  ;  and  to  metastatic  tumors  (six  cases  cited 
by  Groenouw  and  Bernheimer). 

In  other  cases  it  is  produced  by  disease  of  the  nerves,  especially  a 
primary  tabic  degeneration  (Dejerine,  Marina)  ;  very  rarely  by  second- 
ary new  growths  occurring  in  the  nerves  (Bernheimer). 

A  more  usual  cause  of  orbital  paralysis  is  the  presence  of  tumors 
(especially  gummata,  cavernous  grow^ths,  exostoses,  and  sarcomata)  or 


220  THE    EYE    AND    NERVOUS    SYSTEM 

inflammatory  processes  (orbital  cellulitis,  tenonitis,  periostitis,  muco- 
cele) of  the  contents,  walls,  and  accessory  cavities  of  the  orbit.  These 
processes  may  act  by  causing  secondary  changes  (inflammation,  de- 
generation, or  atrophy)  in  the  muscles  and  nerves,  in  which  case  the 
picture  presented  will  be  that  of  a  true  paralysis  of  some  muscle  or 
group  of  muscles.  They  often,  however,  act  in  a  purely  mechanical 
way  by  pressing  the  eyeball  forward  or  by  enveloping  it  and  its  muscles 
in  rigid  masses  preventing  movement.  In  this  case  the  deviation  of  the 
eye,  the  restriction  of  movement,  and  the  diplopia  produced  may  differ 
altogether  from  what  we  find  in  any  of  the  types  of  paralysis  we  have 
described. 

A  similar  mechanical  restriction  of  eye  movements  with  more  or 
less  aberrant  diplopia  may  be  caused  by  a  pterygium  or  pseudo- 
pterygium. 

Intracranial  Paralysis  comprises  basal,  nuclear,  and  cortical  paralysis. 

Basal  (and  Foraniinal)  Paralysis. — By  hasal  paralysis  we  understand 
a  paralysis  due  to  lesion  of  the  nerve-trunk  after  its  emergence  from 
the  brain  and  before  its  exit  from  the  skull ;  by  a  foraminal  paralysis, 
one  due  to  lesion  of  the  nerve  as  it  passes  through  the  sphenoidal 
fissure.  The  nerves  in  traversing  this  fissure  pass  through  veritable 
foramina,  whose  walls  are  made  up  of  rigid  fascia.  This  arrangement, 
while  protecting  them  from  injury,  renders  them  more  liable  to  com- 
pression and  strangulation. 

Basal  and  foraminal  paralyses  are  due  to — 

(1)  Arrested  or  imperfect  development  of  the  nerve-trunks  (con- 
genital paralysis).     Very  rare. 

(2)  Solution  of  continuity  of  the  nerve  from  blows  on  the  head 
causing  fractures  of  the  base,  or  from  shot  or  stab  wounds  of  the  mouth 
or  orbit. 

-  (3)  Hemorrhage  into  the  nerves  from  traumatism,  or  from  the 
spontaneous  rupture  of  aneurismal  or  sclerotic  vessels. 

(4)  Inflammation  or  degeneration  of  the  nerves  from  syphilis  or 
other  causes. 

(5)  Tumors  of  the  nerves. 

(6)  Pressure  on  the  nerves  from  (a)  hemorrhage;  (6)  inflamma- 
tory exudate  (meningitis,  periostitis)  including  callus;  (c)  hard  or 
distended  vessels;  {d)  tumors  (especially  gummata)  ;  and  (e)  general 
distention  of  the  brain. 

Nuclear  and  Fascicular  Paralyses. — By  nuclear  paralysis  we  under- 
stand a  paralysis  due  to  lesion  of  the  nerve-nucleus  itself;  by  fascicular 
paralysis,  one  due  to  lesion  of  the  root-fibres  which  emanate  from  the 


THE  EXTRA-OCULAR  MUSCLES  221 

nucleus  and  which,  before  leaving  the  brain,  coalesce  to  form  the  nerve- 
trunk. 

Xuclear  and  fascicular  paralyses  are  due  to — 

(1)  Aplasia  of  the  nucleus  {congenital  paralysis).  Very  rare.  (It 
must  be  noted  that,  as  Wilbrand  and  Saenger  point  out,  some  cases  of 
congenital  nuclear  paralysis  are  due  not  to  aplasia  but  to  degeneration 
and  atrophy  of  the  nucleus  from  fetal  or  infantile  disease.  The  dis- 
tinction between  the  two  would  be  that  in  aplasia  there  would  not  be, 
and  in  acquired  atrophy  there  would  be,  a  secondary  denegeration  of 
the  nerve-trunk  and  muscles).     (Compare  Chapter  XIII,  Sect.  II.) 

(2)  Atrophy  and  degeneration  of  the  nucleus  and  root-fibres  from 
syphilis,  tabes,  multiple  sclerosis,  and  rarely  from  other  cerebro-spinal 
diseases ;  also  from  poisons  and  the  toxins  of  diseases  like  diphtheria, 
influenza,  etc.  (Compare  Chapters  XII  and  XV). 

(3)  AncBmia,  hypercemia,  and  hemorrhage,  due  usually  to  vascular 
changes  (arteriosclerosis,  endarteritis,  miliary  aneurisms),  which  are 
themselves  generally  the  results  of  syphilis  or  the  action  of  poisons 
(lead,  diphtheria,  influenza,  etc.).  A  peculiar  form  of  hemorrhagic 
disease  of  the  nuclei  and  root-fibres  is  polioencephalitis  superior  (see 
below  under  Exciting  Causes).  Xuclear  hemorrhages  may  also  be  due 
to  traumatism. 

(4)  Tumors,  especially  gumma,  more  rarely  glioma,  sarcoma,  and 
tuberculoma  (Compare  Chapter  IX). 

It  must  be  noted  that  owing  to  the  connection  between  the  abducens 
nucleus  of  one  side  and  the  opposite  oculo-motor  nucleus,  a  lesion 
(tumor,  etc.)  of  the  pons  involving,  say,  the  right  abducens  nucleus,  does 
not  cause  simple  paralysis  of  the  right  externus,  but  a  conjugate  paraly- 
sis (paralysis  of  dextroversion  in  both  eyes).  To  produce  simple  pa- 
ralysis of  the  abducens,  the  lesion  must  be  so  situated  as  to  cut  off  the 
emergent  root-fibres  of  the  nerve ;  in  other  words,  it  must  be  a  fascicular 
paralysis. 

Supranuclear  and  Cortical  Paralyses. — Lesions  affecting  the  connections 
of  the  nuclei  with  the  cortex  and  the  cortex  itself  do  not  in  general 
cause  paralysis  of  the  individual  ocular  muscles,  but  paralysis  of  the 
co-ordinated  movements  of  the  eye, — i.e.,  of  convergence,  divergence, 
or  the  parallel  movements.  An  exception  seems  to  be  ptosis,  which  in 
some  twenty-five  reported  cases  (Willbrand  and  Saenger)  has  been 
caused  apparently  by  a  cortical  lesion  and  particularly  one  situated  in 
the  supramarginal  and  angular  gyri  (usually  on  the  side  opposite  to 
the  ptosis).  Doubt,  however,  has  been  thrown  on  these  cases,  first, 
because  often  marked  lesion  of  these  parts  may  exist  without  causing 


222  THE    EYE    AXD    NERVOUS    SYSTEM 

ptosis;  and,  second,  because  even  when  ptosis  is  present  we  cannot 
always  be  sure  that  is  not  due  to  a  remote  effect  (nuclear  or  basal 
lesion)  produced  bv  the  cortical  disease  (Compare  Chapter  IX). 

Exciting  Causes  of  Paralysis. — The  causes  producing  the  various 
lesions  enumerated  above  as  present  in  the  different  forms  of  paralysis, 
orbital,  basal,  and  nuclear,  are  as  follows: 

1.  Congenital  Conditions. — Congenital  paralysis  in  most  cases  prob- 
ably is  due  to  non-development  or  faulty  development  of  the  ocular 
muscles  themselves  (orbital  paralysis).  The  congenital  malformations 
of  the  muscles  causing  paralysis  are  as  follows : 

(a)  Absence  of  the  muscle, — e.g.,  to  absence  of  the  internus 
(Krause,  Lawford),  the  externus  (Tvrause,  Bahr),  the  superior  rectus 
(Seiler,  Steinheim),  both  inferior  recti  (Stieren,  A.  E.  Davis),  and  one 
or  both  obliques  (Ilartes,  Seiler). 

(6)  Replacement  of  muscle  by  a  more  or  less  inelastic  cord  of  con- 
nective tissue.  This  has  been  observed  in  the  case  of  the  internus 
(Uhthoff,  Guende),  and  the  externus  (Baumgarten,  Inouye,  Ailing, 
Axenfeld  and  Schiirenberg,  Evans,  Bernheimer). 

(c)  Underdevelopment  of  the  muscle.  This  has  been  observed  by 
Lawford,  Bach,  and  others.  A  moderate  degree  of  underdevelopment  is 
considered  by  Schneller  to  be  a  frequent  cause  of  squint  (structural 
squint).  This  view  he  bases  on  the  findings  in  numerous  squint  opera- 
tions. It  is  questionable,  however,  whether  the  underdevelopment  that 
he  found  was  not  rather  the  result  than  the  cause  of  the  squint. 

(d)  Abnormal  course  and  insertion  of  the  muscle.  Among  the 
abnormal  conditions  found  are:  (1)  Insertion  of  the  muscle  too  far 
back,  causing  it  to  act  as  a  retractor  rather  than  a  rotator  of  the  eye 
(Ileuck)  ;  (2)  bifurcation  of  the  tendon  (Dieffenbach,  Bahr*),  and 
(3)  the  presence  of  adhesions  or  bands  connecting  the  muscles  with 
each  other  or  with  the  orbital  walls  (Morgagni,  Olbers  and  Wrisberg, 
Fitzgerald). 

!Much  more  rarely  congenital  paralysis  is  due  to  aplasia  of  the  nerves 
(either  in  the  orbit  or  the  cranial  cavity)  or  of  their  nuclei. 

Some  cases  of  congenital  paralysis  are  traumatic,  being  produced 
either  by  direct  pressure  or  by  hemorrhage  during  delivery  with  the 
forceps.  Such  paralysis  may  affect  the  external  rectus  with  or  with- 
out involvement  of  the  facial  nerve,  or  the  oculo-motor  nerve,  or  the 
superior  rectus  with  the  levator  (Groenouw,  iSTettleship). 

Other  rare  cases  of  congenital  paralysis  are  apparently  due  to  fetal 

•  In  Bahr's  case  one  of  the  slips  was  inserted  too  far  back,  causing  the  muscle 
to  act  partly  as  retractor. 


THE  EXTRA-OCULAR  MUSCLES  223 

inflammation    or   degeneration   of    the    nerve    nuclei    (Wilbrand    and 
Saenger). 

Congenital  paralyses  may  occur  under  all  kinds  of  forms  (Kunn, 
Zweig).     The  following  comprise  some  of  the  more  frequent  types. 

A.  Paralysis  of  the  Superior  Rectus. — This  is  either  unilateral  or 
bilateral ;  partial  or  more  often  complete ;  and  may  or  may  not  be 
associated  with  ptosis.  This  condition  is  quite  frequent.  It  is  prob- 
ably due  in  most  cases  to  underdevelopment  or  actual  absence  of  the 
muscle. 

B.  Ptosis. — This  is  nearly  always  bilateral.  It  is  usually  incom- 
plete, the  lid  simply  drooping  enough  to  give  the  patient  a  sleepy  look. 
Sometimes,  however,  as  in  Lawford's  cases,  the  eye  is  completely  closed. 
Congenital  ptosis  is  often  combined  with  other  defects,  particularly  with 
paralysis  of  the  superior  rectus. 

It  may  be  nuclear  in  origin,  but  is  probably  in  most  cases  due  to 
structural  defects  in  the  muscle  itself  (absence  or  conversion  into  con- 
nective tissue,  underdevelopment,  restriction  by  adhesions). 

C.  Paralysis  of  the  Externus  with  Paresis  of  the  Internum  and 
Retraction  Movements. — This  condition  usually  presents  itself  under  the 
guise  of  a  well-marked  syndrome  characterized  by  some  or  all  of  the 
following  features : 

(1)  The  affected  eye  can  move  out  little,  if  at  all,  beyond  the 
median  line. 

(2)  Its  movement  inward  is  more  or  less  restricted.  Moreover^ 
even  when  the  amount  of  movement  inward  is  nearly  normal,  the 
movement  itself  is  performed  wdth  difficulty,  the  eye  always  lagging 
behind  its  fellow,  as  showm  by  the  presence  of  a  crossed  diplopia,  in- 
creasing as  the  eye  is  turned  in. 

(3)  When  the  affected  eye  is  turned  in,  it  retracts  into  the  orbit 
and  often  also  shoots  obliquely  up  or  down,  and  its  palpebral  fissure 
contracts,  often  considerably. 

(4)  The  power  of  convergence  is  often  considerably  impaired. 

(5)  The  field  of  single  vision  is  very  narrow  indeed.  The  patient 
when  he  looks  to  the  side  of  the  affected  eye  has  homon^^nous  diplopia, 
which  passes  almost  at  once  into  a  crossed  diplopia  when  he  begins 
looking  in  the  opposite  direction. 

These  cases  are  not  uncommon,  some  fifty-five  having  been  described 
(Duane).    Usually  the  left  eye,  rarely  both  eyes,  are  affected. 

The  underlying  cause  of  this  condition  is  a  congenital  absence  of  the 
external  rectus  or  its  transformation  into  a  connective  tissue  cord.  In 
some  cases  this  cord  is  elastic  and  the  eye  can  be  rotated  inward  by 


224  THE    EYE    AND    NERVOUS    SYSTEM 

passive  traction  (with  forceps) ;  in  other  cases  where  the  cord  is  in- 
elastic this  is  not  possible. 

The  retraction  of  the  eyeball  when  adducted  is  due  in  most  cases 
undoubtedly  to  the  fact  that  the  externus  is  thus  inelastic,  so  that  the 
eyeball,  being  hugged  between  an  inextensible  cord  on  the  one  side  and 
the  contracting  internus  on  the  other,  must  necessarily  recede  (Tiirk, 
Wolff).  Other  cases,  however,  are  due  to  the  fact  that  the  internus  is 
inserted  too  far  back  (Ileuck,  Evans),  or  has  an  additional  slip  that  is 
so  inserted  (Bahr,  Axenfeld  and  Schiirenberg) . 

D.  Somewhat  analogous  to  the  condition  just  described  is  that 
known  as  strabismus  fixus.  In  this  the  eye  is  turned  strongly  in  and  can- 
not be  turned  outward  even  by  traction  with  forceps  (Berry).  Opera- 
tion has  no  effect,  the  eye  being  evidently  fixed  in  its  deviating  con- 
dition by  strong  adventitious  bands.  This  condition  tends  to  grow 
worse  in  after  life  and  is  not  always  congenital  (Fisher,  Donaldson, 
De  Vincentiis). 

There  are  certain  peculiarities  distinguishing  congenital  from  ac- 
quired paralyses.  These  are  according  to  Kunn  (cited  by  Wilbrand 
and  Saenger,  and  Groenouw)  : 

(1)  Absence  of  secondary  contracture  (page  243).  This  is  a  usual 
but  not  an  invariable  sign,  for  secondary  contracture  may  be  absent  in 
acquired  and  present  in  congenital  paralysis  (A.  Graefe).  Correspond- 
ing to  this  absence  of  contracture  is  the  fact  that  there  may  be  no  devia- 
tion in  the  primary  position,  or  there  may  be  a  deviation  contrary 
to  what  we  should  expect.  But  such  an  anomalous  placing  of  the 
eyes  in  congenital  paralysis  seems,  often  at  least,  to  be  due  to  com- 
plications which  would  produce  a  like  result  if  the  paralysis  were 
acquired. 

(2)  Retention  of  convergence  in  spite  of  the  loss  of  ability  to  per- 
form lateral  movements.     This  is  by  no  means  a  sure  differential  sign. 

(3)  Absence  of  secondary  deviation  in  the  sound  eye.  But  many 
cases  of  congenital  paralysis  present  a  well-marked  secondary  deviation 
(Duane). 

(4)  Absence  of  spontaneous  diplopia.  This  again  is  by  no  means 
the  case  in  all  congenital  paralyses. 

(5)  Sudden  spasmodic  action  of  the  apparently  paralyzed  muscle. 
(G)   Absence  of  any  involvement  of  the  iris  and  ciliary  body.     This 

speaks  strongly  for  an  orbital  origin  of  the  paralysis  (structural  paraly- 
sis). If  the  paralysis  were  nuclear  we  should  expect  the  interior 
muscles  to  be  at  least  occasionally  affected. 

A  point  laid   down  by  Werthheim    (cited   by  Zweig)    is  that  if 


THE  EXTKA-OCULAR  MUSCLES  225 

ptosis  is  present,  the  levator  will  show  a  reaction  of  degeneration  in 
acquired  but  not  in  congenital  paralysis. 

Other  alleged  differential  points,  such  as  the  frequent  presence  of 
nystagmus  or  of  impaired  vision  in  cases  of  congenital  paralysis,  are 
of  no  real  diagnostic  value. 

2.  Heredity. — The  effect  of  heredity  is  seen  in  some  forms  of  con- 
genital paralysis,  particularly  ptosis,  which  show  a  distinct  tendency 
to  run  in  families.  The  same  thing  is  occasionally  seen  in  acquired 
paralysis, — e.g.,  in  acquired  ptosis  (Delord)  and  in  a  case  reported  by 
Beaumont,  in  which  ophthalmoplegia  exterior  occurred  regularly  in  sev- 
eral members  in  each  of  four  generations  of  the  same  family. 

Heredity  also  plays  an  important  part  in  inducing  the  conditions 
(arteriosclerosis,  syphilis,  etc.)  which  frequently  lead  to  paralysis  (see 
infra). 

3.  Traumatism. — This  is  a  fairly  frequent  cause  of  paralysis. 
Paralysis  of  the  external  rectus  is  most  frequent  and  may  be  bilateral. 
It  is  sometimes  evidently  orbital  in  origin,  being  due  to  injury  of  the 
muscle  itself  or  the  orbital  section  of  the  abducens  nerve;  at  other 
times,  especially  when  bilateral,  is  basal,  being  due  particularly  to 
fractures  through  the  petrous  bone.  Traumatic  paralysis,  complete  or 
partial,  of  the  third  nerve  is  also  often  basal,  being  due  to  fracture  of 
the  base  of  the  skull  or  penetrating  wounds  through  the  orbit  or  mouth. 
Isolated  paralysis  of  the  superior  or  inferior  rectus  or  of  the  inferior 
oblique  is  also  frequently  (although  not,  as  has  been  asserted  in  the 
case  of  the  latter  muscles,  invariably)  the  result  of  traumatism.  The 
injury  here  usually  affects  the  muscle  itself  or  the  nerve-terminals  in 
the  orbit  (orbital  paralysis).  The  same  is  true  of  the  not  infrequent 
traumatic  ptosis. 

Traumatic  paralysis  of  the  superior  oblique  is  rather  uncommon. 
It  may  be  basal  or  orbital — on^ variety  of  the  latter  being  the  paralysis 
sometimes  observed  after  operations  upon  the  frontal  sinus  and  caused 
by  injury  to  the  trochlea. 

Traumatic  paralyses  are  due  either  to  direct  injury  of  the  muscle  or 
nerve,  in  which  case  they  are  found  immediately  after  the  injury,  or  to 
compression  by  displaced  bone,  effused  blood,  callus,  traumatic  cerebral 
abscess,  or  a  traumatic  aneurism  (especially  of  the  internal  carotid), 
in  which  case  they  may  not  develop  until  some  time  later.  In  some 
cases,  but  probably  not  often,  traumatic  paralysis  is  nuclear,  being  due, 
according  to  Bernheimer,  to  distention  of  the  ventricles  consequent 
upon  the  injury  and  leading  to  hemorrhage  into  the  nuclei  adjoining. 

As  above  noted  (page  222),  traumatic  paralysis  may  be  congenital. 
15 


2-2G  THE    EYE    AND    NERVOUS    SYSTEM 

Some  cases  of  traumatic  paralysis,  especially  when  due  to  injury  of 
the  face,  are  associated  with  enophthalmos.  This,  as  Purtscher  says, 
may  be  caused  by  (a)  retraction  due  to  scar-tissue;  (6)  loosening 
of  the  check-ligaments  or  the  insertions  of  the  obliques;  (c)  displace- 
ment of  the  contents  of  the  orbit;  (d)  paresis  of  the  sympathetic  (in 
this  case  there  will  be  unilateral  miosis)  ;  (e)  trophic  changes  from 
injury  to  the  peripheral  nerves. 

One  variety  of  traumatic  paralysis  that  we  meet  with  pretty  often 
is  that  produced  by  a  tenotomy.  We  must,  in  fact,  remember  that 
whenever  we  produce  an  effective  tenotomy  we  really  produce  a  paresis 
of  the  muscle.  The  action  of  the  check-ligaments  and  of  the  adminicula 
of  the  tendons  prevents  this  paresis  from  becoming  complete,  and  when 
the  tendon  re-attaches  itself  it  is  able  to  work  on  the  eyeball  as  before, 
although  not  as  effectually.  Nevertheless,  even  after  re-attachment,  if 
the  operation  has  had  any  effect  at  all,  we  can  usually  demonstrate 
restriction  of  movement  such  as  would  be  caused  by  paresis  of  the 
muscle,  and  in  many  cases  also  a  characteristic  diplopia.  This  is  espe- 
cially seen  after  tenotomies  of  the  superior  and  inferior  recti.  In  these 
for  years  after  the  original  operation  we  can  in  many  cases  demonstrate 
diplopia  in  one  quadrant  of  the  field  of  fixation. 

The  fact  that  a  tenotomy  has  caused  a  paralysis  may  often  be  in- 
ferred from  (a)  the  presence  of  a  conjunctival  scar  or  of  sub-conjunc- 
tival  adhesions.  If  not  demonstrable  by  inspection  these  can  be  readily 
made  out  by  exploration  through  a  trifling  and  harmless  incision  in  the 
conjunctiva  made  under  cocaine.  (6)  There  may  be  slight  exophthal- 
mus,  particularly  when  the  eye  is  at  rest,  (c)  In  paralysis  of  the 
superior  or  inferior  rectus,  due  to  a  tenotomy,  the  palpebral  fissure  is 
wider  than  normal,  since  with  the  retraction  of  the  tendon,  its  palpebral 
band  also  retracts  and  pulls  the  lid  back.  In  paralysis  of  these  muscles 
due  to  any  other  cause,  the  palpebral  band  is  drawn  upon  less  than 
usual,  because  the  muscle  to  mIucIi  it  is  attached  is  flaccid,  and  hence 
the  palpebral  fissure  is  narrower  than  normal. 

4.  Vascular  Changes  in  the  Brain. — Paralysis,  both  basal  and  nuclear, 
may  be  caused  by — 

(a)  Ilypercemia  and  ancemia  of  the  brain.  These  produce  often 
very  transient  paralyses  (Bernheimer). 

(6)   Embolism. 

(c)  Sclerosis  of  the  vessels  due  to  senile  changes,  syphilis,  lead- 
poisoning,  etc.,  causes  paralysis  by  pressure  or  more  often  by  hemor- 
rhage due  to  their  rupture. 

(d)  Aneurisms  either  (1)  of  the  internal  carotid  or  posterior  cere- 


THE  EXTRA-OCULAK  MUSCLES  227 

bral,  or  (2)  miliary  aneurisms  of  the  small  vessels.  The  former  act 
by  pressure.  Aneurism  of  the  internal  carotid  causes  usually  gradual 
paralysis  of  the  third  nerve,  combined  with  paralysis  of  the  fifth ;  less 
often  paralysis  of  the  fourth  and  sixth  nerves.  Miliary  aneurisms, 
which  are  frequently  due  to  syphilis  or  to  the  effect  of  alcohol,  tobacco, 
or  other  deteriorating  influences,  give  rise  to  hemorrhages  producing 
basal  or  nuclear  paralyses. 

5.  General  Distention  of  the  Brain,  or  Increase  of  Intracranial  Pressure. 
— Abscesses  or  tumors  in  the  interior  of  the  brain,  or  other  processes 
(hydrocephalus)  which  are  not  so  situated  as  to  cause  direct  pressure 
on  the  nerves,  yet  may  induce  paralysis  by  indirect  pressure,  especially 
if  at  the  same  time  there  are  other  conditions  (arteriosclerosis,  etc.) 
which  favor  the  development  of  paralysis.  In  such  a  case  lumbar 
puncture  may  relieve  the  condition  (Brieger  cited  by  Eversbusch). 

6.  Inflammation  of  the  Brain  and  Membranes. — (a)  Encephalitis  of 
adults  and  of  children  (inflammatory  cerebral  softening,  cerebral  in- 
fantile paralysis)  is  an  occasional  cause  of  paralysis. 

(&)  Abscess  of  the  brain,  either  traumatic  or  otitic;  especially  the 
otitic  abscess  of  the  temporal  lobe,  which  may  cause  paralysis  of  the 
third  (rarely  of  the  sixth)  nerve.  It  is  often  associated  with  other 
evidences  of  pressure,  especially  optic  neuritis  and  a  crossed  hemiplegia. 

(c)  Polioencephalitis  superior,  a  condition  of  hemorrhagic  infiltra- 
tion of  the  gray  matter  about  the  ventricles,  producing  destruction  first 
of  the  association  tracts  and  then  of  the  nuclei,  and  ending  in  complete 
or  nearly  complete  ophthalmoplegia.  It  occurs  particularly  in  drinkers, 
but  may  also  be  caused  by  influenza.  It  is  usually  but  not  invariably 
fatal. 

{d)  Meningitis,  syphilitic,  tuberculous  or  cerebrospinal.  The 
nerves  at  the  base  may  be  affected  either  because  they  are  compressed 
by  the  exudate  or  because  they  become  secondarily  inflamed.  Cerebro- 
spinal meningitis  is  especially  apt  to  cause  abducens  paralysis  and 
ptosis. 

7.  Other  Diseases  of  the  Brain  producing  Ocular  Paralyses. —  (a) 
Acute  bulbar  paralysis  causing  often  paralysis  of  the  abducens,  and 
not  infrequently  of  the  oculo-motor  nerve  also. 

(&)  Myasthenia  gravis.  In  this  evidences  of  paresis  of  the  ocular 
muscles  (especially  ptosis)  are  very  frequent  and  are  often  the  initial 
symptoms. 

(c)  Progressive  amyotrophic  bulbar  paralysis  and  pseudobulbar 
paralysis  very  rarely  cause  ocular  paralyses. 

8.  Tumors  of  the  brain  or  skull,  especially  glioma,  tuberculoma,  and 


228  THE    EYE    AND    NERVOUS    SYSTEM 

gumma,  of  the  brain;  gumma,  exostosis  and  sarcoma  in  the  cranial 
cavity  and  orbit;  and  especially  tumors  of  the  hypophysis  (acromeg- 
aly.) In  some  cases,  according  to  Bernheimer,  these  tumors  act  not 
only  by  causing  pressure,  but  also  by  virtue  of  the  toxins  which  they 
discharge  into  the  circulation.  In  cases  of  basal  paralyses  due  to 
tumors,  the  third  nerve  is  particularly  apt  to  be  affected.  Involvement 
of  other  cranial  nerves  (second,  fifth,  seventh,  and  twelfth)  is  common, 
and  optic  neuritis  is  very  frequently  although  not  invariably  present. 

Tumors  of  the  pons  readily  give  rise  to  abducens  paralysis,  often 
combined  with  paralysis  of  the  facial  and  with  crossed  hemiplegia,  and 
not  infrequently  with  involvement  of  the  fifth,  eighth,  and  twelfth 
nerves. 

Rarely,  as  we  have  already  seen  (page  219),  paralysis  may  be  due  to 
metastases  of  tumors  taking  place  in  the  nerves  or  the  muscles  them- 
selves. 

9.   Spinal  Diseases. — Chief  among  these  are — 

(a)  Tabes. — This  is  a  very  frequent  cause  of  paralysis  of  the  eye 
muscles  both  interior  (see  Chapter  XII)  and  exterior.  According  to 
Uhthoff,  one-fifth  of  all  ocular  paralyses  are  tabic  and  rather  more  than 
one-fifth  of  all  tabic  patients  have  ocular  paralyses.  Partial  or  com- 
plete paralyses  of  the  third  nerve  are  the  most  common  (54  per  cent.), 
next  comes  paralysis  of  the  abducens  (33  per  cent.),  and  of  the  superior 
oblique  (8  per  cent.),  while  in  5  per  cent,  all  the  exterior  muscles  are 
affected.  In  about  one-quarter  of  the  cases  the  optic  nerve  is  simul- 
taneously affected,  and  in  10  per  cent,  there  is  facial  paralysis. 

Tabic  paralyses  are  apt  to  occur  early  in  ihe  disease.  This  is  par- 
ticularly true  of  ptosis,  which  is  very  frequent  indeed,  and  often,  in 
fact,  the  only  evidence  of  paralysis.''^  In  any  case,  but  especially  when 
occurring  early,  tabic  paralyses  are  usually  incomplete,  fluctuating  and 
temporary.  Often  the  paralysis  affects  only  one  or  two  muscles,  and 
these  incompletely;  then,  after  lasting  for  a  period  varying  from  a 
few  days  to  several  months,  and,  after  extending  perhaps  to  other 
muscles  in  the  same  eye  or  its  fellow,  it  disappears  spontaneously. 
Recurrences  are  freq\ient  either  in  the  muscles  originally  affected  or  in 
others.  The  Argyll-Robertson  pupil  is  usually  found  and  the  knee- 
jerk  is  usually  absent. 

Paralyses  which  develop  later  in  the  course  of  the  disease  are  apt 

•  Fraenkel  thinks  that  in  many  of  these  cases  of  tabic  ptosis,  especially  when 
very  transient,  there  is  no  real  paralysis,  the  patient  failing  to  lift  the  lid  simply 
because,  owing  to  the  accompanying  anesthesia,  he  cannot  tell  whether  it  is  raised 
or  not.    To  this  condition  may  be  given  the  name  of  pseudoptosis  ancesthetica. 


THE  EXTEA-OCULAK  MUSCLES  229 

to  be  both  more  complete  and  more  lasting,  and  some  are  absolutely 
permanent.  A  fair  proportion  of  the  cases  of  progressive,  ultimately 
total  ophthalmoplegia  are  tabic, 

A  tabes  which  has  been  preceded  by  syphilis  or  one  which  is  com- 
bined with  psychic  symptoms  is  much  more  likely  to  cause  eye-paralyses 
than  an  uncomplicated  tabes. 

Most  tabic  paralyses  are  nuclear,  although  some  are  undoubtedly 
basal  and  even  orbital  in  origin.  Striimpell  thinks  that  the  initial, 
transitory  paralyses  are  especially  apt  to  have  a  peripheral  site. 

(b)  Combined  Lateral  and  Posterior  Sclerosis. — This  not  infre- 
quently produces  paralysis  of  the  eye  muscles. 

(c)  Multiple  Sclerosis. — Paralysis  of  the  ocular  muscles  occurs 
very  frequently  in  this  disease  (according  to  Uhthoff  in  from  17  to 
20  per  cent,  of  the  cases).  Paralysis  of  the  abducens  (sometimes 
bilateral)  is  most  frequent;   trochlear  paralysis  is  very  rare. 

As  in  tabes,  paralysis  often  occurs  early,  being  sometimes  the  in- 
itial symptom  of  the  disease;  and  also,  as  in  tabes,  the  paralysis  is 
apt  to  be  incomplete  and  transitory.  Indeed,  a  complete  and  permanent 
paralysis  is  very  rare. 

An  associated  symptom  of  considerable  diagnostic  importance  is 
nystagmus  or  pseudonystagmus  (see  Etiology  of  Nystagmus  in  Sec- 
tion V,  page  278). 

The  site  of  the  paralysis  is  usually  nuclear,  but  sometimes 
peripheral. 

(d)  Syringomyelia. — Ocular  paralyses,  especially  paralysis  of  the 
abducens,  occur  in  about  11  per  cent,  of  all  cases  of  syringomyelia 
(Uhthoff).  Here  also  true  nystagmus  is  a  frequent,  associated  symp- 
tom (Compare  Chapter  XV). 

(e)  Acute  Ascending  Paralysis. — This  not  infrequently  causes 
paralysis,  due  to  change  in  the  nuclei  or  the  peripheral  nerves,  with,  in 
some  cases,  degenerative  changes  in  the  muscles  themselves.  Bilateral 
abducens  paralysis  is  most  frequently  observed;  next  unilateral  ab- 
ducens paralysis  without  or  less  often  with  paralysis  of  the  third  and 
fourth  nerves.  Paralysis  of  the  third  or  fourth  nerves  without  involve- 
ment of  the  abducens  is  rare  (Uhthoff).     (Compare  Chapter  XV.) 

(/)  Progressive  muscular  dystrophy  causes  a  primary  orbital 
paralysis  due  to  atrophic  changes  in  the  muscles  themselves. 

(g)  Other  spinal  diseases,  very  rarely  causing  ocular  paralysis,  are 
lateral  sclerosis,  Friedreich's  ataxia,  amyotrophic  lateral  sclerosis,  pro- 
gressive muscular  atrophy,  poliomyelitis  acuta,  and  myelitis.   Chap.  XV. 

10.   General  Paresis. — Paralyses  of  the  ocular  muscles  may  form  a 


230  THE    EYE    AND    NERVOUS    SYSTEM 

prodromal  symptom  of  general  paresis  or  may  develop  after  the  psycho- 
sis has  become  well  established.  They  are  not  very  freqnent  and  are 
usually  transitory.  In  1  to  2  per  cent,  of  the  cases,  however,  a  chronic 
and  progressive,  ultimately  total  ophthalmoplegia  develops  (Chapter 
XII). 

11.  Hysteria,  Neurasthenia,  and  the  Traumatic  Neuroses  very  rarely  in- 
deed produce  paralysis  of  the  individual  muscles,  causing  rather  paraly- 
sis or  spasm  of  the  associated  parallel  movements  or  of  convergence. 
Apart  from  hysterical,  ptosis,  which  is  probably  in  most  cases  referable 
to  a  spasm  of  the  orbicularis,  and  which  in  other  cases  is  probably  a 
pseudo-paralysis  due  simply  to  anaesthesia  of  the  lid  (cf.  Fraenkel,  p. 
228,  note),  cases  of  paralyses  of  the  abducens  and  of  branches  of  the 
third  nerve  have  been  reported.  Most  of  these  are  doubtful.  In  Sachs's 
case,  for  example,  reported  as  an  hysterical  ophthalmoplegia,  the 
paralysis  apparently  affected  rather  the  associated  movements  than  the 
muscles  themselves.  But  genuine  cases  of  abducens  paralysis  due  to 
hysteria  have  been  reported  as  by  Duchenne,  Leber,  Parinaud,  and 
Suker  (See  Chapter  XVI). 

It  need  scarcely  be  added  that  diplopia  occurring  in  hysteria  is  not, 
without  further  examination,  to  be  taken  as  an  evidence  of  paralysis. 

12.  General  Diseases  causing  paralysis  are — 

(a)  Syphilis. — This,  especially  in  the  tertiary  stage,  is  by  far  the 
most  frequent  cause  of  paralysis.  Apart  from  the  post-syphilitic  dis- 
eases, like  tabes,  general  paresis,  and  lesions  of  the  vessels  (sclerosis 
and  miliary  aneurisms),  all  of  which,  as  we  have  seen,  so  often  produce 
ophthalmoplegias,  syphilis  acts  directly  to  induce  paralysis,  by  causing 
periostitis  or  gimimy  growth  in  the  orbit;  periostitis,  meningitis,  or 
gummy  infiltrations  at  the  base  of  the  brain ;  and  gummata  or  degenera- 
tive processes  in  or  near  the  nuclei.  Probably  at  least  half  of  the  cases 
of  ocular  paralysis  are  due  thus  directly  to  syphilis. 

Paralysis  of  the  exterior  muscles,  due  to  syphilis,  is  usually  basal. 
The  third  nerve,  in  some  or  all  of  its  branches,  is  the  one  most  commonly 
affected,  although  any  or  all  of  the  motor  nerves  of  the  eye  may  be 
attacked.  The  paralysis  often  develops  very  suddenly,  and  is  fre- 
quently associated  with  or  preceded  by  evidences  of  involvement  of 
other  cranial  nerves,  especially  headache  and  anaesthesia  or  neuralgia 
along  the  branches  of  the  fifth  nerve.  There  may  also  be  optic  neuritis 
and  either  scotomata  or  contraction  of  the  visual  field,  which  latter  may 
be  more  or  less  symmetrical,  especially  if,  as  often  happens,  the  region 
of  the  chiasm  is  occupied  by  a  syphilitic  exudate.  The  paralysis  when 
once  developed  is  often  progressive,  but  may  be  stationary  or  show 


THE  EXTRA-OCULAE  MUSCLES  231 

sudden  variations,  or  even  disappear  altogether.  It  is  not,  however, 
so  apt  to  be  transitory,  nor  so  apt  to  be  limited  to  single  muscles  as  in 
the  case  of  tabic  paralysis  (Fournier). 

Congenital  syphilis  very  rarely  indeed  causes  paralysis.  Cases, 
however,  have  been  reported  (Lawford,  Zappert,  and  Schmidt-Rimpler 
cited  by  Bernheimer,  who  also  reports  a  case;    Cruchandeau). 

(6)  Diphtheria. — About  5  or  6  per  cent,  of  all  cases  of  diphtheria 
are  followed  (usually  in  the  course  of  three  or  four  weeks)  by  a  paralysis 
of  accommodation,  which  is  generally  bilateral  and  is  generally  unac- 
companied by  paralysis  of  the  sphincter. 

Probably  some  2.5  per  cent.  (Goodall,  cited  by  Groenouw)  of  the 
cases  of  diphtheria  are  followed  by  paralysis  of  some  of  the  exterior 
muscles  of  the  eyeball.  Much  the  most  frequent  form  is  paralysis  of 
the  abducens,  which  is  often  bilateral.  This  is  frequently  associated 
with  paralysis  of  the  accommodation.  Complete  paralysis  of  the  third 
nerve  is  very  rare  (two  of  Tellais's  cases  and  one  of  Parkinson's  cited  by 
Groenouw),  and  partial  third-nerve  paralysis  is  uncommon. 

Cases  of  trochlear  paralysis  have  been  described  by  Graefe  and 
Krause.  TJhthoff  and  others  report  a  number  of  cases  in  which  all  the 
exterior  muscles  of  both  eyeballs  (mostly  without  involvement  of  the 
accommodation)  have  been  attacked. 

Other  paralyses,  especially  of  the  soft  palate,  and  not  infrequently 
of  the  body -muscles  (hemiplegia  and  paraplegia)  may  occur  at  the  same 
time  with  the  paralysis  of  the  ocular  muscles. 

Paralysis  of  the  exterior  muscles  of  the  eyes  is  especially  apt  to  fol- 
low severe  cases  of  diphtheria,  while  paralysis  of  accommodation  may 
occur  in  connection  with  very  light  forms  of  the  disease  (Groenouw). 

The  paralysis  in  some  epidemics  appears  and  disappears  very 
suddenly,  leaving  one  muscle  to  attack  another  (Pagenstecher, 
cited  by  Groenouw).  In  almost  all  cases  the  paralysis  ultimately 
disappears  altogether.  Miihsam  (cited  by  Groenouw),  however 
reports  one  case  of  permanent  paralysis  of  acommodation ;  and  Mor- 
vat  and  I  have  reported  cases  of  permanent  paralysis  of  the  exterior 
muscles. 

In  at  least  three  of  the  reported  cases  (Groenouw,  Tellais),  death 
ensued  soon  after  the  development  of  the  paralysis. 

The  lesion  causing  the  paralysis  has  been  variously  ascribed  to 
capillary  haemorrhages  in  the  nerve-nuclei,  disease  of  the  blood-vessels 
supplying  the  nuclei  or  nerves,  basal  or  orbital  neuritis,  and  inflam- 
matory changes  in  the  muscles  themselves  (Groenouw). 

(c)   Influenza  often  causes  a  paralysis  of  accommodation,  and  pa- 


232  THE    EYE    AND    NERVOUS    SYSTEM 

ralyses  of  the  exterior  muscles  are  not  very  infrequent.  The  abducens 
is  especially  apt  to  be  affected,  although  paralysis  of  some  or  all  of  the 
muscles  supplied  by  the  third  nerve  is  also  observed.  Isolated  trochlear 
paralysis  is  very  rare.  In  some  instances  there  is  paralysis,  complete 
or  partial,  of  all  the  muscles  moving  the  eyeball. 

The  paralysis  of  the  accommodation  is  usually  bilateral,  the  paraly- 
sis of  the  exterior  muscles  unilateral,  although  bilateral  paralysis  of 
the  abducens  (Bernheimer),  the  trochlear  (Pfliiger),  and  the  third 
nerve  have  been  reported. 

In  these  paralyses  recovery  is  the  rule.  In  some  cases,  however, 
especially  when  the  exterior  muscles  are  involved,  the  paralysis  remains 
indefinitely. 

The  lesions  causing  the  paralysis  are  supposed  to  be  neuritis,  nu- 
clear or  basal  hemorrhages,  circum ventricular  hemorrhages  with  the 
picture  of  a  polioencephalitis  superior  (page  227),  and  a  true  encephali- 
tis. Influenza  may  also  cause  paralysis  by  inducing  an  orbital  cellu- 
litis or  an  inflammation  of  Tenon's  capsule. 

(d)  Tuberculosis  may  cause  paralysis  by  inciting  a  tuberculous 
meningitis  (page  227).  Otherwise,  paralysis  is  very  rarely  indeed  the 
result  of  tuberculosis,  although  now  and  then  it  may  be  induced  by 
tuberculous  foci  situated  at  the  foramina  of  exit  of  the  nerves,  or  at  the 
base  of  the  skull  or  in  the  brain  itself.  The  paralysis  is  usually  basal 
or  foramina],  very  rarely  nuclear. 

(e)  Rheumatism. — Acute  articular  rheumatism  occasionally  pro- 
duces paralysis,  mostly  as  a  result  of  the  metastatic  processes  (menin- 
gitis, orbital  cellulitis,  tenonitis)  to  which  it  gives  rise. 

Much  more  frequent  are  certain  paralyses  to  which  the  name  rlieu- 
matic  is  usually  applied ;  occurring  generally  as  a  result  of  exposure  to 
cold,  and  especially  in  those  who  are  the  subjects  of  chronic  rheuma- 
tism. The  paralysis  usually  develops  suddenly  and  is  often  associated 
with  various  rheumatic  manifestations,  with  neuralgic  pains  along  the 
course  of  the  fifth  nerve,  and  sometimes  with  paralysis  of  the  facial. 
The  abducens  is  most  commonly  affected,  but  paralyses  of  the  other 
nerves,  complete  or  partial,  are  not  infrequent. 

Such  paralysis  is  usually  quite  amenable  to  treatment  with  salicy- 
lates and  by  diaphoresis. 

(/)  Herpes  zoster  ophthalmicus,  especially  the  forms  that  are  asso- 
ciated with  fever  or  are  due  to  toxic  conditions,  often  cause  paralysis. 
This  usually  affects  isolated  muscles  supplied  by  the  third  nerve, 
although  sometimes  all  the  branches  of  the  latter  are  attacked.  The 
fourth  and  sixth  nerves  are  rarely  affected. 


THE  EXTEA-OCULAK  MUSCLES  233 

(g)  Typhoid  Fever. — Paralysis  of  any  of  the  motor  nerves  of  the 
eye  may  develop  in  typhoid  fever,  usually  after  the  second  week  but 
sometimes  earlier.  They  are  ascribed  to  complicating  affections  of  the 
brain  or  meninges  or  to  a  peripheral  neuritis  (Groenouw),  but  may 
perhaps  also  be  due  to  hemorrhage.  They  are  sometimes  transient  but 
may  be  permanent. 

(h)  Cerebrospinal  meningitis  (page  227). 

(t)  Malaria  very  rarely  causes  an  oculo-motor  or  an  abducens  paraly- 
sis, which  sometimes  recurs  periodically  with  the  fever  and  which  dis- 
appears under  the  use  of  quinine. 

(;)  Gerlier's  disease  (a  peculiar  disease  endemic  in  Switzerland, 
marked  by  recurrent  attacks  of  vertigo  and  paresis  of  the  extremities) 
often  produces  ptosis  and  paralysis  of  the  abducens  or  of  the  individual 
branches  of  the  third  nerve.  A  somewhat  similar,  but  often  fatal,  affec- 
tion is  hubi-sagari,  endemic  in  Japan.  In  this,  too,  ptosis  and  other 
ocular  paralyses  are  frequent. 

(k)  Beriberi  often  produces  paralyses  which  are  usually  transient. 

(I)  Other  infectious  diseases  occasionally  producing  paralysis  are 
pneumonia  (Alt,  Aldrich)  ;  tetanus  and  cephalic  tetanus ;  Weil's  dis- 
ease (Groenouw);  mumps;  whooping-cough  (Go  wring.  Batten); 
erysipelas  and  pysemic  states.  These  probably  act  mainly  by  their 
secondary  effects  (meningitis,  orbital  cellulitis,  hemorrhages  due  to 
convulsions). 

13.     General  conditions  not  essentially  infectious  are — 

(a)  Diabetes. — In  some  dozen  recorded  cases,  paralysis  of  the  eye 
muscles  has  been  caused  by  diabetes  mellitus,  and  in  one  or  two  in- 
stances by  diabetes  insipidus.  In  the  latter  case  there  was  probably  a 
lesion  about  the  fourth  ventricle,  causing  both  the  polyuria  and  the 
paralysis. 

(&)  Renal  Disease. — A  few  cases  of  paralysis  due  to  this  cause  have 
been  reported  by  Dunn,  and  by  other  authorities  (Knies,  Bernheimer). 
They  are  in  all  probability  due  to  hemorrhage.  They  appear  usually 
to  indicate  a  fatal  issue. 

(c)  Exophthalmic  goitre  occasionally  causes  paralysis  of  the  ab- 
ducens and  less  often  of  the  exterior  muscles  supplied  by  the  third 
nerve. 

(d)  Acromegaly. — Apart  from  the  rather  frequent  cases  of  paraly- 
sis produced  by  the  tumors  of  the  hypophysis  in  acromegaly  (page  228), 
paralysis  is  sometimes  apparently  due  to  some  influence  that  this  disease 
exerts  upon  the  general  condition' (Bernheimer).  The  abducens  and 
trochlear  nerves  are  rarely,  if  ever,  affected. 


2U  THE    EYE    AND    .NERVOUS    SYSTEM 

(e)  Aido-inloxicaiious  from  the  digestive  tract  verj  rarely  cause 
paralysis  of  the  exterior  muscles. 

(/")  Profuse  Hemorrhage. — Two  cases  of  bilateral  abducens  paraly- 
sis due  to  this  cause  are  recorded  by  Xeuburger. 

14.  Poisons. — Tliere  are  a  number  of  poisons  which  produce  paraly- 
sis of  the  interior  muscles  of  the  eye  (sphincter  and  ciliary  muscle). 
For  these,  called  cycloplegics,  reference  must  be  had  to  Chapter  VI. 
Paralysis  of  the  exterior  muscles,  on  the  other  hand,  is  rarely  caused 
by  j)oisons. 

{a)  Alcohol. — Chronic  alcoholism  in  a  few  cases  (about  0.5  per 
cent.,  according  to  Uhthoff)  produces  paralysis.  Bilateral  paralysis 
(usually  incomplete)  of  the  abducens  is  the  most  common  form.  This 
seems  to  be  due  to  a  nuclear  losion. 

Alcoholism  also  appears  to  play  an  important  part  in  the  genesis  of 
polioencephalitis  superior,  which,  as  already  stated  (page  227)  is  asso- 
ciated with  a  progressive,  ultimately  total  ophthalmoplegia. 

(6)  Sulphuric  acid  may  in  very  rare  instances  cause  paralysis 
by  producing  polioencephalitis  superior. 

(c)  Gelsemium  often  causes  ptosis  and  diplopia. 

(d)  Other  poisons  occasionally  producing  paralysis  of  the  exterior 
muscles  are  tobacco  (cases  not  certain,  although  Wilkinson's  case  seems 
authentic);  atropine  (Baas);  snake-venom;  lead;  carbon  monoxide 
(illuminating  gas)  ;  fungi;  and  decayed  meat,  fish,  oysters,  etc.  (pto- 
maine-poisoning). In  ptomaine-poisoning  ptosis  is  comparatively  fre- 
(juent. 

15.  Diseases  of  the  nose,  and  especially  of  its  accessory  sinuses,  produce 
paralysis,  mainly  by  mechanical  pressure,  but  sometimes  apparently 
because  of  resorption  of  pent-up  purulent  matter.  The  same  is  ap- 
parently true  of  some  cases  of  otitis,  which  produce  paralysis,  not  by  the 
])ressure  due  to  an  otitic  abscess  (page  227),  but  by  the  resorption  of 
infective  material   (Bernheimer). 

Diagnosis  of  Cause. — 1.  Most  Frequent  Causes. — Of  the  long  list  of 
causes  above  given,  the  ones  most  frequently  encountered  are  syphilis, 
tabes,  multiple  sclerosis,  exposure  to  cold  (rheumatic  paralyses),  trau- 
matism, meningitis,  tumors  and  abscess  of  the  brain,  arteriosclerosis 
and  aneurisms  (producing  pressure  or  hemorrhage).  Less  frequent 
are  general  paresis,  diphtheria,  influenza,  alcoholism  (especially  as  in- 
ducing polio-oncephalitis). 

A  suddenhj  developing  paralysis  is  usually  due  to  exposure  to  cold, 
traumatism,  hemorrhage,  embolism,  or  thrombosis.  In  some  cases  of 
diphtheria  the  paralysis  also  appears  and  disappears  suddenly. 


THE  EXTRA-OCULAR  MUSCLES  235 

Incomplete  and  temporary,  more  or  less  fluctuating,  paralyses  are  the 
special  characteristic  of  tabes  (particularly  in  the  early  stages)  general 
paresis,  and  multiple  sclerosis.  Post-diphtherial  and  rheumatic  paraly- 
ses are  also  usually  temporary,  and  traumatic  paralyses  often  so. 

2.  Abducens  Paralysis  is  frequently  caused  by  syphilis,  tabes,  and 
tuberculous  meningitis,  although  these  conditions  still  more  often  pro- 
duce oculo-motor  paralysis.  On  the  other  hand,  traumatism,  exposure 
to  cold,  cerebrospinal  meningitis,  multiple  sclerosis,  syringomyelia, 
acute  ascending  paralysis,  acute  bulbar  paralysis,  pons  lesions,  diph- 
theria, influenza,  and  alcoholism  much  more  often  cause  paralysis  of 
the  abducens  than  of  the  exterior  muscles  supplied  by  the  third  nerve. 
Congenital  paralysis  also  often  affects  the  abducens.  In  some  of  the 
conditions  here  noted,  especially  acute  ascending  paralysis,  diphtheria, 
and  alcoholism,  hath  abducens  nerves  are  often  affected  simultaneously. 

3.  More  or  less  complete  paralysis  of  the  third  nerve  is  very  often  due 
to  syphilis.  Other  conditions  particularly  apt  to  cause  this  form  of 
paralysis  are  tumors  and  abscesses  of  the  temporal  lobe,  tumors  at  the 
base  of  the  brain  (especially  the  hypophysis  tumors  of  acromegaly), 
and  aneurism  of  the  internal  carotid.  Complete  paralysis  of  the  third 
nerve  is  not  common  in  tabes,  and  bilateral  complete  third-nerve  paraly- 
sis is  hardly  ever  tabic  (L'hthoff). 

4.  Paralysis  of  isolated  branches  of  the  third  nerve  is  found  especially 
in  tabes  and  herpes  zoster,  but  is  also  often  due  to  syphilis,  and  may  be 
caused  by  traumatism  or  be  congenital. 

5.  One  form  of  isolated  paralysis  of  the  third  nerve, — namely,  ptosis, 
— is  of  special  diagnostic  importance. 

Ptosis  without  paralysis  of  any  of  the  mi&cles  moving  the  eyeball 
occurs  most  often  under  the  following  conditions : 

(a)  As  a  congenital  state.  Here  usually  bilateral,  often  incom- 
plete, and  always  permanent. 

(6)  As  a  condition  developing  without  assignable  cause  in  old  age. 
Always  bilateral,  gradual  in  development,  and  unassociated  with  any 
other  evidence  of  nerve  trouble. 

(c)  Tn  tabes.  Frequent,  especially  in  initial  stage  of  tabes.  Usu- 
ally incomplete  and  transitory,    ^fay  be  a  pseudoptosis  (page  228  note). 

(d)  In  multiple  sclerosis.     Rare. 

(e)  As  an  initial  symptom  of  chronic  progressive  ophthalmoplegia. 
(/)   In  syphilis.     Rare. 

(^f)   In  cerebral  softening  (embolic  or  traumatic).     Quite  frequent. 

(h)   In  cerebral  abscess,  particularly  otitic  abscess  of  the  temporal 

lobe  on  the  same  side  as  the  ptosis.     Here  often  associated  with  paresis 


236  THE    EYE    AND    NERVOUS    SYSTEM 

of  the  opposite  side  of  the  face  and  arm,  sometimes  of  the  leg.  The 
same  combination  may  occur  in  tumors  of  the  brain. 

(i)   In  cerebrospinal  meningitis.     Not  uncommon. 

(j)   In  cerebral  hemorrhage.     Occasionally. 

(^•)  Traumatism  affecting  the  lid  or  levator  directly,  or  occasionally 
traumatism  producing  intracranial  lesion  (even  fracture  of  the 
base). 

(l)  As  evidence  of  a  cortical  lesion.  (Usually  in  or  near  the  angular 
gyrus  on  the  opposite  side  to  the  ptosis).    Rare. 

(m)  In  hysteria.     Usually  bilateral. 

(n)  In  ptomaine-poisoning.  Associated  with  mydriasis  and  paraly- 
sis of  accommodation. 

(o)  A  slight  degree  of  ptosis  is  produced  by  paralysis  of  the  superior 
rectus,  provided  this  is  not  due  to  division  of  the  tendon,  a  lesion  which 
causes  retraction  of  the  lid  (page  226). 

Ptosis  with  more  or  less  involvement  of  the  extra-ocular  muscles 
supplied  by  the  third  nerve  is  found  often  in  tabes,  syphilis,  cerebral 
softening,  cerebral  abscess,  tumors  in  various  parts  of  the  brain,  men- 
ingitis (especially  tuberculosis,  Gerlier's  disease,  polioencephalitis 
superior,  and  as  a  result  of  traumatism. 

A  bilateral  incomplete  ptosis^  which,  slight  in  the  morning,  in- 
creases markedly  in  the  evening,  is  characteristic  of  myasthenia  gravis. 

Ptosis  associated  with  abducens  paralysis  (with  or  less  frequently 
without  involvement  of  the  extra-ocular  muscles  supplied  by  the  third 
nerve)  is  very  frequent  in  tabes. 

In  using  ptosis  as  a  diagnostic  sign  we  must  be  careful  to  exclude 
those  conditions  in  which  a  drooping  of  the  lid  obtains  but  is  not  due 
to  third-nerve  paralysis.     Such  conditions  are — 

(a)  Sympathetic  Ptosis. — In  this  the  palpebral  fissure  is  moder- 
ately contracted,  but  as  much  by  raising  of  the  lower  lid  as  by  drooping 
of  the  other.  Repeated  instillations  of  cocaine  will  not  widen  the 
palpebral  fissure  as  they  will  in  a  third-nerve  ptosis.  There  is  usually 
miosis,  and  the  pupil  is  undilatable  by  cocaine.  There  may  be  hyperi- 
drosis  or  anidrosis  with  redness  or  pallor  of  the  face  on  the  side  of  the 
lesion.     This  kind  of  ptosis  is  quite  frequent  in  tabes. 

(b)  Pseudoptosis  from  spasm  of  the  orbicularis  (spastic  ptosis) 
occurs  in  irritative  conditions  of  the  eye,  and  is  the  usual  form  of  ptosis 
in  hysteria.  Perhaps  also  the  ptosis  observed  in  pons  lesions  is  due  to 
this  cause  (Schoeler).  In  this  form,  as  in  sympathetic  ptosis,  both 
lids,  the  lower  as  well  as  the  upper,  take  part  in  producing  contraction 
of  the  palpebral  fissure. 


THE  EXTRA-OCULAR  MUSCLES  237 

(c)  Drooping  of  the  lid  from  mere  excess  of  weight  or  mechanical 
causes  as  in  tarsitis  syphilitica,  trachoma,  marked  oedema,  etc. 

{d)  The  pseudoptosis  ancesthetica  (Fraenkel,  page  229,  note). 

(e)  The  slight  drooping  of  the  lid  produced  by  an  advancement  of 
the  superior  rectus.  This  operation,  by  carrying  the  tendon  forward, 
draws  forward  also  the  superior  palpebral  band  and  with  it  the  upper 
lid,  which  is  thus  depressed  one  or  two  millimetres.  Similarly,  advance- 
ment of  the  inferior  rectus  causes  a  quite  perceptible  elevation  of  the 
lower  lid. 

6.  Paralysis  of  the  exterior  muscles  supplied  by  the  third  nerve  without 
involvement  of  the  fourth  or  sixth  nerves  is  rare  in  acute  ascending 
paralysis,  syringomyelia,  acute  bulbar  paralysis,  diphtheria,  influenza, 
and  alcoholism. 

7.  Isolated  trochlear  paralysis  is  most  often  due  to  syphilis,  tabes, 
meningitis,  sometimes  to  traumatism  (especially  injuries  about  the 
trochlea).  It  is  seldom  the  result  of  diphtheria,  influenza,  or  pons 
lesions,  and  is  very  rarely  caused  by  multiple  sclerosis. 

8.  Simultaneous  paralysis  of  the  third  and  sixth  nerves  on  the  same  side 
ocurs  in  fractures  of  the  base,  especially  one  which  traverses  the  petrous 
bone  and  splits  off  the  clinoid  process. 

9.  Total  ophthalmoplegia, — i.e,  the  condition  in  which  all  three  of  the 
motor  nerves  of  the  eyes  are  paralyzed, — is  most  frequently  due  to 
syphilis,  tabes  (especially  the  later  stages),  orbital  disease,  and  trau- 
matism.    It  is  very  rare  in  general  paresis,  diphtheria,  and  influenza. 

10.  Ophthalmoplegia  exterior  occurs  often  in  syphilis,  also  in  tabes, 
and  various  basal  and  nuclear  diseases.  It  is  usually  bilateral  in 
polioencephalitis  superior  and  the  chronic  progressive  ophthalmoplegia 
of  Graefe  (page  242). 

Diagnosis  of  Site. — The  following  distinctive  signs  often  obtain 
in  cases  of  Orbital  paralysis. 

1.  The  paralysis  is  usually  unilateral. 

2.  It  frequently  affects  a  single  muscle. 

3.  The  interior  muscles  are  not  usually  affected,  except  in  the  case 
of  a  paralytic  mydriasis  produced  by  a  blow  upon  the  eye  itself,  or  a 
sympathetic  miosis  produced  by  injury  to  the  orbit. 

4.  In  traumatic  cases,  particularly,  there  may  be  enophthalmos 
often  associated  with  a  sympathetic  miosis  (page  226). 

5.  In  tumors  or  inflammatory  processes  in  the  orbit  there  is  often 
exophthalmos  or  a  lateral  displacement  of  the  eye. 

6.  Other  evidences  of  orbital  lesion  are  often  present,  such  as  scars 
in  the  lids  or  the  margin  of  the  orbit,  depressed  bone,  orbital  swelling 


238  THE    EYE    AND    NERVOUS    SYSTEM 

ai)i)reciable  by  palpation,  deep  pain  elicited  by  pressure  made  into  the 
orbit,  chemosis,  evidences  of  disease  of  the  accessory  sinuses. 

It  must,  however,  be  remembered  that  the  presence  of  these  evi- 
dences of  orbital  disease  is  not  proof  positive  that  the  paralysis  itself  is 
orbital  in  site.     Thus : 

(a)  Exophthalmos  may  indicate  a  paralysis  due  to  aneurism  of  the 
internal  carotid  or  a  thrombosis  of  the  cavernous  sinus. 

(6)  Paralysis  may  occur  with  shot  or  stab  wounds  of  the  orbit  be- 
cause the  skull  has  been  penetrated  and  the  nerves  injured  at  tlie  base. 

(c)  Paralysis  occurring  in  an  orbital  cellulitis  or  orbital  tumors 
may  be  caused  by  an  extension  of  the  morbid  process  backward  into 
the  cranial  cavity  and  involvement  of  the  nerves  at  the  base. 

(d)  Paralysis  associated  with  marked  evidences  of  orbital  disease 
may,  especially  if  syphilitic,  be  due  to  an  intracranial  process  which 
after  affecting  the  nerves  at  the  base  extends  into  the  orbit.  This  is 
particularly  apt  to  be  the  case  if  the  process  started  by  involving 
isolated  muscles,  especially  those  supplied  by  the  third  nerve. 

A  total  oculo-motor  paralysis  with  completely  intact  fourth  and 
sixth  nerves  is  rarely,  if  ever,  orbital  (Wilbrand  and  Saenger). 

On  the  other  hand,  paralysis  of  all  three  motor  nerves  of  the  eye 
without  involvement  of  other  cranial  nerves  except  the  first  branch  of 
the  fifth  is  almost  certainly  orbital  or  foramina!  in  origin. 

The  following  points  are  useful  in  diagnosticating  a  Basal  paralysis. 

1.  Paralysis  of  the  other  cranial  nerves  are  often  present  (see  next 
section  on  Affections  of  Other  Nerves). 

2.  In  traumatic  paralysis  there  are  often  evidences  of  fracture  of 
the  base  (discharge  of  cerebrospinal  fluid,  bleeding  from  the  ears). 
There  may  also  be  blood  in  the  fluid  obtained  by  lumbar  puncture. 

3.  In  paralysis  due  to  meningitis  there  are  retraction  of  the  head, 
fever,  delirium,  and  severe  headache,  and  leucocytes  are  present  in  the 
fluid  obtained  by  lumbar  puncture. 

4.  In  paralysis  due  to  otitis  there  may  be  found,  besides  the  evi- 
dences of  ear  disease  with  the  accompanying  deafness  and  vertigo,  the 
signs  of  an  abscess  in  the  temporal  lobe  (optic  neuritis,  crossed  facial 
})aralysis  and  hemiplegia,  aphasia,  word-deafness,  mind-blindness, 
homonymous  hemianopsia).  Similar  signs  would  be  found  in  temporo- 
sphenoidal  tumors. 

5.  Complete  or  nearly  complete  third-nerve  paralysis,  especially  if 
bilateral,  is  usually  basal  or  foraminal. 

fi.  Recurrent  ophthalmoplegia  (ophthalmoplegic  migraine — see 
Cha]).  XV 11)  i^i  usually  duo  to  basal  lesions. 


THE  EXTRA-OCULAR  MUSCLES  2:19 

7.  It  was  formerly  thought  that  an  ophthalmoplegia  exterior  and 
isolated  paralyses  of  muscles  supplied  by  the  third  nerve  must  be  nu- 
clear, but  in  a  number  of  cases  these  conditions  have  been  shown  to  be 
due  to  basal  lesions  (Uhthoff,  Bernheimer,  Salomonsohn,  Hochwart). 

Many  of  the  cases  of  what  were  thought  to  be  nuclear  paralysis  are 
really  fascicular,  and  not  a  few  of  the  rest  are  really  basal.  If,  as  is 
generally  the  case  in  tabes  and  multiple  sclerosis,  the  paralysis  is  evi- 
dently due  to  central  causes,  it  is  probably  fascicular  if  more  than  one 
nerve  is  involved.  This  rule,  however,  is  not  without  its  exceptions,  as 
several  instances  of  raultiiile  nuclear  paralyses  have  been  noted  (Bern- 
heimer). 

The  diagnosis  of  paralysis  due  to  a  lesion  of  the  pons  is  based  on 
the  presence  of  the  following: 

1.  Paralysis  of  one  (sometimes  both)  abducens  nerves,  or  more  fre- 
quently a  paralysis  of  conjugate  movement  to  the  right  or  left  (pages 
221  and  270). 

2.  Optic  neuritis  (in  25  per  cent,  of  the  cases). 

3.  Associated  paralysis  of  the  fifth,  seventh  (very  frequent),  eighth, 
or  twelfth  nerves. 

4.  Ptosis  (in  some  cases). 

5.  Paralysis  or  paresis  on  the  opposite  side  of  the  body.  Very 
common.  Sometimes  also  there  are  convulsive  movements  or  general 
convulsions  and  sensory  disturbances. 

Affections  of  Other  Nerves  as  Determining  the  Diagnosis. 

1.  Olfactory  Nerve. — Paralysis  of  this,  causing  anosmia,  occurs  in 
paralyses  associated  with  nasal  disease  and  in  tabes. 

2.  Optic  Nerve. — An  optic  neuritis  associated  with  paralysis  of  the 
eye  muscles  suggests,  in  most  cases,  the  probability  that  the  paralysis  is 
due  to  tumor  or  abscess  of  the  brain  or  to  syphilitic  or  tuberculous 
meningitis.  Less  often  it  occurs  in  paralysis  due  to  multiple  sclerosis 
or  to  morbid  processes  in  the  orbit,  implicating  the  optic  nerve. 

Total  optic-nerve  atrophy  occurs  in  at  least  25  per  cent,  of  paralyses 
due  to  tabes  and  is  also  common  in  paralysis  resulting  from  orbital  in- 
flammation or  tumors  (then  usually  associated  with  exophthalmos), 
traumatism  (especially  fractures  at  the  apex  of  the  orbit),  and  perios- 
titis of  the  sphenoidal  fissure.  A  usually  partial  (temporal),  but  some- 
times total  optic  atrophy  often  accompanies  the  motor  paralyses  pro- 
duced by  multiple  sclerosis. 

Homonymous  hemianopsia  occurs  in  paralysis  due  to  basal  lesions 
affecting  the  optic  tract,  brain  tumors,  and  otitic  abscesses  of  the  tem- 
poro-sphenoidal  lobe. 


240  THE    EYE    AND    NERVOUS    SYSTEM 

Bitemporal  hemianopsia  may  occur  in  paralyses  due  to  chiasm 
lesion   (especially  acromegaly). 

Concentric  contraction  of  the  visual  field  occurs  in  tabic  paralyses 
and  irregular  contraction  or  a  central  scotoma  often  in  multiple 
sclerosis. 

3.  Trigeminus. — Paralysis  of  all  branches  of  the  fifth  combined  with 
paralysis  of  the  third  (less  often  of  the  fourth  or  sixth)  nerves  occur 
especially  in  syphilis,  in  tabes,  in  tumors  of  the  base,  and  in  aneurism  of 
the  internal  carotid. 

Paralysis  of  the  first  branch  of  the  fifth  (with  perhaps  keratitis  neu- 
roparalytica)  combined  with  paralysis  of  the  third  and  with  optic-nerve 
atrophy  indicates  disease  (syphilitic  periostitis)  in  the  sphenoidal 
fissure. 

Paralysis  of  the  fifth  combined  with  paralysis  of  the  abducens 
occurs  especially  in  pons  lesions  and  in  syringomyelia. 

Neuralgia  of  the  fifth  occurs  often  in  syphilitic  and  rheumatic 
paralysis. 

4.  Facial. — Paralysis  of  the  facial  occurs  conjointly  with  paralysis 
of  the  eye  muscles  as  a  result  of 

(a)   Tabes  (in  about  10  per  cent,  of  tabic  paralyses). 

(6)  Otitis  and  particularly  otitic  abscess  of  the  temporo-sphenoidal 
lobe,  in  which  case  the  most  usual  combination  is  a  paralysis  (generally 
partial)  of  the  third  nerve  on  the  side  of  the  abscess  and  paralysis  of 
the  opposite  facial.  Similar  conditions  may  be  produced  by  a  tumor 
situated  in  the  frontal  or  temporal  lobes. 

(c)  Exposure  to  cold.  Here  the  most  frequent  combination  is 
paralysis  of  facial  and  abducens. 

(d)  Traumatism,  especially  fracture  through  the  petrous  bone  im- 
plicating the  facial  and  the  abducens  or  sometimes  the  facial  and  the 
third  nerve  together. 

(e)  Po7is  lesions.  Paralysis  of  the  facial  (usually  the  upper  facial 
being  exempt)  occurs  in  about  two-thirds  of  the  cases  of  pons  disease, 
and  is  then  very  frequently  combined  with  paralysis  of  the  abducens. 

(/)  Acute  ascending  paralysis  (not  infrequent)  and  occasionally 
also  in  other  spinal  diseases  (myelitis,  etc.).  The  most  usual  combina- 
tion is  paralysis  of  the  facial  and  abducens. 

5.  Auditory. — Paralysis  of  the  eighth  nerve  (nerve  deafness,  may 
occur  combined  with  eye  paralyses  in  otitis,  fracture  of  the  petrous 
bone,  syphilis,  tumors  of  the  auditory  nerve,  and  pons  lesions  (in  the 
latter  case  in  conjunction  with  abducens  paralysis). 

6.  Hypoglossal. — Tliis  is  very  often  involved  in  conjunction  with  the 


THE  EXTRA-OCULAR  MUSCLES  241 

abducens  of  the  same  or  the  opposite  side  in  pons  lesions;  sometimes 
also  in  lesions  situated  in  the  posterior  cranial  fossa  near  the  middle 
line. 

7.  Hemiplegia. — Paralysis  of  the  third  nerve  with  complete  hemi- 
plegia of  the  opposite  side  of  the  body  indicates  usually  either  a  lesion 
in  the  ^^eduncle,  or  an  otitic  abscess  in  the  temporo-sphenoidal  lobe  (in 
the  latter  case  often  also  combined  with  paralysis  of  the  opposite  facial), 
or  a  tumor  in  the  frontal  or  temporal  lobe. 

Paralysis  of  the  sixth  nerve  with  hemiplegia  of  the  opposite  side  of 
the  body  indicates  a  lesion  of  the  pons  situated  on  the  same  side  as  the 
paralyzed  abducens. 

Course  and  Prognosis  of  Paralyses. — As  already  stated,  some 
paralyses — especially  those  due  to  direct  effects  of  traumatism  and  to 
hemorrhage,  embolism,  thrombosis,  and  exposure  to  cold — hegin  sud- 
denly. This  is  also  the  case  with  many  diphtherial  and  some  syphilitic 
and  tabic  paralyses. 

In  other  cases  the  paralysis  develops  slowly — sometimes  very  slowly 
indeed,  taking  many  years  before  it  reaches  its  height. 

The  duration  of  a  paralysis  varies  from  a  few  hours  to  a  lifetime. 
A  similar  variation  is  seen  in  the  completeness  of  the  paraWsis,  which 
may  range  from  a  slight  restriction  of  movement  of  a  single  muscle  to 
a  total  ophthalmoplegia  producing  absolute  immobility  of  the  eyeball. 

Transitory  paralyses  are  the  rule  in  tabes  and  multiple  sclerosis. 
The  paralysis  is  also  usually  temporary  when  due  to  diphtheria,  ex- 
posure to  cold,  beriberi,  and  ptomaine-poisoning;  and  often  so  when 
caused  by  general  paresis  and  influenza.  Traumatic  paralysis  is  fre- 
quently temporary  if  due  to  the  pressure  of  effused  blood,  which  can 
undergo  absorption,  to  division  of  a  tendon  which  subsequently  becomes 
re-attached,  or  to  some  similar  remediable  condition. 

Paralyses  which  occur  ea7'ly  in  the  course  of  a  central  nervous  dis- 
ease— particularly  tabes,  multiple  sclerosis,  and  syringomyelia — are  apt 
to  be  less  severe  and  less  permanent  than  those  developing  in  the  later 
stages.  The  same  is  probabh'  true,  although  perhaps  not  so  markedly,  of 
syphilitic  paralyses.  In  syphilis,  however,  the  paralyses  usually  occur 
late  in  the  disease,  and  in  any  case,  although  they  vary  greatly  in 
intensity  and  duration,  are  generally  more  lasting,  more  complete,  and 
more  likely  to  involve  a  number  of  muscles  than  are  the  paralyses  found 
in  tabes  and  multiple  sclerosis. 

Congenital  paralyses  usually  remain  unchanged  through  life.  An 
exception  seems  to  be  strabismus  fixus  (page  224),  which,  even  when 
congenital  in  origin,  may  be  aggravated  by  consecutive  changes  in  adult 


242  THE  EYE  AND  NERVOUS  SYSTEM 

Traumatic  paralyses,  too,  are  apt  to  remain  stationary,  especially  if 
complete,  and  the  same  is  true  of  other  paralyses  when  a  cause  that  has 
ceased  to  act  has  produced  complete  destruction  of  a  nerve  or  muscle. 

In  some  cases  a  paralysis  shows  a  sudden  and  great  increase.  This 
may  occur  in  incomplete  traumatic  paralyses  very  soon  after  the  injury, 
and  is  then  referable  to  a  sudden  giving  way  of  the  adhesions  or  of  a 
severed  but  re-attached  tendon.  But  it  may  also  occur,  and  without 
apparent  cause,  in  paralyses  whether  traumatic  or  not,  which  have 
remained  stationary  for  a  long  time. 

In  some  cases  an  apparent  sudden  increase  in  a  paralysis  is  pro- 
duced by  the  fact  that  the  patient  fixes  with  the  paretic  eye,  allowing 
tlie  sound  eye  to  deviate.  This,  without  really  changing  the  muscular 
conditions,  causes  a  great  increase  in  the  deviation  (page  215). 

If,  as  sometimes  happens  (page  217),  fixation  is  performed  with 
each  eye  alternately,  the  deviation  will  show  a  sudden  great  increase 
when  the  paretic  eye  is  used,  and  it  will  seem,  as  the  patient  shifts  from 
one  eye  to  the  other,  as  if  the  paralysis  was  undergoing  sudden  and 
great  changes  in  amount. 

In  other  cases  still,  a  paralysis  tends  to  advance  gradually  and  either 
continuously  or  discontinuously  {progressive  paralysis).  Cases  of  this 
sort  in  which  ultimately  nearly  all  the  eye  muscles  are  involved,  occur 
in  syphilis  and  tabes. 

A  peculiar  form  of  progressive  paralysis  is  the  rare  chronic  progres- 
sive ophthalmoplegia  of  Graefe.  This  is  an  extremely  chronic  affection, 
which  often  begins  in  childhood  and  affects  very  gradually  one  muscle 
after  another  until  after  many  years  the  picture  of  a  more  or  less  com- 
plete ophthalmoplegia  exterior  is  produced.  It  may  be  arrested  in  any 
stage  of  its  course.  In  any  case  the  interior  muscles  are  exempt,  and, 
aside  from  the  ophthalmoplegia,  no  sign  of  other  disease  of  the  nervous 
system  subsequently  develops.     The  affection  is  always  bilateral. 

A  paralysis,  however  caused,  may  disappear  suddenly.  Much  more 
frequently  the  recovery  is  gradual,  and  often  enough  it  is  incomplete, 
a  more  or  less  marked  paresis — sometimes  only  a  very  slight  insuffi- 
ciency— of  one  or  more  muscles  remaining  indefinitely. 

Even  when  the  recovery  is  complete,  recurrences  may  take  place. 
Such  recurrences  may  affect  the  muscle  originally  involved,  or  some 
other  muscle  in  the  same  eye,  or  one  or  more  muscles  in  the  fellow-eye. 
They  are  observed  especially  in  tabes  and  in  syphilis,  but  maj'  also 
occur  in  paralysis  resulting  from  exposure  to  cold  and  those  caused  by 
tumors  and  aneurisms,  syringomyelia,  chronic  meningitis,  multiple 
neuritis,  chronic  progressive  ophthalmoplegia,  and  other  causes. 

Paralyses  which   disappear  and  recur  at  short,  regular  intervals 


THE  EXTRA-OCULAR  MUSCLES  243 

may  occasionally  develop  in  connection  with  malaria  and  perhaps  in 
other  conditions.  Very  peculiar  are  the  cases  described  by  Salzmann 
(cited  by  Wilbrand  and  Saenger),  and  by  Axenfeld  and  Schiirenberg, 
in  which,  with  an  oculo-motor  paralysis,  there  was  rhythmical  return  of 
power — indeed  an  actual  spasm — in  the  levator  and  in  the  interior 
muscles  every  minute  or  so.  Such  conditions  are  attributed  to  periodi- 
cal changes  in  the  blood-supply  of  the  oculo-motor  nucleus  (Fuchs), 
or,  with  more  plausibility,  to  the  intermittent  pressure  of  a  self-empty- 
ing angioma  on  the  trunk  of  the  nerve  at  the  base  of  the  brain  (Axen- 
feld and  Schiirenberg). 

A  peculiar  variety  of  recurrent  paralysis  usually  affecting  the  third, 
but  sometimes  the  fourth  or  sixth  nerves,  is  the  so-called  ophthalmo- 
plegic migraine  (Chapter  XVII).  Another  affection  characterized  by 
intermittent  recurrent  paralyses  is  Gerlier's  disease  (Chapter  X). 

In  acquired  paralysis,  particularly  when  it  is  complete  and  when 
it  has  lasted  a  long  time,  a  consecutive  contracture  of  the  antagonist  to  the 
paralyzed  muscle  commonly  develops.  Thus,  in  paralysis  of  the  ex- 
ternus,  the  internus  becomes  contractured,  causing  a  strong  deviation 
of  the  eye  inward.  In  paralysis  of  the  internus,  contracture  takes  place 
in  the  externus;  in  paralysis  of  the  superior  rectus  the  contracture 
affects  sometimes  the  inferior  rectus,  sometimes  the  superior  oblique; 
and  in  paralysis  of  the  inferior  rectus,  it  affects  either  the  superior 
rectus  or  the  inferior  oblique. 

The  effect  of  the  contracture  is  to  extend  the  deviation  and  render 
it  comitant.  That  is,  a  deviation  which  is  originally  confined  to  one 
portion  of  the  field  of  fixation  becomes  spread  over  all  portions.  Thus, 
in  paralysis  of  the  right  abducens,  there  is  originally  a  marked  inward 
deviation  which  increases  progressively  as  the  eyes  are  carried  to  the 
right,  and  diminishes  to  zero  as  the  eyes  are  carried  to  the  left.  Then 
a  consecutive  contracture  of  the  right  internus  takes  place,  and  now  the 
right  eye  being  pulled  strongly  inward  by  this  muscle,  shows  a  marked 
inward  deviation  in  the  primary  position  and  also  when  turned  to  the 
left.  Ultimately  such  a  case  cannot  be  differentiated  from  a  comitant 
strabismus  convergens  of  the  right  eye. 

So,  too,  in  a  paralysis  of  the  right  superior  rectus  the  right  eye  at 
first  simply  drops  below  the  left  and  does  so  only  when  the  patient 
looks  up,  while  when  he  looks  down  the  two  eyes  are  on  a  level.  After- 
wards a  contracture  of  the  inferior  rectus  or  the  superior  oblique  takes 
place,  and  then  the  right  eye  is  forcibly  carried  down,  so  that  it  is 
below  the  left,  not  only  when  the  eyes  are  directed  up,  but  also  when 
they  are  in  the  primary  position  and  when  they  are  directed  doAvn.     The 


244  THE    EYE    AND    NERVOUS    SYSTEM 

condition  is  thus  transformed  from  a  non-comitant  deviation  into  a 
more  or  less  comitant  vertical  squint  (strabismus  deorsumvergens) . 
Similarly  a  paralysis  of  the  superior  oblique  or  the  inferior  rectus  may 
through  contracture  of  the  opposing  elevators  produce  a  comitant  strabis- 
mus sursumvergens. 

While  contracture  of  the  opponent  is  regularly  present  in  old  ac- 
quired paralyses,  it  is  as  regularly  absent  in  congenital  paralysis.  Both 
rules,  however,  have  their  exceptions  (A.  Graefe). 

In  some  cases  the  paralysis  itself  may  disappear  and  the  contracture 
persist,  leaving  a  pronounced  spastic  deviation  (A.  Graefe). 

^lore  or  less  variable  spasm  may  occur  in  the  opponents  of  the  par- 
alyzed muscle.  Thus,  in  paralysis  of  the  inferior  rectus,  a  condition 
which  is  associated  mainly  with  diplopia  in  looking  down,  a  transient 
spasm  of  the  superior  rectus  or  inferior  oblique  may  occur,  causing 
sudden  changes  in  the  degree  of  deviation  and  producing  diplopia  in 
looking  up,  which  diplopia  will  vary  in  amount  from  day  to  day.  In 
my  belief  such  spasm  is  the  usual  forerunner  of  a  permanent  contrac- 
ture and  is  indeed  the  cause  of  it. 

Treatment  of  Paralysis. — In  treating  a  paralysis  the  first  and  fore- 
most indication  is  to  remove  its  cause.  Accordingly,  in  paralyses  due 
to  syphilis  and  the  parasyphilitic  diseases  (tabes,  general  paresis)  we 
use  large  doses  of  the  iodides  and  mercury.  In  syphilis,  where  the 
paralyses  often  occur  late  in  the  disease  and  indicate  a  deep-seated  lesion, 
which  may  be  the  immediate  precursor  of  even  more  serious  changes 
in  the  nervous  system,  treatment  should  be  pushed  very  vigorously  and 
kept  up  for  many  months.  The  earlier  such  thorough-going  treatment 
is  initiated,  the  greater  the  prospect  both  of  relieving  the  paralysis 
itself  and  of  preventing  the  development  of  other  serious  manifestations 
later  on. 

In  paralyses  due  to  exposure  to  cold,  treatment  with  diaphoretics 
and  salicylates  is  indieatod  and  is  usually  successful. 

In  paralyses  resulting  from  diphtheria,  influenza,  hysteria,  and 
neurasthenia,  tonic  treatment  should  be  employed.  Use  of  the  eyes  for 
near  work  should  be  restricted  or  forbidden  for  a  time,  at  least,  so  as 
to  give  the  central  neurons  a  chance  to  regain  their  full  activity. 

Strychnine  has  been  recommended  in  paralysis,  but  seems  of  doubt- 
ful value  excej)t  as  a  general  tonic. 

Electricity  has  been  used  with  success  by  Benedikt  (cited  by  Beard 
and  Rockwell),  by  Roy,  and  others. 

Passive  traction  (made  with  forceps  under  cocaine),  exercises  in 
moving  the  eye  (as  in  following  a  moving  object),  and  exercises  in 


THE  EXTRA-OCULAR  MUSCLES  245 

overcoming  diplopia,  either  Avith  or  without  jjrisms,  may  be  of  use  in 
partial  paralyses  and  particularly  as  an  adjuvant  when  the  paralysis 
shows  any  tendency  to  recover.  This  treatment  is  much  more  effective 
in  peripheral  (and  especially  traumatic)  paralyses  than  in  those  of 
central  origin. 

Finally,  if  the  paralysis  has  remained  stationary  for  months,  and 
there  is  no  apparent  prospect  of  a  change  taking  place  in  it,  an  opera- 
tion may  be  done.    This  consists  in 

(a)  Advancement  of  the  paretic  muscle  itself.  Done  especially  in 
partial  and  in  traumatic  paralyses. 

(6)  Advancement  of  another  muscle  in  the  same  eye,  especially  of 
the  superior  rectus  if  the  inferior  oblique  is  paralyzed. 

(c)  Tenotomy  of  the  direct  antagonist  in  the  same  eye, — e.g.,  of  the 
right  internus  when  the  right  externus  is  paralyzed.  Should  be  done 
only  in  case  there  is  well-marked  contracture  of  the  direct  antagonist. 

{d)  Tenotomy  of  the  associate  muscle  (page  194)  in  the  fellow- 
eye,  thus  of  the  left  internus,  externus,  inferior  rectus,  superior  rectus, 
inferior  oblique,  and  superior  oblique,  respectively,  in  the  case  of 
paralysis  of  the  right  externus,  internus,  superior  oblique,  inferior 
oblique,  superior  rectus,  and  inferior  rectus.  This  operation  affords 
very  good  results. 

In  many  cases  a  combinatioii  of  several  of  these  operations  may  be 
required. 

To  obviate  the  diplopia  due  to  the  paralysis  many  patients  find  it 
advantageous  to  shut  out  the  sight  of  one  eye  by  putting  a  patch  or  a 
ground  glass  before  it.     Others  find  these  devices  intolerable. 

Conditions  Allied  to  Paralysis. — Dyskinesis  is  the  condition  in 
which  the  muscles  are  not  paretic  and  are,  in  fact,  capable  of  contracting 
to  the  full  normal  extent,  yet  act  sluggishly  or  with  pain  and  difficulty. 
It  may  occur  after  any  exhausting  illness,  as  an  evidence  of  neuras- 
thenia or  hysteria,  or  as  one  of  the  symptoms  of  general  muscular  en- 
feeblement  from  any  cause.  In  some  cases  it  seems  to  occur  without  ob- 
vious cause  and  as  an  independent  affection  in  otherwise  healthy  people. 

A  peculiar  ataxia  of  the  eye-muscles  sometimes,  although  very  rarely, 
occurs  in  tabes  (Uhthoff).  If  the  atactic  movements  are  associated 
(comitant)  so  that  one  eye  acts  precisely  like  the  other,  there  will  be 
no  diplopia.  In  this  case  the  condition  is  an  ataxia  rather  of  the  ocular 
movements  than  of  the  muscles  themselves,  and  may  be  regarded  as  a 
kind  of  irregular  nystagmus.  AMien  the  atactic  movements  differ  in  the 
two  eyes,  so  that  the  eyes  matke  dissociated  movements,  a  peculiarly 
irregular  and  changeable  diplopia  will  ensue. 


246 


THE    EYE    AND    NERVOUS    SYSTEM 


Section   III. 
SPASM  OF  THE  INDIVIDUAL  OCULAR  MUSCLES. 

Symptoms. — Normally,  when  the  eyes  are  carried  in  a  given  direc- 
tion, they  move  equally  far  and  equally  fast,  because  the  two  associate 
muscles  (page  194)  chiefly  concerned  in  that  special  movement  work 
precisely  alike.  If  one  of  these  muscles  is  underacting,  the  eye  to  which 
it  is  attached  lags  more  and  more  behind  the  other  in  performing  this 
special  movement,  and  we  have  in  consequence  an  increasing  deviation, 
an  increasing  diplopia,  and  the  other  symptoms  of  paralysis  already 
noted.  Quite  the  same  thing  will  happen  if  one  of  the  associate  muscles 
is  overacting.  In  that  case  the  affected  eye  will  shoot  further  and  further 
ahead  of  the  other,  and  we  will  have  again  a  deviation  and  a  diplopia, 
increasing  as  the  eyes  are  carried  in  the  given  direction. 

Accordingly,  the  evidences  of  a  spasm  or  overaction  are  as  follows: 

1.  Excessive  Movement. — The  affected  eye  shoots  beyond  the  other 
when  the  eyes  are  carried  in  a  certain  direction,  and  its  total  range  of 
excursion  in  that  direction  is  greater  than  that  of  the  fellow-eye. 

2.  The  deviation  thus  produced,  which  can  be  made  out  by  the 
screen  and  other  tests,  is  greater  if  the  sound  eye  is  used  for  fixation 
than  if  the  affected  eye  fixes, — i.e.,  the  primary  is  greater  than  the 
secondary  deviation.     (Cf.  remarks,  page  108,  212). 

3.  Diplopia,  increasing  as  the  eyes  are  carried  in  the  direction  of 
the  field  of  action  of  the  overacting  muscles.  This  diplopia  is  precisely 
the  same  in  character  and  in  behavior  as  that  which  would  be  produced 
by  a  paralysis  of  the  associate  muscles  in  the  fellow-eye, — i.e.,  the  con- 
ditions will  be  as  shown  in  the  following  table. 


CORRKSPONDS  TO 
PARALYSIS  OP  LKPT 

IN  EITHER  CASE 

SPASM  or  RIGHT 

IF  L.   EYE 
FIXES  R. 
DEVIATES 
GREATLY 

IF  R.  EYE 

FIXES  L. 
DEVIATES 

DIPLOPIA  IS 

DEVIATION  AND 

DIPLOPIA  IN- 
CREASE AS  EYES 
ARE  CARRIED 

External  rectus. 
Internal  rectus. 
Superior  rectus. 
Inferior  oblique. 
Inferior  rectus. 
Superior  oblique. 

Internal  rectus. 
External  rectus. 
Inferior  oblique. 
Superior  rectus. 
Superior  oblique. 
Inferior  rectus. 

Out. 

In. 

Up. 

Up. 

Down. 

Down. 

Out. 

In. 

Down. 

Down. 

Up. 

Up. 

Crossed. 

Homonymous. 

Right.* 

Right.* 

Left.t 

Leftf 

ToR. 

ToL. 

Up  and  to  R. 

Up  and  to  L. 

Down  and  to  R. 

Down  and  to  L. 

NoTK. — The  conditions  obtaining  in  spasm  of  the  left  eye  (paralysis  of  the  right) 
may  be  deduced  by  substituting  right  for  left  and  vice  versa. 

*  I.e.,  vertical  diplopia  with  the  image  of  the  right  eye  below. 
f  I.e.,  vertical  diplopia  with  the  image  of  the  left  eye  below. 


THE  EXTKA-OCULAR  MUSCLES  247 

4.  False  Projection. — If  the  projection  test  is  made  in  the  manner 
indicated  on  page  210  by  having  the  patient  close  the  good  eye  and  try 
to  put  his  finger  on  an  object  situated  within  the  field  of  action  of  the 
affected  muscle  it  will  generally  be  found  that  in  a  case  of  spasm  the 
patient  undershoots  his  mark,  just  as  in  paralysis  he  overshoots  it. 
Thus,  a  patient  with  a  spasm  of  the  right  abducens  who  is  trying  to 
touch  an  object  on  his  right,  will  put  his  finger  to  the  left  of  it.  The 
reason  for  this  is  that  the  patient  underestimates  the  distance  that  the 
eye  travels,  since  he  is  aware  only  of  the  effort  that  he  is  putting  forth 
in  order  to  look  at  the  object,  and  with  an  overacting  muscle  this  effort 
is  less  than  normal. 

5.  An  apparent  movement  of  objects  looked  at  may  be  produced  by 
the  false  projection,  and  this  movement  together  with  the  diplopia  may 
cause  vertigo. 

As  will  be  seen  from  the  above,  the  appearances  presented  by  a 
spasm  of  a  muscle  in  one  eye  closely  simulate  those  of  a  paralysis  of 
the  associate  muscle  in  the  other  eye.  Indeed,  unless  the  objective 
signs  of  a  paralysis,  such  as  marked  restriction  of  movement,  are 
present,  the  diagnosis  may  be  difficult.  It  can  be  facilitated  by  the 
following  considerations : 

Points  in  Common. 
One  eye  moves  faster  and  further  than  the  other  when  both  are  carried  in  some 
■one  particular  direction;    and  both  this  discrepancy  between  the  position  of  the  eyes 
and  also  the  diplopia,  false  projection,  and  vertigo  become  more  and  more  pronounced, 
the  further  the  eyes  are  carried  in  that  direction. 

SPASM.  PARALYSIS. 

Measured  by  the  perimeter  or  tropometer,  Measured  by  the  perimeter  or  tropometer, 
the  absolute  excursion  of  the  faster  the  absolute  excursion  of  the  faster 
moving  eye  in  the  given  direction  is  moving  eye  is  normal  in  all  direc- 
greater  than  normal;  that  of  the  tions;  that  of  the  other  eye  is  sub- 
other  eye  is  normal.  normal. 

The  total  excursion  of  the  faster  moving  The  total  excursion  of  the  faster  moving 

eye  in  the  given  direction  and  in  the  eye  is  normal,  that  of  the  other  eye 

direction  opposite  is  greater  than  nor-  is   subnormal, — i.e.,   the   field   of   fixa- 

mal,— i.e.,  the  field  of  fixation  is  ex-  tion  of  the  latter  is  contracted  in  one 

cessively  large  in  one  of  its  diameters.  of  its  diameters. 

Fixation    is    usually    performed    by    the  Fixation  usually  performed  by  the  faster 

slower  moving  eye.  moving  eye. 

The  amount  of  deviation  may  show  great  The   amount   of   deviation   rarely   shows 

and  sudden  changes  from  time  to  time.  these  sudden  changes. 

If  false  projection  is  present,  it  occurs  False  projection  occurs  when  the  slower 

when  the  faster  moving  eye  is  used  for  moving  eye  is  used  for   fixation,  and 

fixation    (the  other  being  closed),  and  the  patient  overshoots  the  mark  he  is 

tlie  patient  undershoots  the  mark  he  trying  to  touch, 
is  trying  to  touch. 


248  THE    EYE    AND    NERVOUS    SYSTEM 

Etiology  of  Spasm. — Overaction  of  an  ocular  muscle  may  occur 
either  because  the  muscle  itself  is  excessively  strong  and  overdeveloped 
(structural  overaction),  or  because  the  tendon  is  inserted  so  close  to 
the  cornea  that  it  exerts  an  undue  leverage  on  the  eye  (insertional 
overaction).  A  marked  example  of  insertional  overaction  is  that  pro- 
duced by  the  operation  of  advancement. 

True  spasm  is  that  form  of  overaction  produced  by  excessive  in- 
nervation.    It  may  be  either  primary  or  secondary. 

Primary  spasm  of  the  ocular  muscles  is  rare.  A  spastic,  sometimes 
painful,  contraction  of  one  or  more  muscles  occurs  at  times  in  menin- 
gitis and  other  irritative  affections  of  the  brain.  It  has  also  been  ob- 
served as  a  result  of  reflex  irritation  due  to  decayed  teeth  (Eversbusch), 
or  to  middle-ear  disease.  Lucae  found  that  artificial  heightening  of 
the  labyrinthine  pressure  produces  a  divergent  squint  which  he  ascribes 
to  spasm  of  the  abducens.  Tonic  spasm  also  occurs  in  tetanus.  Mac- 
kenzie says  that  in  this  disease  sometimes  all  the  eye  muscles,  interior  as 
well  as  exterior,  are  thus  affected.  The  eyeball  in  this  case  is  fixed  and 
motionless,  so  that  the  condition  closely  resembles  a  total  ophthalmo- 
plegia, from  which,  indeed,  it  can  be  differentiated  only  by  the  fact  that 
there  are  miosis  and  spasm  of  accommodation. 

Spasm  also  is  occasionally  due  to  hysteria.  In  most  of  the  cases, 
however,  in  which  the  spasm  is  reported  as  affecting  the  eye  muscles,  it 
really  affected  some  one  of  the  co-ordinate  movements  of  the  eyes.  In 
particular,  the  cases  of  spasm  of  the  interni  that  have  been  reported  are 
probably  really  cases  of  spasm  of  convergence.  The  same  is  probably 
true  of  Theobald's  case  of  spastic  squint  occurring  after  influenza. 

The  spasm  that  accompanies  convulsive  attacks  is  also  usually  a 
conjugate  spasm,  affecting  the  associated  movements  rather  than  a 
single  muscle.  Gowers,  however,  records  several  cases  in  which  a 
single  muscle,  especially  the  external  rectus,  was  the  seat  of  a  true  epi- 
leptoid  attack, — i.e.,  the  muscle  was  suddenly  seized  with  tonic  or 
combined  tonic  and  clonic  spasms,  which,  after  lasting  a  short  time, 
disappeared,  leaving  the. eye  completely  normal  until  the  next  attack. 
The  seizures  were  sometimes  preceded  by  an  aura  and  were  accom- 
panied by  vertigo,  diplopia,  and  some  obscuration  of  consciousness. 

Gowers  also  states  that  slight  clonic  spasms  of  the  eye  muscles  may 
occur  in  chorea. 

Desmarres,  the  celebrated  ophthalmologist,  suffered  from  a  clonic 
spasm  of  the  superior  oblique,  due  to  a  lesion  of  the  opposite  cerebral 
hemisphere  (Landolt,  cited  by  A.  Graefe). 

Secondary  spasm  is  much  more  common  than  the  primary.     By  sec- 


THE  EXTRA-OCULAK  MUSCLES  249 

ondary  spasm  we  mean  one  which  is  the  result  of  a  paralysis  of  another 
muscle,  either  in  the  same  eye  or  in  its  fellow.  It  occurs  under  the 
following  forms: 

1.  Secondary  spasmodic  deviation  of  the  sound  eye  when  the  paretic 
eye  fixes  (page  215).  This  deviation,  as  already  shown  (page  217  and 
cf.  Table,  page  24T),  always  appears  under  the  guise  of  a  spasm  of 
the  associate  to  the  paralyzed  muscle. 

2.  Spasm  of  a  synergic  muscle  in  the  same  eye.  If  the  right 
superior  rectus  is  paralyzed,  the  extra  nervous  effort  employed  in  keep- 
ing this  eye  in  place  will  be  imparted  equally  to  its  synergic  muscle, 
the  right  inferior  oblique.  This  muscle,  in  consequence,  acts  ex- 
cessively. In  most  cases  the  effect  of  this  excessive  action  is  not  ap- 
parent, because  the  associate  muscle  in  the  left  eye  (the  superior  rectus) 
receives  a  similar  excessive  impulse.  Sometimes,  however,  the  im- 
pulse imparted  to  the  oblique  preponderates,  and  then  we  have  the 
picture  of  a  combined  paralysis  of  the  right  superior  rectus  and  spasm 
of  the  right  inferior  oblique.  That  is,  we  have  a  left  diplopia  which 
increases  in  looking  up  and  to  the  right,  and  a  right  diplopia  which 
increases  up  and  to  the  left.  This  finding  is  quite  like  that  which  we 
get  in  a  paralysis  of  both  superior  recti,  and  often  differs  only  in  the 
fact  that  the  diplopia  in  the  left  upper  field  fluctuates  markedly  in 
amount  from  one  examination  to  another. 

In  an  abducens  paralysis  we  sometimes  find  a  similar  associated 
spasm  of  the  obliques.  This  causes  the  eye  to  move  by  zig-zag  jumps 
a  little  further  out  than  the  externus  alone  can  carry  it.  If  the  eye 
is  above  or  below  the  horizontal  plane,  the  amount  of  additional  move- 
ment thus  obtained  may  be  considerable. 

3.  More  or  less  irregular  and  varying  spasm  in  the  opponents  of  the 
paralyzed  muscle  (page  244). 

4.  Permanent  spastic  contraction  {contracture)  of  the  opponent  to 
the  paralyzed  muscle  (page  243).  As  already  noted,  in  this  case  the 
paralysis  itself  may  disappear  and  the  contracture  remain.  When  this 
happens  the  picture  presented  w^ill  be  that  of  spasm,  pure  and  simple. 

Section  IV. 

CONVERGENCE  AND  DIVERGENCE  ANOMALIES.     COM- 
ITANT  HETEROPHORIA  AND   SQUINT. 

Nature  of  these  Anomalies. — The  remaining  motor  anomalies  of 
the  eye  are  not  caused  by  weakness  or  overaction  of  the  muscles,  per  se. 


250  thp:  eye  and  xervous  system 

nor  by  any  affection  of  the  nerves  supplying  them.  They  are  due 
to  involvement  of  the  centres  or  tracts  connecting  these  nerves  with 
one  another  and  with  the  cortex,  and  consequently  appear  under  the 
guise  of  either  a  deficiency  or  an  excess  of  one  of  the  co-ordinated 
movements  of  the  eyes  (convergence,  divergence,  or  parallel  move- 
ment). 

Convergence,  divergence,  and  parallel  movements  are  binocular 
acts, — i.e.,  each  eye  takes  a  precisely  equal  part  in  executing  them. 
Hence,  if  there  is  any  excess  or  deficiency  in  the  performance  of  these 
movements,  owing  to  involvement  of  the  centres  governing  them,  the 
excess  or  the  defect  will  show  itself  in  each  eye  equally.  Perversions 
of  these  functions  are  always  binocular. 

When  we  look  to  the  right  we  converge  or  diverge  our  eyes  with 
the  same  facility  as  when  we  look  to  the  left,  and  with  nearly  the 
same  facility  as  Avhen  we  look  straight  ahead.  The  deviation  pro- 
duced by  a  convergence  or  divergence  anomaly,  therefore,  will  remain 
about  the  same,  no  matter  how  the  eyes  are  turned,  provided  that  the 
object  of  fixation  is  kept  at  the  same  distance  from  the  eyes.  But 
since  generally  we  use  our  convergence  strongly  when  looking  at  near 
objects  and  do  not  use  it  when  looking  at  distance,  the  effect  of  a  con- 
vergence anomaly  in  producing  deviations  will  be  much  greater  in  near 
than  in  distant  vision.  The  opposite  will  be  the  case  with  a  divergence 
anomaly.  Convergence  and  divergence  anomalies  then  are  comitant 
in  that  the  deviation  changes  hut  little  as  the  eyes  are  carried  to  the 
right  or  left,  and  are  periodic  in  that  the  deviation  due  to  a  con- 
vergence anomaly  increases,  and  that  due  to  a  divergence  anomaly 
decreases,  as  the  object  of  fixation  approaches  the  eyes. 

Nearly  all  cases  of  periodic  comitant  heterophoria  and  squint  are 
produced  by  an  anomaly  of  convergence  or  divergence.  Nearly  all 
cases  of  continuous  comitant  squint  are  produced  by  an  anomaly  of 
convergence  combined  with  one  of  divergence. 

Convergence-excess  will  produce  an  active,  and  divergence-insuffi- 
ciency a  passive,  convergence  of  the  visual  lines  (esophoria  or  eso- 
tropia). Convergence-insufficiency  and  divergence-excess  will  pro- 
duce exophoria  and  exotropia.  Ordinary  comitant  convergent  squint 
(esotropia)  is  due  to  a  convergence-excess  combined  with  a  divergence- 
insufficiency,  and  ordinary  divergent  squint  (exotropia)  to  divergence- 
excess  combined  with  convergence-insufficiency. 

Types  of  Periodic  and  Continuous  Deviations. — The  different 
types  of  periodic  and  continuous  deviations  thus  produced  have  the 
following  characters : 


THE  EXTRA-OCULAR  MUSCLES 

PERIODIC   HETEROPHORIA    AND   SQUINT 


251 


Deviation  for  dis- 
tance by  all  tests 
(screen,  parallax, 
Maddox  rod,  pho- 
ro  meter). 

Deviation  for  near 
(by  ^11  tests). 


Convergence    near- 
point. 


Pr  J  sm-con  vergence 
(adduction. ) 


Prism-divergence 
(abduction. ) 


Primary  and  second- 
ary screen  devia- 
tion. 

Field  of  binocular 
fixation. 


Field   of   binocular 
single  vision. 


ESOPHORIA 
AND  CONVERGENT  SQUINT 


CONVERGENCE-       [         DIVERGENCE- 
EXCESS  I       INSUFFICIENCY 


Orthophoria   or 
slight  esopho- 


Esophoria 
marked  (much 
more  than  for 
distance). 


Normal  (or  ab- 
normally 
close). 


Normal. 


Normal. 


Esophoria 
marked. 


Esophoria 
slight  or  none 
(decidedly 
less  than  for 
distance). 

Normal. 


Normal. 


EXOPHORIA 
AND  DIVERGENT  SQUINT 


CONVERGENCE- 
INSUFFICIENCY 


Orthophoria  or 
slight  exopho- 
ria. 


Exophoria  very 
marked  (much 
more  than  for 
distance). 


Remote  (more 
than  7  cm. 
from  root  of 
nose ) . 

Low  and  ac- 
complished 
only  with  ef- 
fort and  dif- 
ficulty. 

Normal. 


Low   less    than     Normal.  Abnormally 

(2°-3°)     or  great  (over  9° 

negative  (i.«.,  of  prism), 

insuperable 
homonymous 
diplopia  for 
distance). 

In  all  four  types  deviation  of  one  eye  behind  the  screen,  measured 
with  prisms,  equals  precisely  that  of  the  other  eye  (distinction 
from  paretic  deviations).* 

In  all  four  types  excursions  of  each  eye  normal  in  all  directions. 
Neither  eye  lags  behind  the  other  when  both  are  making  lateral, 
vertical,  or  oblique  movements  (distinction  from  paretic  devia- 
tions). 


DIVERGENCE- 
EXCESS 


Exophoria 
marked. 


Exophoria  de- 
cidedly less 
than   for  dist- 


Normal. 


Normal. 


Homonymous  diplopia  if  present, 
not  increasing  (if  anything, 
diminishing)  as  eyes  are  carried 
to  right  and  left  (distinction 
from  paretic  deviations).  In- 
creases somewhat  in  looking 
down,  diminishes  in  looking 
up. 


Crossed  diplopia  not  increasing  as 
eyes  are  carried  to  right  and 
left  (distinction  from  paretic 
deviations).  Increases  some- 
what in  looking  up,  diminishes 
in  looking  down. 


*  In  some  few  cases  one  eye  does  deviate  more  than  the  other  when  covered.  This  seems  to 
occur  in  old  cases  of  squint,  in  which  secondary  muscular  changes  have  taken  place  in  the  squinting 
eye,  so  that  the  latter  has  in  effect  either  a  paretic  or  an  overacting  musculature.  It  also  happens 
when,  owing  to  excess  of  hyperopia  in  one  eye  or  to  some  similar  cause,  the  patient  exerts  more  accom- 
modative effort  when  seeing  with  one  eye  than  when  seeing  with  the  other.  If,  for  instance,  a  man's 
right  eye  is  much  more  hyperopia  than  his  left  he  will  accommodate  strongly  when  the  right  fixes  and 
the  left  is  screened,  and  hence  the  left  will  then  turn  strongly  in.  Then  when  the  screen  is  shifted  to 
the  right  eye  and  the  left  fixes,  less  accommodative  effort  will  be  put  forth,  and  the  right  eye  will  turn 
in  but  little  or  may  even  under  certain  conditions  turn  out  (Graefe). 


252  THE    EYE    AND    NERVOUS    SYSTEM 

CONTINUOUS  HETEROPHORIA  AND  SQUINT 


KSOPHORIA  AND  CX>NVEBGEMT  SQUINT 

"Oonvei^ence-excess  +divergence- 
iusufficiency. 


EXOPHORIA  AND  DIVERGENT  SQCINT 

=convei^ence-insuflBciency+diver- 
gence-excess. 


Deviation  for  dis-  j  Very  marked  esophoria  or  a 
tance  and  near  marked  convergent  squint  at  all 
(by  all  tests)  ranges  (somewhat  greater  for 
but  especially  near  than  for  distance  if  the  con- 
the  screen.  vergence-excess  was  the  primary 

condition,  and  greater  for  dis- 
tance than  for  near,  if  the  di- 
vergence-insuflSciency  was  the 
primary  condition). 


Convergence  near- 
point. 


Normal  or  excessively  close. 


Prism  converg-  If  it  can  be  elicited  {i.e.,  if  there 
ence  (adduc-  is  no  suppression),  normal  or 
tion).  supernormal. 


Prism    divergence 
(abduction). 


Prim  ary  and 
secondary  screen 
deviation. 

Field  of  binocular 
fixation. 

Field  of  binocular 
single  vision. 


Low.  Usually  (in  case  of  squint, 
always)  negative  (i.e.,  there  is 
homonymous  diplopia  at  all 
ranges,  so  that  no  prism,  base 
in,  is  overcome,  or  there  is  mon- 
ocular vision  with  suppression). 

As  in  periodic  deviations.  (See 
table  preceding. ) 


As  in  periodic  deviations.     (See 
table  preceding. ) 

As  in  periodic  deviations.     (See 
table  preceding. ) 


Marked  exophoria  or  marked  di- 
vergent squint  at  all  ranges 
(somewhat  greater  for  near  than 
for  distance  if  the  convergence- 
insufBciency  was  the  primary 
condition,  and  greater  for  dis- 
tance than  for  near  if  the  diver- 
gence-excess was  the  primary 
condition). 

Remote.  In  case  of  actual  squint, 
none  (patient  may  try  to  con- 
verge out  cannot  secure  binoc- 
ular fixation  at  any  distance). 

Low.  In  case  of  actual  squint 
negative  {i.e.,  there  is  either 
crossed  diplopia  at  all  ranges  so 
that  no  prism  is  overcome,  or 
there  is  monocular  vision  with 
suppression). 

If  it  can  be  elicited  {i.e.,  if  there 
is  no  suppression)  it  is  much 
greater  than  normal. 


As  in   periodic  deviations, 
table  preceding. ) 


As  in   periodic  deviations, 
table  preceding. ) 

As   in   periodic  deviations, 
table  preceding. ) 


(See 


(See 


Esophoria  and  the  Development  of  Convergent  Squint. — 1.  De- 
velopment of  Squint  from  a  Convergence-excess. — A  convergence-excess, 
as  shown  in  the  table,  has  the  following  characters: 

Esophoria  or  evidences  of  inward  deviation  by  all  tests,  pronounced 
for  near,  slight,  or  absent  for  distance;  convergence  near-point  and 
prism-convergence  normal ;  prism-divergence  normal  or  nearly  so ; 
inward  and  outward  excursions  of  the  eyes  normal,  deviation  not  in- 
creasing to  either  right  or  left. 

Convergence-excess  is  nearly  always  accommodative,  being  due  to 
the  excessive  effort  which  the  patient  makes  to  overcome  hyperopia  or 
astigmatism.     An  effort  of  accommodation   regularly  implies  a  cor- 


THE  EXTRA-OCULAR  MUSCLES  253 

responding  effort  of  convergence,  so  that  as  he  strains  one  he  also  strains 
the  other. 

A  similar  accommodative  convergence-excess  may  be  produced 
temporarily  by  the  instillation  of  atropine  or  homatropine.  The  patient 
in  this  case  tries  extremely  hard  to  use  his  failing  accommodation,  and 
makes  the  effort  to  do  so  even  when  his  accommodation  is  paralyzed. 
To  this  effort  his  accommodation  cannot  respond,  but  his  convergence 
does  so,  producing  a  temporary  marked  deviation. 

A  similar  variety  of  convergence-excess,  due  to  the  attempt  to 
employ  a  subnormal  accommodative  power,  occurs  when  the  eyes  are 
used  too  soon  after  debilitating  diseases,  as  measles,  etc. 

A  convergence-excess  may  also  be  caused  by  an  attempt  to  see 
clearly  in  conditions  of  difficult  vision  due  to  opacities  of  the  media, 
low  degrees  of  astigmatism,  or  even  a  slight  amount  of  myopia. 

In  some  cases  of  accommodative  convergence-excess  the  peculiar 
conditions  present  cause  an  esophoria  that,  contrary  to  the  general 
rule,  is  more  marked  for  distance  than  for  near.  Thus,  an  adult 
with  a  high  amount  of  hyperopia  may  succeed  in  overcoming  it  for 
distance,  but  in  overcoming  it  may  use  so  much  accommodation  as  to 
produce  also  a  considerable  degree  of  convergence,  while  for  near- 
points  he  may  be  utterly  unable  to  maintain  the  necessary  accommo- 
dation, and  hence  neither  accommodates  nor  converges. 

Occasionally  a  convergence-excess  is  met  with  in  which  no  ac- 
commodative element  is  present. 

The  use  of  prisms,  base  out,  especially  for  constant  wear,  often 
produce  a  convergence-excess  or  decidedly  aggravates  one  already 
present. 

Cases  of  true  spasm  of  convergence  (spastic  contraction  of  the 
interni  producing  a  marked  convergent  squint)  are  sometimes  seen, 
especially  in  hysteria  (Hitzig  cited  by  Graef e ;    Fuchs). 

A  convergence-excess  when  moderate  may  disappear.  This,  for  in- 
stance, happens  in  adults  who,  because  of  their  advancing  years,  no 
longer  exert  enough  accommodation  to  overcome  their  hyperopia,  and 
hence  give  up  the  effort  and  with  it  the  excessive  convergence. 

In  other  cases  a  convergence-excess,  especially  when  it  occurs  in 
childhood  and  when  of  high  degree,  tends  to  increase  and  ultimately 
develops  into  a  convergent  squint.  The  steps  by  which  this  occurs  are 
as  follows: 

(1)  A  child  with  a  good  deal  of  hyperopia  or  astigmatism,  as  soon 
as  he  begins  to  make  much  use  of  the  eyes  for  near  work,  develops,  as 
a  result  of  undue  accommodation,  a  very  varying,  evidently  more  or 


254  THE    EYE    AND    NERVOUS    SYSTEM 

less  spasmodic  esophoria,  which  is  marked  for  near  when  his  con- 
vergence is  called  into  play,  but  which  is  little  or  not  at  all  marked  for 
distance.  The  result  is  either  asthenopia,  due  to  the  troublesome 
effort  to  overcome  the  deviation,  or  else  a  confusing  condition  of  alter- 
nating diplopia  and  single  vision.  T'sually  at  this  period  there  is  still 
binocular  fixation  even  for  near,  although  every  now  and  then  when 
the  convergence  is  particularly  called  upon  (for  instance,  when  a  pencil 
at  which  the  eyes  are  directed  is  carried  quickly  toward  the  nose), 
one  of  the  eyes  may  turn  suddenly  and  sharply  in,  producing  a  momen- 
tary spasmodic  squint. 

(2)  Little  by  little  the  deviation  increases  until  binocular  fixation 
for  near  becomes  impossible,  and  the  periodic  esophoria  is  transformed 
into  a  periodic  convergent  squint.  This  at  first  is  intermittent  and 
occasional,  afterwards  more  and  more  constant.  It  will  now  be  found 
that  there  is  more  or  less  esophoria  for  distance  also,  although  always 
less  than  for  near.  There  is  still,  however,  binocular  fixation  for 
distance. 

Careful  tests  at  this  stage  will  show  in  nearly  every  case  that  the 
squint  is  associated  with  diplopia.  This  diplopia  which  presents  sud- 
den spontaneous  variations  in  amount  from  one  moment  to  another^ 
and  which  also  varies  with  the  distance  of  the  test-object  from  the  eyes, 
is  evidently  a  source  of  not  a  little  trouble  and  confusion. 

(3)  After  this  condition  has  lasted  for  a  time,  perhaps  for  some 
years,  the  patient  gives  up  binocular  fixation  for  distance,  so  that  he 
now  has  a  convergent  squint  for  both  distance  and  near  {continuous 
squint).  The  deviation  as  measured  by  the  screen,  however,  is  still 
much  more  marked  for  near  than  for  distance.  Diplopia  can  still 
usually  be  elicited  by  the  tests.  The  condition  is  still  mainly  one  of 
convergence-excess. 

(4)  Later  still,  the  amount  of  deviation  for  distance  begins  to 
increase  until  ultimately  the  squint  for  distance  equals  that  for  near- 
points,  and  both  are  absolutely  greater  than  they  were.  This  change  is 
due  to  the  superadding  of  a  divergence-insufficiency  to  the  primitive 
convergence-excess ;  the  conditions  now,  in  fact,  are  those  shown  in 
the  table  as  obtaining  for  a  convergence-excess  and  divergence-insuffi- 
ciency combined.  The  rotations  of  the  eyes  are  still  normal  in  amount, 
indicating  that  the  muscles  themselves  are  as  yet  unaffected. 

At  this  stage,  diplopia,  if  it  can  be  elicited  at  all,  no  longer  ob- 
trudes itself  upon  the  patient's  notice  or  causes  confusion.  In  many 
cases  it  cannot  be  elicited  by  any  tests,  suppression  of  one  of  the 
images  having  occurred  (page  187). 


THE  EXTRA-OCULAR  MUSCLES  255 

(5)  Later,  secondary  muscular  changes  (contracture  of  the  internus, 
stretching  and  weakening  of  the  externus)  take  place  in  the  squinting 
eje,  producing  an  absolute  reduction  in  the  rotation  outward  and  ex- 
cess of  rotation  inward.  When  this  occurs  we  can  speak  of  the  squint 
as  being  in  a  sense  monocular,  but  until  then  it  must  be  regarded  as  the 
result  of  a  strictly  binocular  process. 

2.  Development  of  Convergent  Squint  from  a  Divergence-insufficiency. — 
A  divergence-insufficiency,  as  shown  in  the  table,  has  the  following  char- 
acters : 

Marked  esophoria  and  very  low  diverging  power,  or,  often  indeed, 
insuperable  or  varying  homonymous  diplopia  for  distance ;  normal  or 
nearly  normal  relations  for  near ;  no  impairment  of  outward  nor  excess 
of  inward  rotations  of  the  eyes. 

As  a  primary  affection  it  is  rather  rare,  although  I  have  seen  several 
well-marked  instances.  Often,  especially  when  slight,  it  seems  to  re- 
main unchanged  indefinitely ;  in  other  cases  it  may  increase,  producing  a 
convergent  squint.  This  it  does  apparently  first  by  the  production  of  an 
increase  of  the  esophoria  for  distance,  then  by  the  superadding  of  a  con- 
vergence-excess, producing  an  esophoria  for  near;  the  result  is,  first  a 
periodic  convergent  squint  (esotrojDia  for  distance,  esophoria  with  bin- 
ocular fixation  for  near)  ;  later  a  continuous  squint  for  all  ranges.  Until 
late  in  their  course  such  cases  are  distinguished  from  those  starting  with 
a  primitive  convergence-excess  by  the  fact  that  the  deviation  for  distance 
always  exceeds  that  for  near. 

Some  cases  of  convergent  squint  develop  suddenly  under  the  guise 
of  a  divergence-paralysis.  This  must  be  regarded  simply  as  an  extreme 
form  of  a  divergence-insufiiciency  in  which  the  diverging  power  is  not 
simply  diminished  but  abolished.  It  is  characterized  by  homonymous 
diplopia,  which  is  very  great  in  amount  for  distance  and  diminishes 
with  mathematical  regularity  as  the  test-object  is  brought  toward  the 
eyes,  and  which,  instead  of  growing  greater,  tends  to  grow  less  as  the 
eyes  are  directed  to  the  right  or  to  the  left ;  marked  esotropia  for  dis- 
tance diminishing  progressively  as  the  object  of  vision  is  carried  toward 
the  eyes  and  finally  changing  to  orthophoria  at  some  near  point ;  no 
restriction  in  the  movement  of  either  externus  or  excess  of  action  of 
either  internus,  so  that  the  excursions  of  the  eyes  are  normal.  Vertigo 
is  often  marked  and  there  may  be  false  projection. 

Divergence-paralysis  usually  develops  suddenly.  In  some  seven  of 
the  reported  cases,  including  two  of  my  own,  organic  cerebral  lesion 
appeared  to  cause  the  difficulty,  there  being  a  history  of  syphilis,  tabes, 
multiple  sclerosis,  lead-poisoning,  or  the  evidences  of  cerebral  tumor. 


256  THE    EYE    AND    NERVOUS    SYSTEM 

Another  case  appeared  to  be  due  to  concussion  of  the  brain,  and  still 
another  to  an  antecedent  pneumonia  and  pleurisy.  The  condition  in  two 
of  Uhthoff's  cases  was  preceded  by  bilateral  abducens  paralysis,  and 
one  of  Bielschowsky's  appeared  to  go  into  a  unilateral  paresis  of  the 
abducens.  In  many  cases  the  affection  occurs  without  obvious  cause. 
Its  duration  is  uncertain,  some  cases  lasting  at  least  four  or  five  years, 
others  recovering  in  a  year  or  so. 

Exophoria  and  the  Development  of  Divergent  Squint. — 1.  Develop- 
ment of  Squint  from  a  Convergence-insufficiency. — Most  cases  of  divergent 
squint  develop  from  a  convergence-insufficiency.  This  has  the  following 
character : 

Exophoria,  marked  for  near,  slight  for  distance  and  not  increasing 
to  either  right  or  left ;  convergence  near-point  remote,  being  seven  centi- 
metres or  more  from  the  root  of  the  nose ;  prism-convergence  subnormal 
and  acquired  with  difficulty ;  prism-divergence  about  normal ;  rotation 
of  the  eyes  inward  and  outward,  normal. 

In  many  cases  the  convergence-insufficiency  is  accommodative,  that 
is,  it  occurs  in  those  who  do  not  require  to  use  their  accommodation  in 
the  normal  way.  The  accommodation  and  convergence  being  intimately 
associated,  the  disuse  of  one  means  the  disuse  of  the  other.  This  disuse 
of  the  convergence  often  shows  itself  at  first  simply  by  the  presence 
of  a  large  amount  of  exophoria  for  near  without  any  recession  of  the 
convergence  near-point,  but  later  it  develops  into  a  true  convergence- 
insufficiency. 

Accommodative  convergence-insufficiency  occurs  in  myopes  who  are 
not  using  concave  glasses,  and  may  also  occur  (from  sudden  relaxation 
of  the  accommodation)  in  hyperopes  and  presbyopes  when  putting  on 
convex  glasses  for  the  first  time.  A  somewhat  similar  relaxation  of 
the  convergence  is  found  in  those  who  for  any  reason  (presbyopia, 
marked  hyperopia,  anisometropia,  etc.)  "do  not  see  well  for  near; 
and  in  this  case  the  convergence-insufficiency  may  be  relieved  by  con- 
vex glasses. 

A  non-accommodative  convergence-insufficiency, — i.e.,  one  which 
develops  without  any  reference  to  a  refractive  error, — occurs  quite  often 
in  hysteria,  traumatic  neuroses,  neurasthenia,  anaemia,  and  debilitated 
conditions,  however  caused ;  also  in  connection  with  intranasal  disease, 
and  not  infrequently  seems  to  occur  in  those  who  are  apparently  healthy. 
According  to  Guillery  it  may  be  produced  by  poisons  (alcohol,  ether, 
chloroform,  and  chloral). 

A  convergence-insufficiency,  especially  if  slight,  may  remain  un- 
changed indefinitely;   in  other  cases,  particularly  if  of  large  amount,  it 


THE  EXTRA-OCULAR  MUSCLES  257 

develops  into  a  divergent  squint.     The  successive  stages  of  the  process 
will  then  be  as  follows : 

( 1 )  Marked  exophoria  for  near  with  at  first  a  normal  near-point  of 
convergence ;  orthophoria  for  distance  and  normal  diverging  power 
{periodic  exophoria). 

(2)  Beginning  divergent  squint  for  near  with  recession  of  the  con- 
vergence near-point;  beginning  exophoria  for  distance  with  increase 
in  the  prism-divergence  {periodic  divergent  squint).  This  squint,  at 
first  intermittent,  is  afterwards  constant. 

(3)  Marked  increase  of  the  exophoria  for  distance  with  gradual 
transformation  into  squint  (consecutive  divergence-excess)  converting 
the  periodic  into  a  continuous  squint. 

(4)  Muscular  changes  (contracture  of  the  externus,  stretching  and 
weakening  of  the  internus)  producing  absolute  impairment  of  rotation 
inward  and  excess  of  rotation  outward. 

In  some  cases  a  periodic  divergent  squint  develops  suddenly  from  a 
convergence-paralysis.  This  is  characterized  by  crossed  diplopia  and  ex- 
otropia,  which  both  increase  with  mathematical  regularity  as  the  test- 
object  is  carried  towards  the  eye.  The  deviation  and  the  diplopia  do  not 
increase  either  to  the  right  or  to  the  left.  Movements  of  each  eye  out- 
ward and  inward  are  normal. 

A  peculiar  symptom  of  convergence-paralysis  is  the  absence  of  the 
convergence-reaction  of  the  pupil.  If,  as  is  usually  the  case,  the  light 
reaction  is  present,  the  appearance  presented  is  the  converse  of  the 
Argyll-Robertson  pupil.  Accommodation  is  sometimes  diminished  or 
absent,  sometimes  normal. 

Convergence-paralysis  often  develops  suddenly  and  is  frequently 
associated  with  very  marked  vertigo  (Parinaud)  and  with  more  or  less 
false  projection  (M.  Sachs).  It  is  not  very  infrequent,  occurring  in 
tabes,  multiple  sclerosis  (in  3  per  cent,  of  the  cases),  and  probably  as  a 
result  of  other  organic  nervous  diseases.  It  is  sometimes  associated 
with  paralysis  of  divergence  or  with  paralysis  of  up  and  down  move- 
ments (Parinaud).  I  have  seen  a  marked  instance  of  the  former  com- 
bination in  a  woman  who,  at  a  distance  of  twenty  inches,  had  ortho- 
phoria and,  an  inch  or  two  further  out,  homonymous  diplopia,  increas- 
ing pari  passu  as  the  object  of  vision  was  carried  off,  while  within 
twenty  inches  she  had  crossed  diplopia,  increasing  fast  as  the  object 
was  carried  towards  the  eyes. 

A  similar  absence  of  divergence  and  convergence  power,  constituting 
what  A.  Graefe  calls  diminution  in  the  amplitude  of  fusion,  is  found 
in  multiple  sclerosis  (Uhthoff)  and  paralysis  of  the  ocular  muscles  due 
17 


258  THE    EYE    AND    NERVOUS    SYSTEM 

to  central  disease  (Soelberg-Wells).  This  condition  is  marked  by  the 
fact  that  the  patient  cannot  overcome  any  amount  of  diplopia,  however 
minute,  produced  by  prisms.  Such  inability  to  overcome  prisms,  how- 
ever, may  also  be  due  to  sudden  failure  of  the  fusion  faculty  (p.  185). 

Convergence-paralysis  may  get  well  spontaneously  or  remain  in- 
definitely. 

The  anatomical  site  of  the  lesion  is  probably  in  the  fibres  connecting 
the  non-decussating  fasciculi  of  the  two  internal  recti  nuclei  with  each 
other,  or  connecting  these  fasciculi  with  the  cortex  (Bernheimer)  ;  or 
it  may  be  cortical. 

2.  Divergent  Squint  developing  from  a  Divergence-excess. — The  charac- 
ters of  a  divergence-excess  are  as  follows : 

Exophoria  by  all  tests,  pronounced  for  distance,  slight  or  absent 
for  near;  near-point  of  convergence  and  prism-convergence  normal; 
prism-divergence  excessive,  being  over  9°  ;  movements  of  eyes  inwards 
and  outwards  normal ;  deviation  not  increasing  to  either  right  or  left. 

This  condition  often  remains  unchanged  for  a  long  period,  even  for 
years.  In  not  a  few  instances,  however,  it  develops  into  a  divergent 
squint.    The  steps  in  the  process  in  this  case  are  as  follows : 

( 1 )  Marked  exophoria  for  distance  with  excessive  prism-divergence ; 
exophoria  for  near,  little  or  none,  and  near-point  of  convergence  nor- 
mal (periodic  exophoria). 

(2)  Beginning  divergent  squint  for  distance  with  crossed  diplopia 
and  still  further  increase  in  the  prism-divergence ;  beginning  exophoria 
for  near,  but  with  convergence  near-point  still  normal  (periodic  diver- 
gent squint).     This  squint  is  at  first  intermittent,  afterwards  constant. 

(3)  Marked  increase  of  the  exophoria  for  near  and  gradual  reces- 
sion of  the  convergence  near-point  (consecutive  convergence-insuffi- 
ciency), producing  a  squint  for  near,  which,  however,  is  less  than  for 
distance), 

(4)  Marked  divergent  squint  which  is  of  about  the  same  amount 
for  both  distance  and  near  (conversion  of  the  periodic  into  a  continuous 
squint  by  the  superadding  of  a  convergence-insufficiency  to  the  primi- 
tive divergence-excess).  Diplopia  still  often  present,  but  now  usually 
negligible  by  the  patient.  The  rotations  of  the  eye  inward  and  outward 
are  still  about  normal.  The  patient  may  still  make  strong  converging 
efforts,  so  that  though  he  has  no  longer  an  absolute  he  has  still  a  rela- 
tive convergence  near-point. 

(5)  Muscular  changes  (weakening  of  the  interni ;  contracture  of 
the  externi)  causing  impairment  of  rotation  inwards  and  excess  of  rota- 
tion outwards. 


THE  EXTRA-OCULAK  MUSCLES  259 

Rationale  of  the  Development  of  Squint. — How  and  when  Squint 
develops. — A  squint  in  most  cases  apparently  arises  from  an  instinctive 
endeavor  to  avoid  diplopia  and  confusing  images.  It  seems  quite  cer- 
tain that  in  their  inception  the  different  periodic  deviations  are  accom 
panied  by  diplopia.  If  the  deviation  is  slight  the  patient  overcomes 
the  double  vision  with  ease;  if  it  is  great,  with  more  and  more  diffi- 
culty. In  the  latter  case  ultimately  he  gives  up  the  effort  to  overcome 
the  diplopia  and  then  sees  double  all  the  time. 

Xow  it  becomes  apparent  \vhen  we- study  these  cases  that  the  more 
nearly  continuous  (non -periodic)  the  deviation  is  the  easier  it  is  for 
the  patient  to  ignore  the  diplopia  to  which  it  gives  rise,  and  the  less 
pronounced,  consequently,  are  the  subjective  disturbances  which  are 
dependent  on  this  diplopia. 

Again,  it  is  almost  always  the  case  that  a  large  deviation  causes 
much  less  subjective  trouble  than  does  a  small  one:  first,  because  the 
image  projected  by  the  deviating  eye  is  so  faint  and  so  far  from  the 
other  that  the  patient  can  readily  ignore  it;  second,  because  the  devia- 
tion being  so  large  that  the  patient  cannot  possibly  overcome  it,  he  does 
not  even  make  the  attempt  to  do  so,  and  thus  escapes  the  reflex  disturb- 
ance that  such  an  attempt  often  produces.  Accordingly,  it  is  for  the 
patient's  interest  that  //  he  has  a  periodic  deviation  it  shall  he  trans- 
formed into  one  that  is  as  nearly  continuous  as  possible;  and,  second, 
that  if  it  is  small  it  shall  increase  in  amount  until  the  diplopia  is  readily 
negligible. 

As  we  have  just  seen,  both  kinds  of  change  occur  regularly  in  the 
development  of  heterophoria,  and  particularly  in  the  evolution  of  con- 
vergent and  divergent  squint. 

The  processes  by  which  these  changes  take  place  are  not  located  in 
the  muscles  themselves,  for  until  very  late  in  the  development  of  most 
cases  of  squint  the  rotations  of  the  eyes  are  normal,  indicating  that  the 
power  of  the  muscles  per  se  is  neither  impaired  nor  excessive.  The 
changes  that  take  place  are  doubtless  central,  following,  as  Hoffmann 
pointed  out,  the  law  promulgated  by  Sherrington,  that  excessive  stimu- 
lation of  the  centre  for  one  movement  produces  inhibition  of  the  centre 
for  the  opposing  movement,  and  vice  versa.  In  other  words,  in  cases  of 
convergence-excess  the  centre  for  convergent  movements  being  subjected 
to  continuous  overstimulation,  becomes  more  and  more  active,  producing 
a  continually  greater  excess  of  convergence  action  and  at  the  same  time 
causing  greater  and  greater  inhibition  of  the  centre  for  the  divergent 
movements.  The  consequence  is  that  superadded  to  the  convergence- 
excess  we  have  ultimately  a  progressive  divergence-insufficiency.      It 


260  THE    EYE    AND    NERVOUS    SYSTEM 

is  only  late  in  the  course  of  the  condition  that  the  persistent  stimulation 
of  the  intemus,  coupled  with  the  maintenance  of  one  eye  in  the  forced 
inward  position,  produces  hypertrophy  or  contracture  of  this  muscle 
and  stretching  or  atrophy  of  its  opponent. 

A  small  proportion  of  cases  of  squint  do,  however,  develop  out  of  a 
true  muscular  defect.  Thus,  some  cases  of  convergent  squint,  both  in 
childhood  and  in  adult  life,  develop  out  of  a  primitive  paralysis  of  the 
abducens  which  has  been  converted  into  a  comitant  deviation  by  con- 
secutive overaction  and  contracture  of  the  intemus  (page  243).  Fur- 
thermore, a  convergent  squint  due  to  a  convergence-excess  may  be 
aggravated  by  the  presence  of  a  primitive  weakness  of  the  externi  or 
overdevelopment  of  the  interni.  Again,  a  divergent  squint  may  be 
produced  by  developmental  changes  in  the  orbit  causing  a  relative  pre- 
ponderance of  the  externi  (page  221),  or  by  weakness  of  tlie  intemus 
due  to  operation.  Such  factors,  however,  play  a  very  subordinate  part 
in  the  production  of  squint. 

Conditions  favoring  the  Development  of  Squint;  Period  at  which  it  de- 
velops.— The  development  of  a  convergent  squint  out  of  a  heterophoria 
in  the  way  just  outlined  is  much  more  apt  to  occur  if  there  is  any  con- 
dition such  as  anisometropia,  opacities  of  the  media,  fundus  disease, 
congenital  anomalies,  etc.,  that  renders  the  sight  of  one  eye  much  worse 
than  that  of  the  other.  In  such  a  case  the  image  of  the  worse  eye  often 
confuses  that  of  its  fellow  instead  of  reinforcing  it,  and  hence  the  patient 
really  sees  better  when  he  allows  the  worse  eye  to  deviate.  In  many 
cases  it  is  apparent  that  he  actually  forces  it  to  deviate  so  as  to  get  the 
disturbing  image  out  of  the  way.  This  is  particularly  the  case  in  diver- 
gent squint. 

A  factor  which  may  be  of  considerable  importance  and  which,  in- 
deed, by  many  is  regarded  as  the  predominating  element  in  the  pro- 
duction of  squint  is  the  lack  of  development  of  the  fusion-faculty  (page 
185).  The  effect  of  this  would  be  that  the  patient  would  have  np  ten- 
dency to  blend  the  two  retinal  images,  and  hence  would  have  no  spe- 
cial motive  for  maintaining  binocular  fixation.  Consequently,  if  there 
was  a  deviation  of  the  eyes,  he  would  not  try  to  overcome  it,  but  would 
allow  one  of  them  to  squint. 

Ill-development  of  the  fusion-faculty  and  the  squint  that  it  produces 
may  be  hereditary.  Some,  indeed,  go  so  far  as  to  regard  heredity  as 
the  main  factor  in  the  genesis  of  squint  This  view,  I  think,  is  not 
substantiated  by  the  facts. 

A  convergent  squint  usually  develops  in  early  childhood.  Once 
developed,  it  increases  to  a  certain  point,  then  generally  remains  un- 


THE  EXTRA-OCULAR  MUSCLES  261 

changed  through  life.  In  not  a  few  cases,  however,  it  diminishes  in 
later  childhood,  and  may  even  disapjiear  altogether  or  be  converted  into 
a  divergent  squint.  These  changes  are  probably  due  to  developmental 
processes  (divergence  of  the  orbits  during  the  growing  period),  which 
tend  to  render  the  power  of  the  extemi  greater  and  that  of  the  intemi 
less  (see  Weiss,  page  221). 

Squint  develops  very  often  in  congenital  spastic  paralysis  (Little's 
disease). 

Divergent  squint  generally  occurs  later  in  life  and  is  more  often 
accompanied  by  persistent  diplopia  than  is  convergent  squint.  It  some- 
times develops  after  a  tenotomy  done  for  convergent  squint,  and  this 
may  occur  even  years  after  the  operation.     This  secondary  esotropia 


is  traceable  sometimes  to  an  insufficiency  of  the  internus  produced  by 
the  tenotomy;  more  often  it  is  not  attributable  to  the  latter,  being  due 
either  to  the  developmental  changes  (divergence  of  the  orbits)  above 
mentioned,  or  to  the  supervention  of  a  true  divergence-excess. 

Convergent  squint  occurs  about  three  times  as  often  as  divergent 
squint.*  In  anisometropia,  however,  divergent  squint  is  the  more  fre- 
quent form. 

Squint  a  Bilateral  Affection. — In  squint  the  patient  appears  simply  to 
fix  with  one  eye  and  to  turn  the  other  inwards  or  outwards.  In  reality, 
however,  both  eyes  are  turned.     Thus,  as  shown  in  Fig.   15,  when  a 

*  The  disproportion  is  greater  than  this  if  we  judge  from  most  hospital  sta- 
tistics, but  this  probably  arises  from  the  fact  that  slight  degrees  of  divergent  squint 
are  frequently  overlooked. 


262  THE    EYE    AND    NERVOUS    SYSTEM 

patient  with  convergent  squint  is  told  to  look  at  the  object  A,  both  eyea 
tend  to  converge  equally,  so  that  they  really  look  at  some  nearer  point, 
like  B;  then,  in  order  to  get  the  image  of  A  on  the  fovea  of  at  least 
one  eye,  the  patient  turns  both  eyes  to  one  side — say  to  the  right — 
through  the  equal  angles  BRA  and  BLD.  The  right  eye,  R,  is  now 
directed  towards  A  or  fixes  it,  the  left  eye  squints  in  by  the  angle 
ALD=2XBLD.  Thus,  here  really  the  squint  is  bilateral,  each 
eye  actually  turning  in  by  an  equal  amount,  yet  apparently  the 
left  eye  alone  squints,  because  the  patient  happens  to  use  the  right  for 
fixation. 

Which  eye  the  patient  actually  uses  to  fix  with  depends  usually  upon 
the  vision.  If  he  sees  much  better  with  one  eye  than  with  the  otlier  he 
will  fix  with  the  better  and  squint  with  the  worse  eye ;  the  squint  will 
then  be  unilateral.  Tf  the  two  eyes  are  about  alike  in  vision  and  refract- 
ing power,  we  often  find  an  alternating  squint,  the  patient  using  either 
eye  indifferently  for  fixation  and  squinting  with  the  other.  In  some 
cases,  particularly  in  anisometropia,  the  patient  may  fix  with  one  eye 
for  distance  and  with  the  other  for  near. 

Hyperphoria  and  Vertical  Squint. — Hyperphoria  in  a  great  many 
instances  is  caused  by  paresis,  or  at  least  insufficiency  of  one  of  the 
elevator  or  depressor  muscles  of  the  eye.  In  this  case  the  deviation  and 
its  evidences  (vertical  diplopia,  etc.)  increase  and  decrease  character- 
istically in  looking  in  some  particular  direction  of  the  gaze  (page  215). 
Hyperphoria  of  high  degree  and  real  vertical  squint  (hypertropia  and 
hypotropia)  are  almost  always  of  this  origin,  being,  therefore,  non- 
comitant  deviations. 

In  some  cases,  even  when  thus  originating,  a  hyperphoria  or  vertical 
squint  may  become  nearly  comitant  by  virtue  of  consecutive  contracture 
of  the  opponents  of  the  paretic  muscles  (page  243), 

In  a  few  instances  even  a  very  high  degree  of  hyperphoria  or  a  true 
vertical  squint  may  be  truly  comitant  from  the  start, — i.e.,  we  can  find 
no  evidence  of  insufficiency  of  any  elevator  or  depressor  muscle.  Hyper- 
phorias of  low  degree  are  usually  comitant. 

Some  cases  of  hyperphoria  seem  to  be  spasmodic  in  origin,  changing 
rapidly  and  lasting  only  a  short  time.  Sometimes  a  spasmodic  hyper- 
phoria of  this  sort  seems  to  be  due  in  some  way  to  accommodative  action, 
since  it  disappears  apparently  as  tlie  result  of  the  correction  of  a  re- 
fractive error.  In  most  cases  hyperphoria  once  developed  lasts  indefi- 
nitely. It  does  not,  as  a  rule,  tend  to  increase,  except  in  the  form  due 
to  marked  paralysis  of  an  elevator  or  depressor  where  the  deviation  is 
often  exaggerated  as  time  goes  on,  either  by  secondary  contractures  of 


THE  EXTRA-OCULAR  MUSCLES  263 

the  opposing  muscles  or  by  a  secondary  deviation  in  the  good  eye  due 
to  fixation  with  the  one  that  is  paretic  (page  215). 

Symptoms  of  Heterophoria  and  Squint. — These  are — 

1.  Diplopia. — This,  especially  when  slight  so  that  the  double  images 
are  close  enough  together  to  confuse  each  other,  produces  blurring  of 
sight  and  sometimes  vertigo. 

2.  Reflex  Symptoms  induced  by  the  Effort  made  to  correct  the  Devia- 
tion.— These  are  asthenopia,  pain  in  the  eyes,  conjunctival  irritation, 
headache,  which  may  be  supraorbital,  orbital,  temporal,  or  occipital, 
and  other  pains  situated  in  various  parts  of  the  body.  Occasionally, 
also,  we  find  spasm  in  the  facial  muscles,  and  sometimes  other  forms 
of  spasm;  not  infrequently  also  digestive  disturbances,  nausea,  and 
interference  with  the  general  nutrition. 

Reflex  symptoms  are  especially  pronounced  when  the  deviation  is 
so  slight  that  the  patient  can  overcome  it,  and  consequently  does  so, 
although  with  effort.  They  are  particularly  marked  when  the  deviation 
is  inconstant  and  varies  under  different  conditions,  because  in  this  case 
the  patient  cannot  as  readily  allow  for  the  changing  position  of  the 
eyes  as  he  can  when  the  deviation  is  the  same  all  the  time.  Conse- 
quently, reflex  disturbances  are  greater  in  heterophoria  than  they  are 
in  squint  and  greater  in  a  periodic  squint  than  they  are  in  one  which  is 
continuous.  Indeed,  as  we  have  seen,  this  very  fact  seems  to  constitute 
the  reason  why  a  periodic  is  regularly  converted  into  a  continuous 
squint. 

Both  the  tendency  to  diplopia  and  the  reflex  disturbances  caused 
by  muscular  anomalies  increase  in  proportion  to  the  difficulty  that  the 
patient  meets  with  in  maintaining  binocular  fixation.  They  are  hence 
greater  under  conditions  demanding  very  accurate  and  very  sustained 
fixation, — ^greater,  for  instance,  when  the  patient  is  doing  continuous 
fine  work  or  when  he  is  looking  steadily  at  brightly  illuminated  or 
sharply  defined  objects,  particularly  when  such  objects  are  in  motion. 
Hence,  many  persons  with  moderate  muscular  anomalies  always  suffer 
from  headache  and  fatigue  (panorama  headache,  panorama  asthenopia) 
after  shopping  or  going  to  the  theatre,  or  after  seeing  a  ball-match  or 
a  street-parade. 

The  most  marked  and  disagreeable  symptoms  are  found  in  hyper- 
phoria and  in  divergence-insufficiency.  In  these  conditions  the  symp- 
toms are  usually  most  troublesome  in  distant  vision. 

In  convergence-insufficiency  the  commonest  symptoms  are  asthen- 
opia, headache,  and  conjunctival  irritation,  and  these  are  developed 
generally  by  near  icorJi-.     They  are  much  more  apt  to  occur  in  the  non- 


264  THE    EYE    AND    NERVOUS    SYSTEM 

accommodative  than  in  the  accommodative  form  of  convergence-insuflB- 
ciency. 

Treatment  of  Heterophoria  and  Squint. — 1.  Correction  of  Refrac- 
tion.— This  is  a  very  important  element  in  all  of  these  anomalies  and 
may  alone  suffice  to  effect  a  cure,  particularly  when  the  deviation  is 
still  of  the  periodic  type.  It  is  of  special  importance  and  efficacy  in 
convergence-excess,  particularly  convergence-excess  which  is  developing 
into  squint.  In  this  condition  it  is  necessary  to  correct  the  whole  of 
the  hyperopia  and  astigmatism  present.  Similarly,  in  convergence- 
insufficiency  accompanying  myopia,  it  is  of  great  importance  to  make 
the  patient  wear  a  glass  fully  correcting  his  error  and  for  near  as  well 
as  for  distance. 

In  cases  in  which  the  deviation  is  no  longer  periodic, — i.e.,  in  which, 
in  addition  to  a  convergence  anomaly,  there  is  superadded  an  anomaly 
of  divergence, — correction  of  the  refraction  is  less  apt  to  afford  a  cure, 
and  in  the  case  of  a  continuous  squint  very  rarely  does  more  than 
diminish  the  amount  of  the  deviation.  In  any  case,  the  effect  of  cor- 
rection of  refraction  is  rarely  an  immediate  one;  it  usually  takes  some 
months — perhaps  a  year  or  two — for  the  full  effect  of  the  glasses  to  be 
secured. 

2.  General  treatment  must  be  employed  in  neurasthenia  and  hysteria 
and  in  conditions  of  debility  from  whatever  cause.  This  will  often 
relieve  the  symptoms  without  itself  producing  any  material  change  in 
the  muscular  condition  of  the  eyes. 

3.  Treatment  of  Associated  Conditions. — The  treatment  of  other  affec- 
tions, particularly  intranasal  disease,  which  may  possibly  be  sources 
of  reflex  trouble,  should  never  be  neglected. 

4.  Exercises  in  moving  the  Eyes. — Exercises  of  the  eyes  in  converging 
on  a  pencil  carried  towards  the  nose  or  in  overcoming  prisms,  base  out, 
are  useful  in  convergence-insufficiency  and  occasionally  in  divergence- 
excess. 

5.  Orthoptic  Exercises. — With  a  squint  that  develops  in  early  life 
much  can  be  done  by  educating  the  fusion  faculty.  This  can  be  done 
with  the  stereoscope,  or,  better  still,  with  Worth's  amblyoscope  (Fig. 
8).  Both  of  these  instruments,  by  compelling  the  eyes  to  act  together, 
train  the  fusion-faculty.  Both  are  at  first  adjusted  for  the  patient's 
angle  of  squint,  then  by  successive  adjustments  the  patient  is  gradually 
taught  to  look  into  the  instruments  with  the  eyes  more  and  more  nearly 
straight  and  yet  maintaining  fusion.  In  this  way  the  angle  of  squint  is 
steadily  diminished,  and  there  is  finally  orthophoria. 

Another  orthoptic  exercise  is  bar-reading.     This  consists  in  reading 


THE  EXTKA-OCULAE  MUSCLES  265 

with  a  bar  held  between  the  eyes  and  the  print.  Unless  the  patient  is 
using  both  eyes  at  once  for  seeing,  the  bar  will  hide  some  of  the  letters. 
The  use  of  the  bar  is  an  exercise  in  binocular  vision. 

The  squinting  eye  can  also  be  exercised  either  by  bandaging  the 
good  eye,  or,  better  still,  by  keeping  the  latter  for  some  weeks  under 
atropine. 

6.  Prisms  for  Wear. — Deviations  of  low  degree  may  be  corrected  by 
prisms  worn  constantly,  either  alone  or  combined  with  the  glass  cor- 
recting the  refraction.  This  is  most  serviceable  in  hyperphoria.  In 
esophoria  and  exophoria,  prisms  constantly  worn  often  tend  to  increase 
the  deviation,  and  their  use  is  not  generally  advisable.  Moreover,  it  is 
not  usually  possible  for  a  patient  to  wear  prisms  of  3°,  or  at  most  4°, 
before  one  eye,  so  that  the  total  amount  of  deviation  that  we  can  correct 
by  this  means  would  not  be  more  than  7°  at  most. 

7.  Operation. — When  everything  else  has  been  tried  and  has  failed, 
an  operation  is  indicated. 

With  an  esophoria  or  convergent  squint,  tenotomy  of  one  or,  better, 
of  both  internal  recti  is  done,  provided  the  condition  is  mainly  one  of 
convergence-excess.  WTien  the  condition  is  one  of  divergence-insuffi- 
ciency, the  better  plan  is  advancement  of  one  or  both  externi. 

Similarly,  in  exophoria  or  divergent  squint  caused  by  divergence- 
excess,  tenotomy  of  one  or,  better,  of  both  external  recti,  is  indicated. 
Cases  of  convergence-insufficiency,  on  the  other  hand,  should  be  treated 
by  advancement  of  the  interni.  In  marked  divergent  strabismus  tenot- 
omy of  one  or  both  externi,  combined  with  advancement  of  the  interni, 
usually  has  to  be  done. 

In  hyperphoria,  if  non-comitant,  we  proceed  according  to  the  rules 
laid  down  for  operative  interference  in  paretic  deviations  (page  245). 
In  a  comitant  hyperphoria  the  best  operation  is  a  tenotomy  of  the 
superior  rectus,  which  should  never  be  pushed  so  far  as  to  render  the 
muscle  paretic  nor  so  far  as  to  produce  even  a  moderate  overcorrection. 

SECTioisr  V. 

DERANGEMENTS  OF  THE  ASSOCIATED  PARALLEL 
MOVEMENTS   OF  THE   EYES. 

The  derangements  of  the  associated  parallel  movements  of  the  eyes 
comprise — 

A,  Paralysis  and  spasm  of  the  parallel  movements  (conjugate 
paralysis,  conjugate  spasm). 

B.  Tremor  and  ataxia  of  parallel  movements  (nystagmus,  pseudo- 
nystagmus). 


266  THE    EYE    AND    NERVOUS    SYSTEM 

CoNJUGATK  Paralysis  and  Spasm. 

Nature  of  these  Anomalies. — We  not  infrequently  meet  with  cases 
in  which  the  ability  of  the  eyes  to  perform  some  parallel  movement  is 
impaired  or  abolished  (conjugate  paralysis).  Thus,  both  eyes  may  move 
from  left  to  right  as  far  as  the  middle  line,  and  then  stop  short,  refusing 
to  go  any  farther  (paralysis  of  dextroversion).  In  this  case  commonly 
the  two  eyes  and  sometimes  also  the  head  are  deviated  in  the  opposite 
direction,"*^ — i.e.,  show  a  conjugate  deviation  to  the  left.  This  conjugate 
deviation  in  a  paralysis  of  the  right-rotators  is  caused  by  an  overaction 
of  the  antagonists,  or  left-rotators.  Such  overaction  is  probably  central 
in  origin,  since  Sherrington's  experiments  seem  to  indicate  that  abolition 
of  function  in  the  nerve-centre  presiding  over  any  movement  is  regularly 
associated  Avith  exaltation  of  function  in  the  centre  that  presides  over 
the  opposing  movement. 

In  some  cases  conjugate  deviation  is  due  not  to  a  conjugate  paralysis, 
but  to  a  conjugate  spasm.  For  example,  we  may  find  both  eyes  deviated 
to  the  left,  not  because  the  right-rotators  are  paralyzed,  but  because  the 
left-rotators  are  in  a  state  of  primary  spastic  contraction.  Such  a  con- 
jugate spasm  results  from  an  irritative  lesion  of  the  centre  for  left- 
rotation,  just  as  a  conjugate  paralysis  results  from  a  destructive  or 
inhibiting  lesion  of  the  centre  for  right  rotation.  Tt  is  distinguished 
from  paralysis  by  the  fact  that,  although  strongly  deviated  in  one  direc- 
tion, the  eyes  can  usually  be  moved  more  or  less  freely  in  the  direction 
opposite. 

Symptoms. — Since  the  deviation  is  alike  in  the  two  eyes,  conjugate 
paralysis  and  spasm  do  not  cause  diplopia.  They  may,  however,  cause 
vertigo  and  false  projection  (A.  Graefe;  M.  Sachs).  In  a  conjugate 
deviation  to  the  left,  due  to  paralysis,  the  patient  would  overshoot  the 
mark  when  trying  to  touch  an  object  situated  on  his  right,  while  if  the 
deviation  were  due  to  spasm  he  would  undershoot  the  mark  when  trying 
to  touch  an  object  situated  on  his  left. 

Very  frequently  in  cases  of  conjugate  deviation  any  symptoms  that 
may  be  occasioned  by  the  eye  trouble  are  masked  by  the  grave  general 
symptoms  due  to  the  causal  disease. 

Course. — Conjugate  paralysis  occurs  in  all  degrees  of  intensity, 
being  sometimes  very  slight,  sometimes  complete.  It  usually  develops 
suddenly,  and  is  often  transient.  The  conjugate  deviation  which  the 
paralysis  produces  often  disappears  long  before  the  paralysis  itself. 
This  is  especially  true  of  cerebral  disease.     Here  the  conjugate  devia- 

•  In  rare  cases  the  head  may  be  deviated  in  one  direction  and  the  eyes  in  the 
direction  opposite   (alternating  conjugate  deviation  of  Bresler). 


CONJUGATE   PARALYSIS   AND   SPASM  267 

tioii,  which  is  quite  pronounced  during  the  period  of  coma,  often  dis- 
appears within  a  fcAv  hours,  and  rarely  lasts  more  than  three  or  four 
weeks,  although  when  it  does  go  it  generally  still  leaves  a  partial  con- 
jugate paralysis  behind. 

Non-paralytic  Restriction  of  Conjugate  Movements;  Diagnosis 
from  True  Paralysis. — A  conjugate  paralysis  must  be  differentiated 
from  the  condition  in  which  the  patient  can  move  the  eyes  in  the  given 
direction,  but  does  not  move  them,  either  from  wish  to  deceive  or  from 
fear  of  pain,  or  because  he  really  does  not  know  how  to  make  the  effort. 
In  some  cases,  according  to  Gunn,  the  patient,  when  told  to  look  to  one 
side,  or  up,  or  doA\ai,  seems  unable  to  initiate  the  movement  of  his  own 
will,  although  he  can  follow  a  slowly -moving  object  that  is  carried  in  the 
^iven  direction.  In  other  cases  he  cannot  even  do  this,  and  yet  the  power 
of  movement,  although  apparently  never  exerted,  is  really  there,  as  we  can 
prove  by  Graefe's  test.  This  test  is  best  performed  by  making  the  patient 
look  at  a  distant  object,  and  then  placing  a  prism  of  10°  or  20°  before 
■each  eye,  with  the  apex  directed  in  the  way  in  which  we  wish  the  eyes 
to  move  {e.g.,  to  the  right  in  paralysis  of  dextroversion,  etc.).  If  the 
patient  really  has  a  conjugate  paralysis,  his  eyes  will  move  neither 
when  the  prisms  are  put  before  them  nor  when  they  are  taken  away. 
If,  however,  he  can  move  the  eyes,  we  shall  see  each  make  a  dis- 
tinct jump  in  the  direction  of  the  prism-apex  when  the  prisms  are 
put  on,  and  a  distinct  jump  in  the  opposite  direction  when  they  are 
taken  off. 

More  or  less  allied  to  conjugate  paralysis  is  the  curious  condition 
of  inertia  of  the  ocular  movements  described  by  Gowers  as  occurring 
in  a  case  of  progressive  muscular  atrophy.  The  patient  when  gazing 
at  one  object  was  told  to  look  at  another  situated  45°  to  the  right  or 
left.  lie  turned  his  head  so  as  to  front  the  second  object,  but  kept  his 
•eyes  still  fixed  on  the  first,  and  only  after  a  time,  and  slowly  then,  trans- 
ferred his  gaze  from  this  object  to  the  one  he  was  told  to  look  at. 

Varieties  of  Conjugate  Paralysis  and  Spasm. — 1.  Paralysis  of  Side- 
to-Side  Movements  (Lateriversion). — Paralysis  of  a  side-to-side  move- 
ment {e.g.,  of  the  conjugate  movement  to  the  right)  occurs  under  the 
following  forms: 

(A)  Neither  eye,  when  attempting  to  follow  an  object  that  is 
moving  to  the  right,  can  move  much  or  at  all  beyond  the  middle  line 
(paralysis  of  dextroversion,  right  and  left).  But  the  left  eye  can 
move  to  the  right  when  converging  on  an  object  which  is  brought  in 
towards  the  eyes  in  the  median  plane  (retention  of  convergence).  The 
condition   here   is,   then,    a   pure   paralysis  of   lateriversion,   and   the 


268  THE    EYE    AND    NERVOUS    SYSTEM 

appearances  it  presents  are,  it  will  be  seen,  the  precise  opposite  of  those 
that  obtain  in  a  paralysis  of  convergence  (page  257). 

(B)  Neither  eye  can  follow  an  object  that  is  carried  to  the  right, 
provided  both  eyes  are  open,  but  the  left  eye  can  follow  the  object  to 
the  right  if  the  right  eye  is  covered.  This  is  really  the  same  case  as 
A,  the  movement  inwards  of  the  left  eye  when  the  right  eye  is  covered 
being  simply  a  movement  of  convergence  (Jeffries,  Mobius). 

Two  unusual  cases  of  this  type  have  been  reported  in  which  there 
was  paralysis  of  the  movements  of  both  eyes  to  the  right,  provided 
both  eyes  were  open,  but  in  which  the  right  as  well  as  the  left  eye  could 
move  to  the  right  when  separately  tested.  In  one  case  (Samelsohn's) 
each  eye  could  move  to  the  right  if  the  eye  itself  was  screened ;  in  the 
other  case  (that  of  Stevens  of  Denver)  each  eye  could  move  to  the 
right  if  the  other  eye  was  screened.  In  both  cases  the  individual 
movement  of  the  right  eye  to  the  right  was  probably  a  movement  of 
active  divergence  (page  196)  just  as  the  movement  of  the  left  eye  to  the 
right  was  one  of  active  convergence. 

(C)  The  right  eye  can  follow  an  object  that  is  moving  to  the  right, 
but  the  left  cannot.  The  left  eye,  however,  as  in  type  A,  can  move 
to  the  right  in  performing  convergence.  This  condition,  which  may 
be  called  conjugate  hemiparalysis  (paralysis  of  left  dextroversion),  is 
distinguished  from  a  paralysis  of  the  left  intemus  by  the  fact  that  in 
the  latter  the  left  eye  could  move  inwards  neither  when  converging  nor 
when  attempting  to  make  a  conjugate  movement  to  the  left. 

(D)  Neither  eye  can  follow  an  object  that  is  carried  to  the  right, 
and  the  left  eye,  moreover,  cannot  move  to  the  right  when  attempting 
to  converge  (combined  paralysis  of  lateriversion  and  convergence). 

Paralysis  of  lateriversion  is  sometimes  bilateral, — i.e.,  both  eyes 
fail  to  follow  an  object  that  is  carried  either  to  the  right  or  to  the  left, 
although  it  may  be  that  both  can  still  converge. 

2.  Spasm  of  lateriversion  occurs  under  the  following  forms : 

(A)  Both  eyes  arc  deviated  spastically  to  one  side,  but  can  be 
moved  freely  in  all  directions  (pure  spasm  of  lateriversion). 

(B)  Both  eyes  are  deviated  spastically  to  one  side,  and  cannot  be 
moved  to  the  side  opposite  (excessive  spasm  of  one  side-to-side  move- 
ment, or  spasm  of  one  side-to-side  movement  combined  with  paralysis 
of  the  movement  in  the  opposite  direction). 

3.  Paralysis  of  Up-and-Down  Movements. — Paralysis  of  the  move- 
ment of  both  eyes  upwards  (sursuni version)  or  downwards  (deorsum- 
version)  is  uncommon.  Cases  are  reported  by  Nieden,  Parinaud, 
Gowers,  Sauvineau,  A.  Graefe,  M.  Sachs,  Snell,  Korniloff,  Posey,  etc. 


CONJUGATE   PARALYSIS   AXD   SPASM 


269 


It  is  probable  that  some  of  the  cases  reported  are  spurious,  the 
power  of  movement  being  present  but  never  put  forth.  This  was  so 
in  one  of  my  own  cases,  in  which  repeated  examinations  extending  over 
several  years  always  showed  complete  inability  of  the  eyes  to  go  up- 
wards above  the  horizontal  plane,  and  yet  Graefe's  test  (page  267) 
proved  that  they  could  really  turn  up  at  least  30°,  and  subsequently 
the  patient  regained  the  power  of  spontaneous  movement. 

Paralysis  of  sursumversion  and  deorsumversion  sometimes  occur 
together,  the  eyes  being  unable  to  move  either  up  or  do\ATi  (Lang  and 
Fitzgerald,  Wernicke,  Graefe,  Uhthoff),  and  both  conditions  are  often 
associated  with  paralysis  of  convergence  (in  one  of  my  cases  apparently 
with  paresis  of  divergence). 

4.   Spasm  of  up  or  down  conjugate  movements  may  occur,  as  in  the 


Fig.  16. — Paralysis  of  sursumversion  and  of  converg^ence.    Case  of  Dr.  Posey. 


case  reported  by  Frost,  in  which  both  eyes  were  directed  down  and 
to  the  right  and  remained  rigidly  fixed  in  this  position.  Or,  as  in  a  case 
reported  by  me,  there  may  be  an  intermittent  clonic  spasm  of  the  oblique 
movements,  so  that  the  eyes  at  intervals  darted  obliquely  far  up  and 
to  the  right  and  returned  more  slowly  to  the  primary  position.  Such 
a  condition  occupies  an  intermediate  place  between  true  spasm  and 
nystagmus  (Compare  Chapter  IX). 

Site  of  Lesion  in  Conjugate  Paralysis  and  Spasm. — Paralysis  and 
spasm  of  lateriversion  may  be  produced  by  lesions  in  the  cerebrum,  cere- 
bellum, or  pons. 

Cerebral  lesions,  especially  those  of  sudden  onset  and  associated  with 
apoplectoid  symptoms  (coma,  hemiplegia),  often  cause  paralysis  of 
lateriversion.     A  lesion  of  the  right  hemisphere  produces  paralysis  of 


270  THE    EYE    AND    NERVOUS    SYSTEM 

movements  to  the  left,  and  consequently  a  conjugate  deviation  to  the 
right.  Or  as  Prevost  puts  it,  the  patient  furim  his  eyes  towards  the 
lesion. 

The  lesion  may  be  situated  at  various  portions  of  the  cortex,  or 
anywhere  in  the  subcortical  association  paths,  or  in  the  tracts  connect- 
ing the  cortex  with  the  third  and  sixth  nerve  nuclei. 

Sometimes  a  cerebral  lesion  produces  a  spasm  of  lateriversion.  In 
this  case,  if  situated  on  the  right  side,  it  will  cause  a  spastic  deviation 
to  the  left, — i.e.,  the  patient  consequently  turns  his  eyes  away  from  the 
lesion. 

According  to  Bernheimer,  a  spastic  conjugate  deviation,  if  due  to 
cerebral  disease,  occurs  only  in  cortical  lesions  (e.g.,  in  meningitis  of 
the  convexity  of  the  brain). 

Conjugate  deviations  due  to  cerebral  disease  are  usually  most  pro- 
nounced during  the  period  of  coma,  and,  after  this  has  passed  off,  gener- 
ally disappear  pretty  soon — within  a  few  days  or  at  most  a  few  weeks, 
sometimes  within  a  few  hours.  Another  distinguishing  point  is  that 
in  a  paralysis  of  right  or  left  movements  of  cerebral  origin  the  con- 
vergence is  always  retained  (Bernheimer),  so  that  the  picture  presented 
is  that  of  a  pure  paralysis  of  lateriversion  (type  A,  page  267). 

Conjugate  deviation  is  occasionally  produced  by  abscess  of  the  cere- 
bellum, the  eyes  being  turned  sometimes  towards  the  lesion,  usually  away 
from  it  (Eversbusch). 

Disease  of  the  pons  often  causes  paralysis  of  lateriversion,  and 
if  on  the  right  side  of  the  pons  will  produce  paralysis  of  right-hand 
movements,  and  rice  rersa, — i.e.,  the  eyes  looh  away  from  the 
lesion. 

Small  lesions  in  the  pons  involving  the  abducens  nucleus  or  its 
immediate  connections  cause  paralysis  of  lateriversion  without  paralysis 
of  convergence  (paralysis  of  types  A  and  B,  pages  267,  268).  Lesions 
high  in  the  pons  involving  only  the  fibres  connecting  the  abducens  and 
the  oculo-motor  nucleus  produce  a  conjugate  hemiparalysis  (paralysis 
of  the  opposite  internus  for  parallel  movements  but  not  for  convergence, 
(type  C,  page  268).  Large  lesions  in  the  jwns  above  and  below  the 
abducens  nucleus  cause  combined  paralysis  of  lateriversion  and  con- 
vergence (paralysis  of  type  D,  page  268),     (Jeffries,  Schooler.) 

Very  rarely  (Schoeler;  Eichhorst's  case  cited  by  Jeffries)  pons 
disease  causes  a  spasm  of  lateriversion.  In  this  case  the  eyes  look 
towards  the  lesion. 

The  diagnosis  between  cerebral  and  pontine  conjugate  deviations 
may  be  made  from  the  following  considerations: 


CONJUGATE  DEVIATIONS 


271 


CONJUGATE    PARALYSIS. 

Tn  both  kinds,  the  eyes  cannot  be  moved  in  a  certain  direction,  and  usually  show 
a  well-marked  deviation  in  the  direction  opposite. 

CEREBRAL. 


Deviation   transient. 

Abducens  paralyzed  on  same  side  as 
hemiplegia  (eyes  look  away  from  par- 
alyzed limbs). 

The  ej'e  that  cannot  turn  inwards  in 
making  lateral  movements  yet  can 
always  turn  inwards  in  making  con- 
vergence movements. 

Alwaj's  some  associated  focal  symptoms. 

Facial  paralysis,  if  present,  on  the  same 
side  as  the  hemiplegia. 


PONTINE. 

Deviation  more  apt  to  be  lasting. 

Abducens  paralyzed  on  side  opposite  to 
hemiplegia  (eyes  look  towards  para- 
lyzed limbs). 

Adduction  in  some  cases  nil  for  con- 
vergence, as  well  as  for  parallel  move- 
ments. 

May  be  none   (then  surely  pontine). 

Involvement  of  the  facial  (usually  on 
the  side  opposite  to  the  body  paraly- 
sis) very  frequent.  Fifth,  eighth,  and 
twelfth  also  often  involved. 


CONJUGATE    SPASM. 
In  both,  the  two  eyes  are  strongly  deviated  to  one  side,  but  can  usually  be  moved 
more  or  less  freely  in  the  direction  opposite. 

CEREBRAL.  PONTINE. 

Abducens    on    same    side    as    hemiplegia       Abducens  on  side  opposite  to  hemiplegia 
spastically   contracted    (eyes   look   to-  spastically  contrasted  (eyes  look  away 

wards  paralyzed  limbs).  from  paralyzed  limbs). 


The  following  differential   table   furthermore  will  hold  good   for 
most  cases: 

SITE    OF    LESION    IN    CONJUGATE    DEVIATIONS. 

BOTH    EYES    ARE    DEVIATED   TO   THE   BIGHT.* 


A.  Eyes  cannot  be  turned  to  the  left. 

(a)  There   is   L.   hemiplegia,   with   or 

without  involvement  of  cranial 
nerves  on  L.  side. 

Destructive  lesion  of  R.  side 
of  cerebrum  or  of  L.  side  of 
cerebellum. 

(b)  There  is  R.  hemiplegia  and  usu- 

ally involvement  of  fifth,  sev- 
enth, and  other  cranial  nerves, 
especially  on  L.  side. 

Destructive  lesion  of  L.  side 
of  pons. 


B.  Eyes   usually   can   be   turned    to   the 
left, 
(a)   There  is  R.  hemiplegia. 


Irritative  lesion  of  L.  side  of 
cerebrum  (cortex  only),  or  pos- 
sibly of  R.  side  of  cerebellum. 
(b)  There  is  L.  hemiplegia  with  in- 
volvement of  fifth,  seventh,  and 
other  cranial  nerves,  especially 
on  R.  side. 

Irritative  or  combined  irrita- 
tive and  destructive  lesion  of 
pons. 


Paralysis  of  upward  movement  in  both  eyes  seems  to  be  due  usually 
to  a  lesion  involving  the  anterior  portions  of  both  third-nerve  nuclei 

•  For  diagnosis  of  conjugate  deviations  to  the  left,  substitute  R.  for  L.  and 

vice  versa. 


272  THE    EYE    AND    XERVOUS    SYSTEM 

or  the  corpora  quadrigemina  or  one  occupying  the  middle  line  close 
behind  the  corpora  quadrigemina  and  affecting  the  gray  matter  about 
the  aqueduct  (Henoch,  Jeffries,  Gowers,  Bruce).  In  a  case  with  the 
paralysis  of  both  up  and  down  movements  Wernicke  found  a  lesion  of 
the  corpus  striatum  and  the  optic  thalamus.  Paralysis  of  up  or  down 
movements  also  occurs  occasionally  in  pons  lesions  (in  seven  out  of 
one  hundred  and  forty  cases  collected  by  Schoeler).  Judging  also 
from  Russell's  and  from  Sherrington's  experiments  on  animals,  such 
a  paralysis  may  also  possibly  be  occasioned  by  a  lesion  of  the  anterior 
cerebral  cortex. 

Cases  simulating  a  Conjugate  Paralysis,  but  of  Basal  or  Periph- 
eral Origin. — Conditions  which  closely  resemble  a  conjugate  paralysis, 
but  which  are  not  really  such  since  they  are  not  due  to  an  affection 
of  the  centres  governing  conjugate  movement,  may  be  caused  by  sym- 
metrical basal  or  peripheral  lesions  (simulated  conjugate  paralysis). 

Thus  Olbers  reported  a  case  of  conjugate  deviation  to  the  right  in 
which  autopsy  showed  that  the  left  internus  was  altogether  absent  and 
the  right  extemus  nearly  so.  So  also  a  number  of  cases  of  paralysis 
of  upward  or  downward  movement  are  apparently  due  to  symmetrical 
aplasia  or  mal-insertion  of  the  superior  recti  and  other  muscles  (Heuck, 
Lawford,  Stieren,  A.  E.  Davis).  A  similar  paralysis  of  upward  move- 
ment (Ormerod)  or  of  both  upward  and  downward  movement  (Uht- 
hoff)  may  be  caused  by  symmetrical  lesions  of  the  two  third  nerves  at 
the  base  of  the  brain.  In  Thomson's  case  of  symmetrical  paralysis  of 
upward  movement,  the  lesions  were  both  basal  and  fascicular. 

Conditions  causing  Conjugate  Paralysis  and  Spasm. — Conjugate 
paralysis  and  spasm  are  often  caused  by  hemorrhage  (especially  cere- 
bral hemorrhage)  and  embolism;  less  frequently  by  tumors,  abscess 
(especially  otitic  abscess,  both  cerebral  and  cerebellar),  and  meningitis. 
They  may  also  occur  in  polioencephalitis  superior  before  the  stage  of 
complete  paralysis,  indicating,  then,  that  the  process  has  involved  the 
gray  matter  beneath  the  aqueduct,  but  not  as  yet  the  oculo-motor  nucleus 
(Bemheimer). 

Paralysis  of  conjugate  movement,  both  lateral  and  vertical,  occurs 
quite  frequently  in  multiple  sclerosis  (in  3  per  cent,  of  the  cases,  accord- 
ing to  Ulithoff).  It  is  also  occasionally  present  in  pseudobulbar  paraly- 
sis, and,  very  rarely  indeed,  in  tabes  and  amyotrophic  lateral  sclerosis. 

Conjugate  paralysis  and  especially  conjugate  spasm  may  be  due 
to  hysteria,  the  traumatic  neuroses,  or  neurasthenia.  In  this  case, 
Graefe's  test  (page  267)  may  show  that  the  power  of  movement,  although 
apparently  absent,  is  actually  present. 


NYSTAGMUS  AXD   PSEUDOXYSTAGMUS  273 

Defects  in  the  associated  movements  of  the  eyes  are  sometimes 
congenital  and  hereditary.  Such  defects,  however,  are  probahly  in 
most  cases  not  instances  of  a  true  conjugate  paralysis,  but  are  simply 
the  results  of  a  symmetrical  absence  of  the  muscles  or  of  some  similar 
anomaly  of  development  (page  272), 

Treatment. — It  is  rare  for  a  conjugate  deviation  to  give  rise  of 
itself  to  any  symptoms  that  require  treatment.  In  cases  in  which 
the  vertigo  is  annoying  and  the  conjugate  deviation  persistent,  the 
condition  may  be  relieved  by  a  symmetrical  tenotomy  {e.g.,  of  the  two 
right  rotators,  if  there  is  paralysis  of  levoversion).     (A.  Graefe.) 

Teemok  of  the  Associated  Parallel  Movements. 

XySTAGMUS  AXD  PSEUDONYSTAGMUS. 

Varieties  of  Tremor. — We  must  distinguish  three  varieties  of  tre- 
mor of  the  eye  movements : 

A.  Searching  Movements. — Both  eyes  make  an  occasional  wide,  com- 
paratively slow,  sweej^ing  movement  from  the  primary  position  in 
some  direction,  and  then,  either  immediately  or  after  a  time,  return 
to  the  primary  position.  The  movements  are  apparently  purposeful, 
just  as  if  the  patient  were  trying  to  look  at  an  object  situated  to  one 
side  of  him.  They  may  take  place  in  any  direction,  vertical,  oblique, 
or  horizontal. 

B.  Pseudonystagmus. — One  or  both  eyes  when  carried  to  a  point 
near  the  limit  of  their  excursion  in  some  direction,  make  a  series  of 
jerky  movements  on  from  this  point  and  back  to  it  again,  but  in  return- 
ing do  not  re-pass  it.  These  jerks  evidently  represent  an  extreme  effort 
to  keep  up  the  original  excursive  movement  of  the  eyes,  the  smooth 
steady  pull  of  the  muscle  that  is  carrying  the  eye  along  being  converted 
into  a  series  of  discontinuous,  spasmodic  tugs. 

The  direction  of  the  jerky  movements  in  pseudonystagmus  is  usually 
the  same  as  that  in  which  the  eyes  were  being  carried  when  the  move- 
ments started, — i.e.,  if  the  eyes  were  moving  to  the  right  the  pseudo- 
nystagmus will  make  them  move  jerkily  still  further  to  the  right  and 
then  more  slowly  back  again.  Sometimes,  however,  when  the  eyes  are 
moving  laterally,  the  spasmodic  attempt  to  carry  them  further  may 
result,  not  in  a  continuation  of  the  lateral  movement,  but  in  a  jerky 
rolling  of  the  eyes  about  an  anteroposterior  axis. 

Pseudonystagmus,  although  more  often  bilateral,  is  not  infrequently 
confined  to  one  eye,  particularly  if  that  eye  has  relatively  weak  muscles. 

C.  Nystagmus. — In  nystagmus  the  eyes  make  a  series  of  very  regu- 
lar, short,  quick  oscillations  about  a  central  point.     Nystagmus  differs 

18 


274  THE    EYE    AND    NERVOUS    SYSTEM 

from  pseudonystagnms,  first,  in  that  it  often  occurs  when  the  eyes  are 
in  the  primary  position  or  near  it,  whereas  pseudonystagmus  occurs 
only  wlien  the  eyes  are  near  the  end  of  an  excursion  in  some  direction ; 
second,  the  movements  in  nystagmus  are  oscillations  to  and  fro  about 
a  central  point,  while  in  pseudonystagmus  they  are  movements  from 
a  terminal  point  and  back  to  it  again ;  third,  the  movements  in  pseudo- 
nystagmus are  jerky  and  bespeak  effort,  in  true  nystagmus  are  smooth 
and  regular,  and,  except  in  miners'  nystagmus,  are  not  often  associated 
with  a  restriction  in  the  excursive  power  of  the  eyes,  which  is  very 
frequently  present  in  pseudonystagmus. 

Number  and  Extent  of  Oscillations. — The  oscillations  in  nystagmus 
range  from  one  to  four  a  second  and  in  width  vary  from  one  to  five 
millimetres. 

In  nystagmus  the  oscillations  may  be  horizontal,  vertical,  or  rotary, 
— ^the  cornea  in  the  last  case  revolving  like  a  wheel  round  its  centre 
to  and  fro,  through  a  more  or  less  considerable  arc.  Combined  vertical 
and  horizontal  nystagmus  producing  an  oblique  oscillation  is  called 
mixed  nystagmus;  and  the  same  term  may  with  propriety  be  ap- 
plied to  tlie  forms  in  which  a  rotary  is  combined  with  a  lateral  or 
a  vertical  oscillation.  Still  more  complicated  are  the  cases  described 
by  A.  Graefe  as  occurring  in  miners'  nystagmus,  in  which,  by  a  com- 
bination of  successive  horizontal  and  vertical  movements,  the  centre  of 
each  cornea  moves  in  a  circular  or  elliptical  path  (circumduction 
nystagmus).  Thompson  and  Spicer  have  described  a  similar  circum- 
duction movement  in  the  nystagmus  associated  with  spasmus  nutans  in 
infants. 

Symmetry  of  Movements. — The  nystagmic  movements  show  a 
marked  symmetry.  The  movements  are  nearly  always  bilateral,  and 
when  bilateral  are  almost,  without  exception,  equal,  simultaneous,  and 
parallel  in  the  two  eyes, — i.e.,  both  eyes  move  equally  far  and  at  the 
same  instant  to  the  right,  or  both  rotate  to  the  right,  etc. 

Bilateral  nystagmus  is  usually  horizontal,  although  the  rotary  form 
is  pretty  frequent.  Mixed  nystagmus  is  less  often  met  with,  and  ver- 
tical bilateral  nystagmus  is  rare. 

Very  rarely  indeed  bilateral  nystagmus  is  disjunctive, — i.e.,  the  two 
eyes,  instead  of  moving  in  a  parallel  path,  move  towards  or  away  from 
each  other.  TliTis  in  Graefe's  case  of  circumduction  nystagmus  the 
eyes  both  travelled  in  elliptical  paths,  but  in  opposite  directions — each 
revolving  from  the  nose  towards  the  temple.  I  myself  saw  a  case  in 
which  there  was  a  converging  rotary  nystagmus, — i.e.,  both  corneae 
made  simultaneous  wheel-like  movements  from  the  temple  towards  the 


NYSTAGMUS  AND   PSEUDONYSTAGMUS  275 

nose.  Thompson  and  Spicer  say  that  infants  affected  with  spasmus 
nutans  sometimes  show  a  form  of  nystagmus  in  which  the  eyes  alter- 
nately and  rhythmically  converge  and  diverge,  and  the  same  form  has 
been  observed  in  adults  as  the  result  of  excessive  accommodation  in 
near  work  (Evershusch). 

Also  very  rare  are  the  cases  of  nystagmus  in  which  the  motions  of 
the  eyes  are  dissociated, — i.e.,  are  quite  unlike,  so  that  they  do  not 
resemble  any  co-ordinated  movement,  either  parallel  or  disjunctive. 
Thus,  the  oscillations  may  be  horizontal  in  one  eye  and  vertical,  rotary, 
or  oblique  in  the  other.  Such  combinations  occur  (a)  in  the  nystagmus 
of  spasmus  nutans  (Thompson)  ;  (&)  sometimes  in  a  unilateral  nystag- 
mus when  it  assumes  the  bilateral  form  ( Schapringer,  Neustatter, 
Duane)  ;  (c)  occasionally  in  ordinary  bilateral  nystagmus  (A.  Graefe, 
Frost,  Lawford,  Duane). 

Unilateral  nystagmus  is  rare,  some  fifty-two  cases  having  been 
reported.  It  is  generally  vertical,  but  was  horizontal  in  cases  described 
by  V.  Keuss,  Everbusch,  Bouchaud,  Nagel,  R.  Sachs,  Norrie  (two  cases), 
Neustatter,  and  Oppenheimer,  and  in  one  that  I  saw  in  Dr.  Arnold 
Knapp's  clinic.  Unilateral  oblique  nystagmus  has  been  seen  by  Neu- 
statter, and  unilateral  rotary  nystagmus  by  Weber,  Simon  (two  cases), 
Neustatter,  and  myself.  Simon  saw  one  case  in  which  for  a  time  the 
nystagmus  was  unilateral  and  mixed  (vertical  and  rotary),  but  later 
became  bilateral  and  horizontal. 

Sometimes  when  the  nystagmus  is  apparently  confined  to  one  eye, 
it  will  be  found  that  slight,  inconstant  oscillations  are  present  in  the 
other.  These  may  be  discovered  only  with  the  ophthalmoscope,  which 
reveals  a  peculiar  indistinctness  of  the  fundus  due  to  the  tremor. 

In  other  cases  still,  as  proved  by  Simon's  experience  just  cited,  and 
in  one  case  of  mine,  a  unilateral  nystagmus  may  become  completely 
bilateral. 

Finally,  just  the  reverse  may  occur, — i.e.,  a  nystagmus  originally 
bilateral  may  become  confined  to  one  eye.  Thus,  in  spasmus  nutans,  the 
nystagmus  may  disappear  from  one  eye  before  it  disappears  from  both 
(Schapringer)  ;  and  a  bilateral  nystagmus  due  to  opacities  of  both 
cornese  may  disappear  first  from  the  eye  which  clears  the  first  (Simon). 

Conditions  causing  Variations  in  Nystagmus. — Nystagmus  is  usu- 
ally more  or  less  intermittent.  And,  whether  intermittent  or  not,  nys- 
tagmus will  in  the  same  individual  show  considerable  variations  in 
both  the  amplitude  and  the  rapidity  of  the  oscillations.  These  varia- 
tions are  often  determined  by  specific  conditions, — e.g.,  by  physical  and 
psychical    excitation   or   depression,   by   sensory   stimuli    (particularly 


276  THE    EYE    AND    NERVOUS    SYSTEM 

changes  in  illumination),  by  the  use  of  alcohol,  and  sometimes  even 
by  voluntary  effort  (A.  Graefe).  Nystagmus  is  often  seen  to  increase 
if  the  patient  looks  aimlessly  into  vacancy  instead  of  fixing;  or  it  in- 
creases if  the  eyes  are  carried  in  some  special  direction,  ceasing  perhaps 
if  they  are  turned  in  another  way.  In  other  cases  it  ceases  when  the 
eyes  are  converged  on  a  near  object.  Lastly,  in  some  cases  a  nystagmus 
is  regularly  developed  in  one  or  both  eyes  as  soon  as  one  eye  is  occluded ; 
in  other  cases  such  occlusion  puts  a  stop  to  a  nystagmus  that  is  present 
in  binocular  vision. 

Nystagmus  may  also  change  its  direction, — e.g.,  from  vertical  to 
oblique  or  even  from  vertical  to  horizontal,  when  the  eyes  are  turned 
in  different  ways. 

A  careful  study  of  the  cases  showing  these  variations  will,  I  believe, 
lead  to  the  conclusion  that  in  a  given  case  of  nystagmus  the  oscillations 
become  least  in  that  position  of  the  eyes  which  for  this  case  involves 
the  least  strain  upon  the  eye-muscles,  or  they  are  least  under  that  con- 
dition of  vision  (exclusion  of  one  eye,  use  of  both  eyes,  use  of  glasses, 
etc.),  which  implies  the  least  confusion  to  sight. 

D.  Combined  Forms  of  Tremor. — In  some  cases  we  find  searching 
movements  combined  with  a  pseudonystagmus.  This  would  seem  espe- 
cially to  occur  when  there  is  absence  of  central  fixation,  and  at  the 
same  time  the  movements  of  the  eyes  are  more  or  less  restricted. 

In  some  cases  we  may  find  nystagmus  and  pseudonystagmus  com- 
bined in  the  same  patient.  Thus,  as  in  a  case  of  Uhthoff's,  there  may 
be  a  continual  horizontal  nystagmus  which  becomes  coupled  with  a  ver- 
tical jerky  pseudonystagmus  as  soon  as  the  eyes  are  carried  far  up  or 
down. 

Etiology  of  Nystagmus  and  Pseudonystagmus. — Searching  Move- 
ments are  found  in  blind  eyes  and  in  eyes  which  from  chorioidal 
lesions  at  the  yellow  spot  or  similar  causes  have  lost  the  power  of 
central  fixation. 

Pseudonystagmus  occUrs  almost  constantly  in  hereditary  ataxia,  and 
in  this  disease  it  is  usually  very  marked.  It  also  is  found  very  fre- 
quently in  multiple  sclerosis  (in  46  per  cent,  according  to  Uhthoff). 
But  as  it  is  also  present  in  a  great  variety  of  other  nervous  diseases, 
both  functional  and  organic,  and  not  infrequently,  in  fact,  is  found 
in  people  who  are  perfectly  healthy,  it  is  not  of  great  importance  as  a 
diagnostic  sign. 

Pseudonystagmus  in  many  cases,  if  not  always,  signifies  some  weak- 
ness of  the  muscles  involved  in  making  the  special  associated  movement 
in  the  course  of  which  it  occurs. 


XYSTAGMUS  AXD  PSEUDOXYSTAGMUS  277 

Nystagmus  is  caused  by — 

(A)  Conditions  producing  bilateral  amblyopia  in  early  infancy 
{optical,  infantile,  or  so-called  congenital  nystagmus).  Such  conditions 
are  opacities  of  the  cornea,  especially  from  blennorrhoea  neonatorum; 
congenital  cataract ;  hemorrhage,  disease  or  abnormalities  of  the  retina 
and  chorioid ;  total  congenital  color-blindness,  and  albinism.  Refractive 
errors,  even  when  great,  do  not  usually  cause  it,  although  they  may 
do  so. 

Such  nystagmus  is  probably  not  often  really  congenital,  but  develops 
during  the  first  few  months  of  life. 

It  must  be  remarked  that  unilateral  amblyopia  very  rarely  produces 
nystagmus  but  tends  rather  to  produce  squint.  Simon,  however,  reports 
two  cases  in  which  an  infantile  unilateral  nystagmus  was  caused  by 
unilateral  opacities  of  the  cornea.  It  is  noteworthy  that  in  one  of 
these  cases  the  nystagmus  was  originally  bilateral,  and  disappeared  from 
one  eye  only  when  the  cornea  of  that  eye  cleared  up.  Later,  w^hen  the 
cornea  of  the  other  eye  cleared,  the  nystagmus  disappeared  from  that 
eye,  too. 

The  unilateral  nystagmus  that  occurs  in  an  amblyopic  eye  affected 
W'ith  squint  (Heimann)  is  probably  not  often  infantile  in  origin,  but 
is  to  be  regarded  as  a  late  acquired  optical  nystagmus  (see  under  type  B, 
infra). 

Sometimes  a  nystagmus  of  this  form,  evidently  dating  from  in- 
fancy, occurs  without  there  being  any  obvious  lesion  of  the  eyes  to 
cause  it.  In  such  cases,  according  to  A.  Graefe,  the  nystagmus  is  proba- 
bly attributable  to  a  congenital  retinal  hemorrhage  which  subsequently 
cleared  up. 

(B)  Conditions  developing  in  later  life  causing  constant  strain  of 
the  eyesight  from  poor  illumination  or  excessive  strain  of  the  eye- 
muscles,  or  usually  strain  of  both  kinds  combined. 

This  variety  of  late  acquired  nystagmus  is  usually  due  to  conditions 
incident  to  the  patient's  occupation  (hence  occupational  nystagmus). 
The  commonest  form  of  this  is  miners'  nystagmus,  occurring  in  coal- 
miners  who  work  with  the  eyes  directed  constantly  up  and  back  and 
by  the  insufficient  light  of  the  safety-lamps.  The  bad  air  of  the  mines 
is  thought  to  be  an  additional  factor  in  causing  it.  Miners'  nys- 
tagmus is  characterized  by  its  markedly  periodic  character  and  its 
tendency  to  recur  under  conditions  of  reduced  illumination,  or  when 
the  eyes  are  directed  up,  or  as  a  result  of  bodily  exertion.  Another 
feature,  distinguishing  it  particularly  from  early  acquired  nystagmus 
(type  A)  is  the  fact  that  the  eye  movements  are  very  greatly  restricted 


278  THE    EYE    AND    NERVOUS    SYSTEM 

and  the  patient  finds  it  hard  to  keep  the  eyes  in  the  fixing  position 
(A.  Graefe). 

Similar  to  miners'  nystagmus  is  the  form  occurring  in  compositors, 
paper-makers,  metal-rollers,  etc.,  who  work  with  tlie  eyes  in  a  constrained 
position  (Snell,  Eversbusch),  and  in  those  who  work  in  an  insufficient 
light  or  under  other  conditions  peculiarly  trying  to  the  eyes  (Frost, 
Clarke).  Magiuis  (cited  by  Snell)  tells  of  the  case  of  a  baby  who 
developed  nystagmus  as  the  result  of  constantly  looking  at  a  light 
above  and  behind  his  crib,  and  who  got  well  when  put  in  a  properly 
lighted  room. 

In  the  same  category  belong  those  cases  in  which  optical  nystagmus 
develops  after  the  age  of  infancy  as  a  result  of  high  astigmatism  (as 
in  a  case  of  mine),  or  of  traumatic  cataract  (Krauss),  or  of  other  con- 
ditions producing  marked  amblyopia.  These  cases  are  rare.  They  are 
comparatively  often  unilateral,  and  then  are  often,  if  not  always,  asso- 
ciated with  squint,  which  may  indeed  be  the  cause  of  the  amblyopia  and 
the  nystagmus  (Ileimann). 

According  to  Bar  a  transient  nystagmus  may  be  induced  by  other 
forms  of  reflex  sensory  irritation  originating  in  the  eye, — for  example, 
by  inflammation  or  foreign  bodies  of  the  conjunctiva  or  cornea.  Here 
the  impulse  for  the  discharge  of  the  oscillatory  movements  presumably 
passes  through  the  fifth  nerve  to  the  pons,  finally  reaching  the  abducens 
and  oculo-motor  nuclei.  It  is  questionable  whether  in  such  cases 
the  irritation  of  the  eye  is  really  the  cause  of  the  nystagmus,  or  whether 
it  has  not  simply  stirred  into  action  a  pre-existing  nystagmus  tem- 
porarily quiescent. 

(C)  Irritation  arising  from  the  ear  {aural  nystagmus).  Nystag- 
mus often  is  produced  by  some  irritation  arising  from  the  inner  ear. 
Such  irritations  appear  to  act  by  setting  in  motion  the  internus  of  tlie 
same  side  as  the  affected  ear  and  the  extemus  on  the  opposite  side.  It 
may  be  produced  l)y  actual  disease  of  the  labyrinth,  by  changes  in  laby- 
rinthine pressure  due  to  rapid  rotation  of  the  body,  and  by  operative 
manipulations  carried  on  in  the  middle  and  external  ear  (probing, 
syringing,  etc.)  (Eversbusch).  Peters  thinks  that  miners'  nystagmus 
is  of  labyrinthine  origin. 

According  to  Schwartze,  aural  nystagmus  is  not  due  primarily  to 
heightened  labyrinthine  pressure,  but  to  irritation  of  the  vestibular 
nerve. 

(D)  Nervous  Disease. — True  nystagmus  occurs  frequently  in  mul- 
tiple sclerosis  (in  12  per  cent,  of  the  cases,  according  to  Uhthoff).  It  is 
also  frequent  in  syringomyelia. 


NYSTAGMUS  AND   PSEUDONYSTAGMUS  279 

Nystagmus  has  also  been  described  as  occurring  often  in  other  ner- 
vous diseases,  as  meningitis,  meningeal  hemorrhage,  sinus-thrombosis, 
hemorrhage  and  softening  of  the  brain,  tumors  of  the  brain,  particularly 
the  cerebellum,  and  pons  disease  (Gowers).  In  many  of  the  reported 
cases,  however,  care  has  not  been  taken  to  distinguish  between  nystagmus 
and  pseudonystagmus.  Except  in  multiple  sclerosis  and  syringomelia, 
and  in  conditions  due  to  ear  trouble,  true  nystagmus  is  rare  in 
nervous  disorders ;  occurring  in  only  3  cases  out  of  500  examined 
by  Uhthoff  and  in  only  1  per  cent,  of  tables.  And  even  in  the  cases  in 
which  it  does  occur  it  is  often  not  due  to  the  nervous  disease  itself,  but  is 
simply  an  infantile  nystagmus  produced  by  opacities  in  the  media, 
congenital  defects,  etc. 

We  may,  therefore,  say  that  a  true  nystagmus,  not  due  to  optical 
defect  nor  dating  from  childhood,  and  not  traceable  to  occupation,  as  in 
miners'  nystagmus,  nor  to  ear  disease,  is  strong  presumptive  evidence 
either  of  multiple  sclerosis  or  less  prohabhj  of  syringomyelia. 

A  transient  nystagmus,  often  of  peculiar  form,  frequently  occurs  in 
spasmus  nutans  (Thompson,  Spicer,  Schapringer).  This  may  be  uni- 
lateral, or  be  bilateral  at  one  time  and  unilateral  at  another. 

Nystagmus  may  occur  in  hysteria;  and  in  hysterical  subjects  has 
been  evoked  and  cured  by  suggestion  (Sabrazes). 

Nystagmus,  probably  due  to  central  nervous  disease,  has  been  occa- 
sioned by  traumatism.  Such  nystagmus  is  rare.  It  may  be  transitory, 
as  in  two  cases  described  by  Norrie.  In  some  instances,  at  all  events, 
as  in  those  given  by  Apelt,  it  seems  to  be  simply  the  result  of  a  func- 
tional nervous  condition  (traumatic  hysteria). 

Not  to  be  confounded  with  these  cases  of  traumatic  nystagmus  are 
those  in  which  an  acquired  optical  nystagmus  (see  under  type  B)  has 
resulted  from  the  optical  defects  produced  by  a  traumatism  (two  cases 
described  by  Krauss). 

Both  nystagmus  and  pseudonystagmus  occurring  in  nerve  disease 
are  often  associated  with  paresis  or  w^eakness  of  the  muscles. 

(E)  Poisons. — Nystagmus  is  said  to  occur  very  often  in  patients 
that  are  under  the  influence  of  ether.  Other  agents  that  very  rarely 
produce  nystagmus  are  alcohol,  cocaine,  sulphonal,  arsenic,  lead,  qui- 
nine, ergot,  and  sewer  gas  (Uhthoff). 

(F)  Voluntary  Nystagmus. — Occasionally  nystagmus  may  be  pro- 
duced voluntarily  (A.  Graefe;  Dodd ;  A.  E.  Davis).  Graefe  thinks 
that  this  can  occur  only  in  those  who  were  formerly  the  subjects  of  an 
involuntary  nystagmus,  which  has  disappeared. 

Nystagmus,  whether  infantile  or  due  to  nervous  disease,  is  some- 


280  THE    EYE    AXD    NERVOUS    SYSTEM 

times  hereditary  ( Xettleship ;  McGillivray;  Audeoud;  see  also  excel- 
lent summary  by  Groenouw). 

Site  of  Lesion  in  Conditions  of  Ocular  Tremor. — In  the  infantile 
form  of  nystagmus,  as  well  as  in  the  form  due  to  occupation  {miners* 
nystagmus) ,  and  to  aural  irritation,  the  tremor  is  not  due  to  any  actual 
lesion  of  the  brain.  The  contrary  has  been  asserted  of  miners'  nystag- 
mus, but,  as  A.  Graefe  says,  without  sufficient  proof.  In  the  nystagmus 
of  multiple  sclerosis  and  other  organic  nervous  diseases,  lesions  have 
been  found  mainly  in  the  corpora  quadrigemina,  corpus  striatum,  resti- 
form  bodies,  cerebellum,  and  medulla.  It  may  be  noted  that  experi- 
mental stimulation  of  the  same  parts  and  also  of  the  cerebral  cortex 
in  animals  likewise  produces  nystagmus.  Occasionally  the  nystagmus 
in  nervous  disease  seems  to  be  produced  not  by  a  lesion  interfering 
directly  with  the  paths  for  the  associated  movements  of  the  eyes,  but 
by  remote  lesions  which  act  by  causing  variations  in  the  intracranial 
pressure  (Uhthoff).     (See  Pages  449  and  472). 

In  pseudonystagmus  due  to  organic  nerve  disease  the  lesions  are 
also  in  most  cases  central,  although  in  some  instances  they  may  be  seated 
in  the  peripheral  nerves  supplying  the  muscles. 

Theory  of  Nystagmus. — Xo  perfectly  satisfactory  explanation  of 
nystagmus  has  yet  been  given.  The  remarkable  regularity  and  paral- 
lelism of  the  movements,  however,  show  that  in  nystagmus  we  have  to 
deal  with  a  perversion  of  the  centres  for  parallel  and  parallel-rotary 
movements,  and  not  with  peripheral  lesions  of  the  muscles  themselves 
or  their  nerves. 

This  perverted  state  of  the  association  centres  is  evidently  produced 
in  optical  and  miners'  nystagmus  by  long-continued  abnormal  stimuli 
passing  up  through  the  visual  tract,  and  in  miners'  nystagmus  also 
through  the  fifth  nerve ;  in  aural  nystagmus  by  abnormal  reflexes  pass- 
ing through  the  auditory  nerve  to  the  centres  in  the  pons  and  perhaps 
also  in  the  corpora  quadrigemina ;  while  in  nystagmus  due  to  nervous 
disease,  the  association  centres  and  tracts  are  probably  affected  directly. 

Xystagmus  in  all  probability  is  produced  by  alternate,  just  as  nor- 
mal fixation  is  produced  by  simultaneous,  discharges  of  motor  energy 
from  the  two  sides  of  the  brain.  That  is,  when  we  fix,  the  eyes  are  held 
steadily  in  place,  because  impulses  are  sent  down  at,  the  same  time  from 
the  right  angular  gyrus  (which  acting  alone  would  cause  both  eyes  to 
move  to  the  left)  and  from  the  left  angular  gyrus  (which  acting  alone 
would  cause  both  eyes  to  move  to  the  right).  In  a  person  with  nystag- 
mus the  impulses  from  the  right  angular  gyrus  and  from  the  left 
angular  gyrus  follow  each  other  in  rapid  succession. 


:N^YSTAGMUS  and   PSEUDONYSTAGMUS  281 

^Nystagmus,  then,  may  be  regarded  as  a  kind  of  imperfect  or  per- 
verted fixation.  Infantile  nystagmus,  in  particular,  is  an  incompletely 
developed  fixation — a  condition  intermediate  between  the  wide,  more  or 
less  irregular,  excursions  of  a  congenitally  blind  eye  and  the  steady  gaze 
of  an  eye  which  fixes  normally. 

This  explanation  of  the  nature  of  nystagmus  holds  good  un- 
doubtedly even  for  the  rare  unilateral  and  dissociated  forms.  These 
differ  from  the  ordinary  bilateral  and  symmetrical  nystagmus  only  in 
the  fact  that  either  the  refiex  discharges  from  the  association  centres, 
which  cause  tlie  oscillation,  are  in  some  way  so  altered  as  to  affect 
one  eye  more  than  the  other,  or  else  there  is  a  contributing  cause, — 
usually  some  muscular  anomaly, — which  being  mainly  seated  in  one 
eye  makes  that  eye  respond  more  readily  than  its  fellow  to  the  abnor- 
mal stimuli. 

Symptoms   of   Nystagmus. — The   symptoms  of  nystagmus   are — 

1.  Apparent  Movement  of  Objects. — We  should  expect  that  the 
rapid  oscillation  of  the  eyes  in  nystagmus  would  make  it  seem  to  the 
patient  as  if  all  objects  that  he  looked  at  were  jumping  to  and  fro.  And 
in  miners'  nystagmus  and  the  form  due  to  aural  disease  this  apparent 
movement  of  objects  is  a  very  prominent  and  distressing  symptom.  It 
is  also  often,  though  not  always,  present  in  the  nystagmus  due  to  nervous 
disease.  Sometimes  in  these  cases  the  patient  not  only  sees  objects 
move  but  feels  that  the  eye  is  trembling. 

On  the  other  hand,  in  optical  nystagmus  due  to  infantile  amblyopia, 
this  apparent  movement  of  objects  is  very  rarely  perceived.  Sometimes 
in  unilateral  nystagmus  and,  under  exceptional  conditions,  in  bilateral 
nystagmus  it  can  be  evoked  (A.  Graefe).  Even  then,  however,  it  seems 
hardly  ever  to  be  troublesome. 

2.  Reading  in  Vertical  Lines. — That  the  apparent  movement  of 
objects,  even  w^hen  it  is  not  perceived  by  the  patient,  yet  causes  indis- 
tinctness of  things  seen,  is  shown  by  the  fact  that  some  patients  with 
horizontal  nystagmus  in  reading  hold  the  book  sideways,  so  that  the 
lines  of  print  run  vertically.  This,  to  a  certain  extent,  obviates  the 
confusion  produced  by  the  oscillations  of  the  letters  ( Soelberg- Wells, 
Wecker,  Duane). 

3.  Vertigo. — This  is  marked  in  some  of  the  late  acquired  forms, 
especially  in  miners'  nystagmus  and  the  form  due  to  aural  irritation. 

4.  Nodding  Movements  of  Head. — These  often  occur  in  optical  nys- 
tagmus. They  were  formerly  thought  to  be  compensatory  in  character, 
acting  to  prevent  the  tremor  of  objects  produced  by  the  oscillation  of  the 
eyes.     This,  however,  is  certainly  not  the  case ;  indeed,  in  one  instance 


282  THE    EYE    AND    NERVOUS    SYSTEM 

that  I  saw  the  nodding  of  the  head  considerably  increased  the  confusion 
of  sight  due  to  the  nystagmus. 

5.  Poor  Vision. — In  most  cases  of  nystagmus  of  infantile  origiil, 
the  vision  is  quite  poor,  and  cannot  be  brought  up  to  normal  by  glasses. 
This  is  doubtless  in  part  due  to  the  blurring  that  the  nystagmus  pro- 
duces, but  much  more  to  the  pathological  condition  causing  the  nystag- 
mus. In  fact,  nystagmus  in  itself  does  not  necessarily  cause  permanent 
poor  sight.  I  have  seen  it  associated  with  a  vision  of  nearly  yj- 
It  is  to  be  inferred  that  in  such  cases  the  application  of  glasses  or  the 
mere  act  of  fixing  attentively  with  the  moving  eye  has  made  the  latter 
quiet  for  the  time  being,  since  it  is  very  difficult  to  suppose  that  the 
sight  could  be  anything  but  bad  so  long  as  the  eyes  are  still  in  active 
motion.  In  fact,  we  can  prove  in  those  cases  in  which  the  nystagmus 
increases  greatly  in  one  direction  of  the  gaze  and  diminishes  in  another, 
that  the  sight  is  very  much  better  in  that  position  in  which  the  oscilla- 
tion is  slight.  In  miners'  nystagmus  the  disturbance  of  sight  produced 
by  the  tremor  is  considerable. 

6.  PhotopJiobia  may  be  present.  In  unilateral  nystagmus  it  may 
occur  only  when  the  oscillating  eye  is  used  to  see  with  (Schapringer). 

Course  of  Nystagmus. — Infantile  nystagmus  generally  lasts  through 
life,  although  it  may  disappear.  Nystagmus  due  to  aural  or  nervous 
disease  naturally  persists  as  long  as  its  cause.  Miners'  nystagmus  dis- 
appears if  the  patient  gives  up  his  work  and  lives  under  proper  condi- 
tions as  regards  illumination  and  eye-strain.  Nystagmus  due  to  spas- 
mus nutans  and  hysteria  is  probably  always  transient.  A  transient 
nystagmus  may  also  result  from  traumatism  (Norrie),  tenotomy 
(Wecker),  or  other  causes  (Simon,  and  others). 

Treatment. — The  treatment  of  nystagmus  must  be  directed  to  the 
cause.  Infantile  nystagmus  is  rarely  affected  by  any  treatment, 
although  isolated  cases  have  been  cured  by  removal  of  a  congenital 
cataract  (Crzellitzer,  Cuperus),  by  tenotomy  or  advancement  causing 
relief  of  a  squint  (Kugel,  Neustatter,  Heimann,  Cuperus),  by  exercises 
with  a  stereoscope  and  in  reading  with  the  aid  of  a  perforated  dia- 
phragm (Lavagna),  by  exercises  in  fixation  in  various  positions  of  the 
gaze  (Brisseaud;  Meige  and  Feindel),  and  by  exercises  with  rotating 
prisms  (Trombetta).  As  a  temporary  help  Kugel  recommends  pro- 
tracted bandaging  of  the  eyes  and  the  use  of  dark  glasses  before  the 
better  eye.  Some  cases  of  nystagmus,  especially  acquired  nystagmus, 
are  relieved  by  the  correction  of  astigmatism  or  other  refractive  errors 
(Kugel,  Duane).  In  miners'  and  other  forms  of  occupational  nystag- 
mus change  of  occupation  is  imperative. 


AXOMALOUS  DEVIATIOXS  283 

Section  VI. 
ANOMALOUS   DEVIATIONS  OF  THE  EYES. 
DISJUNCTIVE  MOVEMENTS. 

In  some  cases  the  eyes  no  longer  follow  the  law  of  associated  move- 
ments, but  each  moves  irrespective  of  the  other.  This  occurs,  for  ex- 
ample, in  eyes  that  have  been  totally  blind  from  birth,  also  in  patients 
deeply  under  the  influence  of  chloroform.  But  it  occasionally  occurs 
in  other  cases,  too. 

Thus,  a  deviation  of  one  eye  down  and  out  and  of  iJie  other  eye  up 
and  in  is  said  by  Gowers  to  occur  in  disease  of  the  middle  cerebellar 
peduncle. 

Lawford  describes  a  case  in  which,  when  the  patient  was  told  to 
look  to  the  left,  both  eyes  moved  outwat'ds.  (This  may  have  been  simply 
an  instance  of  excessive  voluntary  divergence  of  the  eyes — divergence- 
excess). 

Kunn  gives  six  cases  of  dissociated  paralyses. 

Uhthoff  says  that  occasionally  dissociated  ataxic  movements  occur 
in  tabes. 

I  have  seen  a  case  in  which  the  right  eye  at  intervals  moved  slowly 
up  and  after  awhile  moved  down  again,  while  the  left  eye  remained 
perfectly  still.  The  movement  of  the  right  eye  took  place  whether  the 
left  eye  was  covered  or  open.  The  condition  was  probably  a  sort  of 
unilateral  searching  movement  (page  273),  and  may  be  regarded  as 
an  abortive  form  of  unilateral  nystagmus. 

A  special  form  of  partial  dissociation  of  the  ocular  movements  is 
seen  in  those  cases  in  which  on  alternate  covering  each  eye  goes  up  or 
each  eye  goes  down  behind  the  screen.  Cases  of  this  sort  have  been 
described  by  Schweigger  and  especially  by  Stevens.  I  myself  have 
seen  three  or  four.  In  these  cases  there  may  or  may  not  be  binocular 
fixation  when  both  eyes  are  uncovered.  If  there  is  binocular  fixation, 
the  condition  is  called  anophoria  or  double  hyperphoria  if  both  eyes  go 
up,  and  catophoria  or  double  hypophoria  if  both  go  down,  when 
screened.  The  corresponding  conditions  in  which  one  eye  squints  when 
both  are  uncovered  are  called  anotropia  and  catotropia.  Such  a  squint 
may  be  alternating,  and  then  in  anotropia  each  eye  in  turn  will  squint 
up  (double  hypertropia)  and  in  catotropia  each  eye  in  turn  will  squint 
down  {double  hypotropia). 

Some  of  the  cases  described  as  illustrating  dissociation  of  the  eye 
movements  are  not  really  of  this  'character.  Thus,  Konigshofer's  case 
in  which,  without  moving  the  right  eye,  the  left  could  be  voluntarily 


284  THE    EYE    AND    NERVOUS    SYSTEM 

turned  in  or  out  or  be  made  to  look  straight  ahead,  was  evidently  one 
of  left  divergent  squint,  which  was  overcome  by  a  strong  effort  of  con- 
vergence, both  eyes  at  the  same  time  being  carried  to  the  right  by  an 
associated  parallel  movement.  Again,  the  case  given  by  A.  Graefe  in 
which  the  left  eye  went  straight  up  when  the  attempt  was  made  to  move 
both  eyes  to  the  right,  can  readily  be  explained  by  supposing  that,  as 
the  left  eye  could  not  move  inwards  because  its  intemus  was  paralyzed, 
it  was  forced  spasmodically  upward  by  the  overaction  of  its  elevators, 
which  were  brought  excessively  into  play.     {Cf.  remarks,  page  249.) 

Weinhold's  case,  in  which  a  patient  with  usually  normal  eye  move- 
ments could  at  will  produce  a  left  strabismus  sursumvergens,  marked 
when  he  looked  to  the  right  but  more  and  more  slight  when  he  looked 
to  the  left,  was  probably  one  of  moderate  insufficiency  of  the  right 
superior  rectus.  In  this  case,  if  the  patient  fixed  with  the  left  eye 
the  deviation  would  be  but  slight,  even  when  he  looked  to  the  right, 
and  might  indeed  be  absent  altogether  if,  as  could  well  happen,  he  had 
developed  an  excessive  ability  to  diverge  the  eyes  in  a  vertical  direction. 
If,  however,  by  a  voluntary  transition  he  fixed  with  the  paretic  right 
eye,  the  left  eye  would  immediately  assume  a  position  of  marked  sec- 
ondary deviation  and  would  shoot  up,  especially  when  he  looked  to  the 
right. 

An  apparent  dissociation  of  the  ocular  movements,  furthermore,  may 
exist  in  those  cases  in  which  several  muscles  are  paralyzed  in  one  eye 
and  yet  this  eye  is  used  for  fixation.  Here,  as  A.  Graefe  points  out,  the 
secondary  deviations  of  the  sound  eye,  rapidly  changing  their  form  and 
varying  their  amount  as  the  eyes  are  carried  in  different  directions, 
make  it  appear  as  if  the  eyes  were  moving  quite  contrary  to  the  ordinary 
laws  of  association. 


CHAPTER    VI. 

THE  II^TEA-OCULAR  MUSCLES. 

By  JOHN  E.  WEEKS,  M.D. 

IRIS. 

The  iris  is  the  terminal  portion  of  the  vascular  tunic.  Its  free 
border,  which  describes  a  circle  enclosing  the  space  known  as  the  pupil, 
rests  on  the  anterior  surface  of  the  capsule  of  the  lens.  As  the  lens 
surface  occupies  a  plane  anterior  to  the  ciliary  border  of  the  iris,  the  iris 
forms  a  shallow  cone,  the  depth  of  which  depends  on  the  position  of  the 
crystalline  lens.  If  the  lens  is  absent,  the  iris  hangs  in  a  vertical  plane 
and  is  tremulous. 

The  diameter  of  the  iris  (in  small  eyes  9  millimetres,  in  large  eyes 
13  millimetres)  is  approximately  11  millimetres.  The  iris  is  not  of 
uniform  thickness,  but  averages  0.04  millimetre  when  at  rest.  When 
widely  dilated  it  may  double  in  thickness. 

At  the  insertion  of  the  iris  the  posterior  two-thirds  pass  into  the 
ciliary  body.  The  anterior  third  is  continuous  with  the  ligamentum 
pectinatum  (Compare  Page  22). 

Anterior  Endothelial  Layer. — The  iris  is  limited  anteriorly  by  a  layer 
of  flat,  nucleated  endothelial  cells,  which  is  continuous  over  the  surface 
of  the  trabecular  of  the  ligamentum  pectinatum  and  the  posterior  surface 
of  the  cornea. 

Vascular  Stroma  Layer. — The  "  vascular  stroma  layer"  is  composed  of 
bundles  of  connective  tissue  loosely  associated.  This  layer  contains 
numerous  blood-vessels,  nerve-trunks,  and  irregular  lymph-spaces. 
Round,  oval,  and  branching  nucleated  cells,  the  protoplasm  of  which 
becomes  pigmented  in  colored  irides,  are  found  in  this  layer,  as  are  also 
the  muscles  of  the  iris — the  sphincter  and  dilatator  pupilla?. 

The  sphincter  pupillae  muscle  lies  very  near  the  pupillary  margin. 
It  is  a  flat,  circular  band,  measuring  about  one  millimetre  in  width, 
varying  slightly  according  to  the  state  of  contraction,  and  is  about  one- 
tenth  as  much  in  thickness.  It  is  composed  of  bundles  of  non-striated 
muscle-fibres  which  interlace.  The  sphincter  pupilla?  is  separated  from 
the  pupillary  margin  by  the  posterior  pigment  layer  of  the  iris,  which 
extends  a  very  little  way  over  the  stroma  at  the  pupillary  margin  and 

is  seen  as  a  narrow,  black  ring  at  this  point. 

285 


28G  THE    EYE    AND    NERVOUS    SYSTEM 

The  dilatator  pupillae  consists  of  two  or  more  layers  of  long  spindle- 
shaped  cells  possessing  elongated  nuclei.  They  are  undoubtedly  non- 
striated  muscle-fibres.  These  fibres  are  arranged  meridionally  and  lie 
on  the  limiting  membrane.  They  apparently  form  a  continuous  layer 
extending  from  the  ciliary  margin  of  the  iris  to  the  sphincter  pupillaj, 
with  whicli  they  blend,  entering  at  the  outer  border  and  posterior 
surface. 

Lamina  Vitrea,  Membrane  of  Bruch. — This  extremely  delicate  mem- 
brane is  homogeneous  under  low  magnifying  powers,  but  presents  fine 
striations  when  examined  with  high  powers  of  the  microscope.  It  is 
extremely  thin  (0.002  millimetre)  and  is  closely  adherent  to  the  inner 
layer  of  cells  of  the  pigment  layer. 

Pigmented  Layer  (Uvea  of  the  Iris). — This  represents  the  extreme 
anterior  portion  of  the  secondary  eye  vesicle.  The  cells  of  the  deeper 
layer  are  somewhat  flattened,  are  spindle-shaped,  and  are  arranged 
radially  to  the  pupillary  margin.  They  rest  upon  the  lamina  vitrea  of 
the  iris.  The  cells  of  the  outer  layer  are  short,  cylindrical  or  polygonal 
cells.  It  is  now  held  by  some  histologists  that  the  inner  layer  of  cells 
which  forms  the  pars  iridis  retina?  is  concerned  in  the  development  of 
the  sphincter  and  dilator  muscles  of  the  iris.  At  the  ciliary  attach- 
ment, the  pigment  layer  of  the  iris  is  continuous  with  the  pigmented 
layer  of  the  ciliary  body,  the  pars  ciliaris  retina*. 

Vessels  of  the  Iris. — The  arterial  supply  to  the  iris  is  derived  from 
the  long  posterior  ciliary  arteries  and  from  the  anterior  ciliary  arteries. 
The  capillaries  of  the  iris  terminate  in  the  vena;  radical  which  proceed 
radially  to  the  ciliary  body ;  they  empty  into  the  vena;  vorticosa;.  The 
walls  of  the  arteries  are  thick,  due  to  a  preponderance  of  connective 
tissue  without  elastic  fibres.  Muscle-fibres  are  said  to  be  relatively  few. 
The  arteries  and  veins  possess  thick  sheaths  of  connective  tissue,  between 
which  and  the  vessel  wall  proper  the  perivascular  lymph-space  is  found. 

Lymphatics  of  the  Iris. — There  are  no  distinct  lymph-vessels  in  the 
iris.  The  clefts  between  the  tissue  elements  of  the  anterior  border 
layer,  the  irregular  si)aces  between  the  bundles  of  fibres  of  the  vascular 
stroma  and  the  perivascular  spaces,  are  the  lymph-channels  of  the  iris. 
They  communicate  with  the  lymph-spaces  in  the  ciliary  body,  with  the 
lymph-spaces  at  the  filtration  angle,  and  through  the  spaces  of  Fontana 
with  the  anterior  chamber. 

Nerves  of  the  Iris. — The  bundles  of  nerve-fibres  that  enter  the  iris  are 
derived  from  tlie  ciliary  plexus  that  lies  in  the  ciliary  muscle.  Three 
kinds  of  fibres  are  given  off  from  this  plexus:  (1)  The  sensory;  (2) 
the  motor;  (3)  the  sympathetic.     The  motor-fibres  from  the  third  nerve 


THE  INTKA-OCULAR  MUSCLES 


287 


pass  to  the  sjjhincter  pupillse.     The  sympathetic  nerve-fibres  pass  back- 
ward toward  the  dilatator  and  are  supposed  to  supply  it  (Baker). 

Function  of  the  Iris. — The  function  of  the  iris  is  to  regulate  the 
quantity  of  light  that  enters  the  eye  so  that  the  act  of  vision  will  be 
carried  on  with  the  greatest  possible  efficiency.  The  iris  is  a  diaphragm 
which  changes  the  size  of  its  central  opening,  the  pupil,  as  the  conditions 
demand.  The  movements  of  the  iris  are  involuntary  and  are  made 
unconsciously.  They  are  induced  by  stimuli  which  act  through  certain 
"  reflex  arcs."  The  stimuli  which  produce  these  movements  of  the  iris 
are  light,  cutaneous  sensations,  emotions,  convergence,  accommodation, 

Bulbus  sinister 


M.  rectus  superior 


Radix  brevis  seumotoria 


Ganglion  Gasseri 


>M  N.  infratrocblearis 


N.  ethmoidals 


N.  ciliares  longi 


N.  naso-ciliarie 

X.  opticus 
I'lpxus  curoticufi 


Chiasma 


Arteria  carotis 
Tractus  opticus 
N.  oculomotoriua 


N.  trigeminus 

Fig.  1  illustrates  the  anatomical  relation  of  the  short  and  long  ciliary  nerves :  x,  short  root  of  the 
ciliary  ganglion ;  motor  (from  the  oculomotor  nerve)  xx,  long  root  of  the  ciliary  ganglion ;  sensory 
(from  the  trigeminus)  xxx,  sympathetic  root  of  the  ciliary  ganglion  (from  the  carotid  plexus  of  the 
sympathetic  nerve).    (Piltz). 

association  with  certain  movements  of  the  eye  and  eyelids.  Stimulation 
of  the  motor  oculi  nerves  causes  contraction  of  the  sphincter  iridis  with 
consequent  narrowing  of  the  pupil.  Paralysis  of  this  nerve  causes  the 
sphincter  to  relax,  dilating  the  pupil.  Stimulation  of  the  ciliospinal 
centre  in  the  spinal  cord,  or  of  the  superior  portion  of  the  cervical  sym- 
pathetic, causes  contraction  of  the  dilator  of  the  iris,  resulting  in  a 
widening  of  the  pupil  to  a  greater  extent  than  obtains  in  paralysis  of 
the  motor  oculi.  Paralysis  of  the  sympathetic  causes  a  narrowing  of 
the  pupil  (miosis)  greater  than  that  which  obtains  when  the  third 
nerve  is  also  paralyzed. 


288  THE    EYE    AND    NERVOUS    SYSTEM 

Normal  PupU. — The  size  of  the  pupil  varies  greatly  in  different  in- 
dividuals and  in  different  degrees  of  illumination,  ranging  from  1.5 
millimetres  by  bright  illumination  to  9  millimetres  by  weak  illumina- 
tion. The  average  diameter  in  young  adult  life  by  moderate  illumina- 
tion is  4  millimetres.  In  infants  the  pupil  is  small;  in  youth,  large; 
and  in  the  aged  it  is  again  small.  Lange  ^  measured  1000  pupils  in 
patients  of  different  ages  and  sex.  His  conclusions  were  as  follows: 
(1)  The  size  of  tlie  pupil  differs  greatly  in  different  persons.  (2)  As 
a  rule,  women  have  larger  pupils  than  men.  (3)  The  size  of  the  pupil 
decreases  with  age.  (4)  The  width  of  the  pupil  is  less  in  hypermetropic 
eyes  than  in  emmetropes  and  greater  in  myopes  than  in  emmetropes. 
(5)  After  the  fortieth  year  the  difference  in  the  width  of  the  pupil  in 
different  states  of  refraction  is  inconsiderable. 

The  position  of  the  pupil  is,  as  a  rule,  a  little  to  the  nasal  side  of 
tlie  centre  of  the  iris.  The  pupils  change  in  size  symmetrically. 
Marked  inequality  in  the  size  of  the  pupils  is  usually  a  pathological 
phenomenon.  Slight  differences  in  the  size  of  the  pupils  frequently 
exist  under  normal  conditions.  Iwanow  ^  examined  134  military  re- 
cruits, finding  an  equal  width  of  the  pupils  in  only  twelve;  the  right 
pupil  was  larger  in  49 ;  the  left  in  73.  Felton  ^  found  an  inequality 
of  the  pupils  in  28  of  61  persons  examined. 

EXAMINATION    OF    THE    IRIS. 
In  the  examination  of  the  iris  a  number  of  methods  are  employed. 
1.    Simple  Inspection. — For  this,  moderate  illumination  is  necessary. 


Fig.  2.— Priestley-Smith  keratometer. 


Wlien  the  pupils  are  compared,  the  illumination  of  each  eye  should  be 
equal. 

2.  Oblique  Illumination. — This  is  of  service  for  the  purpose  of  throw- 


'  Inaiig.  Dissert.,  Amsterdam,  1901. 

'Vratsch,  No.  7,  1887. 

'  Inaug.  Dissert.,  Bonn,  1895. 


THE  I^sTTEA-OCULAR  MUSCLES 


289 


ing  light  on  various  parts  of  the  retina  in  studying  the  pupillary  light 
reflex. 

3.  Lenses, — These  aid  in  determining  minute  movements  of  the  iris. 
They  may  be  simple  or  compound.  Compound  lenses  are  monocular  or 
binocular.  A  monocular  instrument  that  has  given  some  degree  of 
satisfaction  is  that  devised  by  Howe  of  Buffalo  and  manufactured  by 


Fig.  3. 


Meyrowitz  of  New  York.  The  monocular  instrument  is  inferior  to  the 
binocular,  since  with  the  binocular  compound  lenses  stereoscopic  vision 
is  obtained.  Jackson's  binocular  loupe  adjusted  to  a  head-band  is  a 
very  good  instrument.  Another,  and  probably  the  best,  is  the  corneal 
microscope  of  Czapski,  made  by  Zeiss  of  Jena. 

Methods  of  Measuring  the  Iris. — Measurement  of  the  diameter  or 
width  of  the  iris  may  be  accomplished  by  means  of  a  small  millimetre 
measure,  placing  the  measure  before  the  eyes  as  close  to  the  cornea  as 


Fig.  4.— Circular  pupillometer. 

possible.  The  width  of  the  iris  at  its  line  of  insertion  at  the  ciliary 
body  is  somewhat  hidden  by  the  anterior  border  of  the  sclera  at  the 
sclero-corneal  margin.  This  fact  must  be  taken  into  consideration  in 
making  the  measurements  just  referred  to.  The  keratometer  of  Priest- 
ley-Smith (Eigs.  2  and  3)  may  also  be  employed  for  this  purpose. 

The  size  of  the  pupil  may  be  measured  by  means  of  a  millimetre 
rule,  but,  if  this  is  employed,  it  must  be  remembered  that  the  size  of 
the  pupil  is  actually  greater  than  it  appears  to  be  on  the  rule  and  that 
19 


21)0 


THE    EYE    AND    NERVOUS    SYSTEM 


the  ditference  in  size  is  in  direct  proportion  to  the  distance  of  the  rule 
from  the  pupil,  since  the  observer's  line  of  vision  cuts  the  rule  before 
reaching  the  pupil.  A  very  simple  and  excellent  metliod  is  by  means 
of  the  ordinary  circular  pupillometer  (Fig.  4).  The  little  disk  is 
held  close  to  the  cornea  and  is  rotated  until  an  aperture  in  the  margin  of 
the  disk  corresponds  to  the  size  of  the  pupil.  The  diameter  of  this 
opening,  which  is  marked  on  the  disk,  is  then  recorded. 

Tliero  are  many  other  pupillometers  that  may  be  employed.     The 


O  O  O  o  o  o 


20  JO 


JO  so  70  So 


Fig.  5.— Hirschberg  pupillometer. 

pupillometer  of  Soleberg  Wells,  of  Hirschberg  (Fig.  5),  of  Landolt,"*  of 

Schirmer,^  etc.,  but  these  possess  little  advantage  over  the  simple  one 

first  mentioned. 

LIGHT    REFLEXES. 

The  reaction  of  tlie  pupil  to  light  is  excited  (1)  by  the  direct 
entrance  of  light,  known  as  the  direct  light  reflex;  (2)  by  the  entrance 
of  light  into  one  eye,  the  observed  eye  being  shaded — ^the  indirect  or 
consensual  light  reflex.  Both  reflexes  are  due  to  the  integrity  of  what 
is  termed  the  light  reflex  arc,  which  consists'of  fibres  running  from  the 
eye  to  the  brain,  known  as  centripetal  fibres,  and  fibres  running  from  the 
brain  (nucleus  of  the  third  nerve)  to  the  iris,  centrifugal  fibres. 

The  centripetal  fibres  have  their  origin  in  the  retina  (according  to 
(^oppez "  in  the  amacrine  cells,  but  this  is  not  fully  proven),  being 
apparently  most  plentiful  in  the  region  of  the  posterior  pole,  diminish- 
ing in  number  towards  the  periphery,  apparently  having  the  same  dis- 
tribution as  the  fibres  concerned  in  vision,  that  is,  those  that  decussate 
come  from  the  nasal  half  of  each  retina,  the  non-decussating  from  the 
temporal  half.  They  pass  into  the  optic  nerve  and,  according  to  von 
Gudden,  von  Monakow,  Bernheimer,^  and  others,  can  be  differentiated 


*  Landolt,  De  Wecker  et  Landolt,  Traite  complet  d'OphthalmoIogie,  p.  942. 
'  Schirmer,  Arcliiv  f.  Ophth.,  xl.  5. 

•  Coppez,  Revue  g^nC'rale,  February  28,  1903, 

^  Bemheimer,  Archiv  f.  Ophth.,  Bd.  xlvii.  S.  1. 


PLATE  II. 


(Fig.  6.) 
Pupillary  Light  Reflex  Arc ;  Sympathetic,  Cortical,  and  Sensory  Dilator  Tracts. 

Rvd  and  j/cccti.— Centripetal  tract  of  the  light  reflex  arc  (crossing  at  the  sphincter  centres  not 
shown. ) 

YeUow. — Centrifugal  tract  of  the  light  reflex  arc. 

Blue. — Sympathetic  tract  containing  the  nerve-fibres  which  innervate  the  dilator  pupillse. 

/J/acA-.— Cortical  and  sensory  tracts.    (Uhthoff.) 


THE   INTKA-OCULAR  MUSCLES  291 

from  the  fibres  that  have  to  do  with  vision  by  their  larger  size.  Passing 
into  the  cliiasni,  thej  there  undergo  semi-decussation  and  proceed  along 
the  optic  tracts  to  the  outer  geniculate  bodies,  where,  according  to 
Bemheimer,  they  bend  inward,  pass  to  the  anterior  part  of  the  anterior 
corpora  quadrigemina,  and  thence  to  the  sphincter  nucleus  in  the  an- 
terior portion  of  the  nucleus  of  the  third.  Bernheimer  is  not  positive 
that  the  centripetal  fibres  pass  into,  but  thinks  that  they  may  end  in 
arborations  at  the  sphincter  nucleus.  Experimental  and  pathological 
data  at  hand  make  it  probable  that  the  centripetal  fibres  of  the  light 
reflex  arc  do  not  pass  through  the  anterior  corpora  quadrigemina, 
Moeli  *  reports  a  case  of  absence  of  light  reflex  in  which  the  lesion  was 
found  to  be  a  tumor  involving  both  sides  of  the  posterior  walls  of  the 
third  ventricle,  making  it  probable  that  the  fibres  referred  to  pass  in 
these  walls.  In  this  case  the  pupils  were  5  millimetres  in  diameter, 
equal  in  size;  vision,  motility  of  eyes,  and  convergence  (page  301) 
reflex  normal. 

The  experiments  of  Terrier  and  Turner^  seem  to  prove  conclusively 
that  the  fibres  do  not  pass  into  the  corpora  quadrigemina.  Young 
monkeys  were  employed.  The  corpora  quadrigemina  were  exposed  by 
opening  the  skull  and  removing  the  occipital  lobe  of  the  brain  backward 
from  the  parieto-occipital  fissure.  The  anterior  quadrigeminal  bodies 
were  then  destroyed  with  the  cautery.  This  did  not  abolish  the  pupillary 
light  reflex.  The  movements  of  the  iris  were  affected  only  when  the 
lesion  extended  into  the  underlying  Sylvian  gray  matter.  Clinical 
evidence  to  this  effect  ^^  is  also  obtainable. 

Bach  ^*  is  of  the  opinion  that  the  centripetal  bundle  of  fibres  divides 
at  or  near  the  external  geniculate  body  one  part  proceeding  to  a  centre 
in  the  lower  part  of  the  medulla,  which  he  terms  the  inhibitory  centre, 
fibres  returning  from  this  centre  to  the  sphincter  nucleus.  This  sup- 
position is  not  proven  and  in  the  light  of  the  experiments  of  Ruge,'^ 
who  divided  the  medulla  some  distance  above  its  lower  extremity  with- 
out abolishing  or  changing  the  pupillary  light  reflex,  it  is  highly  prob- 
able that  this  centre  does  not  exist. 

In  regard  to  the  effect  of  lesions  of  this  tract,  the  experiments  of 
von  BechtereM'  ^^  on  dogs  led  to  the  following  conclusions: 


'Moeli,  Archiv  f.  Psychiatric,  Bd.  xviii.  S.  1. 

'  Ferrier  and  Turner,  Brain,  1901,  vol.  xxiv.  p.  27. 

'*  Goldzieher  and  Bechterew,  Archiv  f.  w.  ges.  Physiol.,  Bd.  xxxi..  S.  60. 

"  Bach,  Graefe's  Archiv,  Bd.  Ivii.,  H.  2,  p.  239. 

"Ruge,  Graefe's  Archiv,  1902,  Bd.  liv.',  H.  3. 

"Von  Bechterew,  Archiv  f.  \v.  ges.  Physiol.,  Bd.  xxxi.,  S.  60. 


292  THE    EYE    AND    NERVOUS    SYSTEM 

1.  Monolateral  lesion  of  the  sphincter  nucleus  causes  loss  of  the 
light  reflex  of  the  eye  on  the  same  side  and  loss  of  the  consensual 
reaction  in  the  opposite  eye. 

2.  A  lesion  of  one  side  of  the  third  ventricle  induces  one-sided  loss 
of  light  reflex  with  good  vision. 

3.  A  lesion  affecting  both  sides  of  the  wall  of  the  third  ventricle 
causes  loss  of  light  reflex  in  both  eyes,  with  normal  sight. 

Decussation  of  the  Fibres  of  the  Pupillary  Light  Reflex  Arc. — 
The  question  of  semi-decussation  in  the  chiasm  is  apparently  settled. 
Although  tliere  is  as  yet  no  positive  clinical  proof,  the  experiments  of 
Bernheimer  are  conclusive.  Bemheimer  divided  the  tractus  of  one 
side  in  monkeys  and  then  found  that  the  pupillary  light  reflex,  both 
direct  and  consensual,  was  preserved.  Decussation  or  intercommunica- 
tion at  the  sphincter  nuclei  is  evident  both  from  clinical  and  from 
experimental  observation.  The  case  of  S.  Weir  Mitchell  **  furnishes 
almost,  if  not  quite,  conclusive  clinical  evidence.  The  chiasm  was  cut 
in  half  sagittally  by  a  tumor  which  divided  all  but  a  very  few  fibres. 
There  was  bitemporal  hemianopsia  with  retention  of  the  pupillary  light 
reflex.  Bemheimer  divided  the  chiasm  in  monkeys  by  sagittal  incision 
and  demonstrated  the  retention  of  the  pupillary  light  reflex.  Whether 
the  pupillary  light  reflex  was  preserved  in  the  nasal  portions  of  the 
retinae  is  not  stated.    Theoretically,  this  should  not  occur. 

Lesions  Affecting  the  Pupillary  Light  Reflex  Arc;    Centripetal  Fibres. 

1.  Lesions  affecting  the  retina  or  optic  nerve  on  one  side  producing 
blindness  cause  loss  of  direct  pupillary  light  reflex  on  the  affected  side 
and  consensual  light  reflex  on  the  opposite  side.  The  consensual  reaction 
is  preserved  on  the  blind  side.  With  uniform  illumination  the  pupils 
are  of  the  same  size,  as  a  rule;  in  some  cases  the  pupil  of  the  blind 
eye  is  a  trifle  larger  than  its  fellow  in  moderate  and  bright  illumination 
when  both  eyes  are  equally  exposed.  If,  by  moderate  illumination,  the 
seeing  eye  is  covered  the  pupil  of  the  blind  eye  dilates.  If  there  is 
amblyopia  (blindness  not  complete)  the  direct  pupillary  light  reflex  in 
the  affected  eye  and  the  consensual  pupillary  light  reflex  of  the  fellow- 
eye  are  not  entirely  abolished,  but  are  less  active  than  in  the  normal 
eye. 

2.  Sagittal  section  of  the  chiasm  will,  since  the  distribution  of  the 
fibres  of  the  pupillary  light  reflex  in  the  retina  correspond  to  the  distri- 
bution of  the  fibres  concerned  in  vision,  cause  loss  of  direct  and  consen- 
sual pupillary  light  reflex  in  each  eye  when  the  nasal  half  of  the  retina 

"Jour,  of  Nerv.  and  Ment.  Dis.,  January,  1889. 


THE  INTRA-OCULAR  MUSCLES  293 

only  is  illuminated.^^  Illumination  of  the  temporal  halves  of  the  retinae 
elicit  both  direct  and  consensual  pupillary  light  reflex.  With  this  lesion 
the  pupils  are  slightly  larger  than  normal. 

3.  Sagittal  section  of  the  chiasm  and  lesion  of  one  optic  nerve,  with 
consequent  blindness  on  one  side  and  temporal  hemianopsia  on  the  other 
side,  cause  complete  loss  of  direct  pupillary  light  reflex  in  the  blind  eye 
and  of  consensual  pupillary  light  reflex  in  the  seeing  eye;  complete 
loss  of  direct  pupillary  light  reflex  in  the  seeing  eye  and  loss  of  consen- 
sual puf)illary  light  reflex  in  the  blind  eye  when  the  nasal  half  of  the 
seeing  eye  is  illuminated,  and  retention  of  the  direct  pupillary  light  re- 
flex in  the  hemianopic  eye  and  of  consensual  pupillary  light  reflex  in  the 
blind  eye  when  the  temporal  half  of  the  retina  is  illuminated.  The 
pupil  of  the  blind  eye  will  be  slightly  larger  than  that  of  the  fellow- 
eye  by  bright  and  moderate  illumination,  but  of  the  same  size  in  the 
dark  (as  may  be  demonstrated  by  flash-light  photography). 

4.  Lesion  of  the  tractus  on  the  right  side  produces  left  homonymous 
hemianopsia,  with  loss  of  the  direct  pupillary  light  reflex  in  the  illu- 
minated eye  and  loss  of  the  consensual  pupillary  light  reflex  in  the 
fellow-eye  (absence  of  the  Wernicke  pupillary  light  reflex)  when  the 
right  half  of  the  retina  of  either  eye  is  illuminated.  Illumination  of 
the  left,  or  seeing  half,  of  either  retina  elicits  direct  pupillary  light 
reflex  of  the  illuminated  eye  and  consensual  pupillary  light  reflex  of 
the  fellow-eye.  The  pupils  in  this  condition  are  slightly  larger  than 
normal  (other  things  being  equal)  by  moderate  illumination. 

5.  Lesion  of  a  tractus  and  destruction  of  the  same  half  of  the  chiasm 
or  of  the  optic  nerve  on  that  side  produce  the  same  clinical  conditions 
as  those  described  in  3. 

6.  The  lesions  heretofore  described  are  accompanied  by  blindness  of 
the  corresponding  parts  of  the  retinae.  If  it  were  possible  to  destroy  by 
lesion  the  bundle  of  fibres  of  the  pupillary  light  reflex  arc  between  an 
optic  tractus  and  the  sphincter  nucleus,  there  would  be  no  blindness,  but 
there  would  be  loss  of  pupillary  light  reflex  corresponding  to  that  pro- 
duced by  lesion  of  one  tractus  if  one  side  only  was  involved  and  com- 
plete loss  of  direct  and  consensual  pupillary  light  reflex  on  both  sides 
if  the  bundles  on  both  sides  were  destroyed.  The  pupils  would  remain 
of  normal  size.  Lesions  at  this  point  do  not  abolish  the  convergence 
light  reflex. 

Sphincter  Nuclei. — The  sphincter  nuclei,  according  to  Bernheimer, 


"In  testing  the  reflex  with  such  a  lesion,  the  difficulty  in  excluding  light   (see 
page    302. )   from  the  opposite  part  of  the  retina  must  be  borne  in  mind. 


294  THE    EYE    AND    NERVOUS    SYSTEM 

Parlia,  Kahler  and  Pick,  and  others,  lie  in  the  anterior  part  of  the 
nucleus  for  the  third,  at  the  posterior  boundary  of  the  third  ventricle, 
separated  from  the  nucleus  for  the  ciliary  muscle.  Bernheiraer  de- 
scribes a  central  communication  of  the  two  sphincter  nuclei,  and  is  of 
the  opinion  that  the  communication  is  by  means  of  processes  from  the 
neurons  of  either  nucleus. 

Centrifugal  Fibres  of  the  Pupillary  Light  Reflex  Arc. — These 
fibres  arise  from  the  sphincter  nucleus  of  the  side  corresponding  to  the 
iris  which  they  are  to  innervate,  pass  into  the  underlying  tissue  of  the 
pons,  where  they  are  joined  by  fibres  from  the  ciliary  nucleus.  They 
then  pass  into  the  trunk  of  the  third  nerve  occupying,  according  to 
Ackerman,  the  central  portion  of  this  nerve-trunk;  then  by  the  short 
root  to  the  ciliary  ganglion;  then  by  the  short  ciliary  nerves  (five  to 
seven  in  number)  to  the  posterior  part  of  the  globe,  passing  through 
the  sclera,  and,  forward  between  choroid  and  sclera,  into  the  ciliary 
body,  from  the  anterior  portion  of  which  they  pass  into  the  iris  tissue 
and  to  the  sphincter. 

Lesions  of  the  Centrifugal  Fibres. — Since  the  centrifugal  fibres  after 
leaving  the  sphincter  nuclei  do  not  decussate,  and  since  they  are  joined 
by  the  fibres  from  the  ciliary  nucleus  soon  after  their  origin  and  are 
incorporated  with  the  trunk  of  the  third  nerve  before  it  leaves  the  pons, 
it  is  possible  to  have  an  isolated  lesion  of  the  pupillary  light  reflex  arc 
only  at  or  very  near  the  sphincter  nucleus.  Caspar  ^®  reports  a  case  as 
follows:  P.,  aged  38  years;  loss  of  direct  and  consensual  pupillary  re- 
flex with  preservation  of  vision;  pupil  responds  to  convergence.  He 
places  the  lesion  between  the  sphincter  nucleus  and  the  point  where 
the  fibres  from  the  ciliary  nucleus  join  the  pupillary  light  reflex  bundle. 
Wilder  ^^  reports  two  cases  of  monocular  iridoplegia,  in  both  of  which 
there  was  paresis  of  accommodation.  There  was  complete  preservation 
of  convergence  pupillary  reflex.  Such  a  lesion  produces  loss  of  pupillary 
light  reflex  direct  and  consensual  on  the  side  affected.  It  produces 
paralysis  of  the  sphincter  muscle,  but  does  not  affect  direct  or  consensual 
reaction  in  the  fellow-eye.  Affections  of  this  bundle  in  other  parts  of 
its  course  are  almost  invariably  associated  with  other  third-nerve  paraly- 
ses. If  the  centrifugal  pupillary  light  reflex  bundle  on  one  side  only 
is  affected,  the  pupil  on  the  corresponding  side  is  larger  than  its  fellow, 
except  in  the  dark,  when  (flash-light  photography)  they  are  of  the  same 
size.     If  the  bundle  on  each  side  is  involved,  both  pupils  are  dilated  in 


"Archiv  f.  Augenheilk.,  Bd.  xxvii.  p.  291. 
"Ann.  of  Ophthal..  April,  1897. 


THE  INTKA-OCULAR  MUSCLES  295 

all  degrees  of  illumination.  They  should  be  equally  so,  but  Heddaeus 
states  that  anisocoria  sometimes  exists. 

Complete  section  of  the  third  nerve  produces  total  loss  of  the  pupil- 
lary light  reflex  and  consensual  light  reflex  on  the  affected  side. 

Convergence  Pupillary  Reflex. — When,  under  normal  conditions, 
the  eyes  change  their  regard  from  a  distant  to  a  near  object,  or,  in  other 
words,  when  the  visual  lines  are  simultaneously  deflected  from  paral- 
lelism to  extreme  convergence,  the  pupils  contract  very  markedly.  This 
phenpmenon  is  called  the  convergence  pupillary  reflex.  It  is  most 
pronounced  in  eyes  whose  irides  are  most  active  and  in  those  which, 
with  normal  irides,  have  large  pupils,  as  in  children  and  young  adults. 
In  the  aged,  whose  pupils  are  small,  particularly  in  those  whose  cervical 
sympathetic  nerves  are  paralyzed,  and  in  tabetic  patients,  as  well  as  in 
all  who  possess  the  Argyll-Robertson  pupil,  the  convergence  pupillary 
reflex  causes  but  slight  movement  of  the  iris. 

This  pupillary  reflex  is  often  described  as  a  convergence  and  accom- 
modation reflex  and  sometimes  as  an  accommodation  reflex.  It  is  very 
easy  to  demonstrate  the  fact  that  the  reflex  is  much  more  closely  related 
to  convergence  than  to  accommodation.  In  many  cases  it  is  impossible 
to  determine  that  accommodation  influences  the  pupil  at  all.  The  diflS- 
culty  in  eliminating  convergence  from  accommodation  makes  it  almost 
impossible  to  determine  just  what  influence  accommodation  has  in  the 
production  of  this  reflex,  but  it  is  certain  that  some  pupillary  reaction 
accompanies  accommodation  in  many  individuals. 

The  amplitude  of  the  contraction  depends  on  the  size  of  the  pupil 
and  on  the  activity  of  the  iris. 

In  the  emmetropic  and  hypermetropic  eye,  the  amplitude  and 
promptness  of  the  reaction  is  greater  than  in  myopes. 

In  pupils  of  unequal  size,  the  amplitude  of  movement  is  greater  in 
the  eye  wath  the  larger  pupil,  other  conditions  being  equal. 

If  one  eye  is  amaurotic,  the  fellow-eye  being  good,  the  reflex  is  the 
same.  Contraction  of  the  pupil  also  occurs  on  convergence  if  both 
eyes  are  amaurotic,  possibly  not  to  so  great  a  degree  as  in  seeing  eyes. 

If  there  is  no  reaction  of  the  pupil  in  convergence,  paralysis  of  the 
sphincter  is  to  be  suspected. 

This  pupillary  reflex  disappears,  as  a  rule,  with  absence  of  con- 
vergence, whether  the  absence  is  voluntary,  that  is,  is  not  exercised,  as 
in  cases  of  loss  of  one  eye,  in  which  case  the  act  of  convergence  is  often 
abandoned  after  the  lapse  of  some  time,  or  in  true  absence  or  paralysis 
of  convergence. 

The  frequent  association  of  loss  of  accommodation,  paralysis  of  the 


296  THE    EYE    AND    NERVOUS    SYSTEM 

ciliary  muscle  and  of  the  sphincter  pupillae,  with  loss  of  convergence, 
points  to  the  close  association  of  the  sphincter  and  ciliary  centres  in  the 
nucleus  of  the  third  with  the  portion  of  the  brain  controlling  con- 
vergence, but  no  anatomical  convergence  centre  has  as  yet  been  demon- 
strated. According  to  Ileddeaus,  destruction  of  the  left  sphincter 
nucleus  with  the  fibres  coming  to  it  and  going  from  it  causes  paralysis 
of  the  pupillary  light  reflex  in  that  eye,  but  does  not  cause  paralysis  of 
the  convergence  reflex.  Division  of  the  third  nerve  abolishes  the  con- 
vergence reflex.  This  makes  it  probable  that  the  impulses  necessary 
for  the  production  of  the  pupillary  light  reflex  and  convergence  reflex 
are  conducted  along  separate  fasciculi  of  nerve-fibres  coming  from 
separate  centres. 

Heddaeus  holds  that  this  reaction  is  synergistic  with  convergence. 
The  remarkable  experiments  of  Marian  ^*  show  conclusively  that  the 
reflex  is  not  necessarily  associated  with  contraction  of  the  interni,  but 
do  not  disprove  the  necessity  for  the  integrity  of  the  convergence  action 
of  muscles  innervated  by  the  third  for  its  manifestation.  Marian, 
operating  on  the  eyes  of  monkeys,  transplanted  the  tendon  of  the 
superior  oblique  to  the  insertion  of  the  intemus  and  the  tendon  of  the 
internus  to  the  insertion  of  the  superior  oblique.  Divergence  was  the 
immediate  result,  but  subsequently  the  eye  could  be  made  to  converge 
with  its  fellow,  showing  that  the  convergence  does  not  depend  on  the 
integrity  of  the  internus.  Again,  the  place  of  attachment  of  the  in- 
ternus was  exchanged  with  that  of  the  externus  of  the  right  eye.  On 
movement  of  both  eyes  to  the  right  (contraction  of  both  interni)  there 
was  no  contraction  of  the  pupils.  The  extemi  were  made  to  take  the 
place  of  the  interni  and  eventually  convergence  became  possible.  This 
was  accompanied  by  contraction  of  the  pupil,  showing  that  the  centre 
for  the  interni  in  the  nucleus  of  the  third  has  no  intimate  connection 
with  the  centre  for  the  convergence  pupillary  reflex.  The  experiments 
do  not  disprove  the  connection  of  the  superior  and  inferior  recti,  which 
undoubtedly  assist  in  convergence,  with  the  convergence  pupillary  re- 
flex. Consequently,  Marian's  conclusion  to  that  effect,  although  possibly 
correct,  is  not  warranted.  These  experiments  serve  to  emphasize  the 
fact  that  little  is  known  of  the  production  of  the  convergence  pupillary 
reflex.  Marian  thinks  it  possible  that  an  inhibiting  impulse  may  affect 
the  dilator  centre  and  permit  of  a  passive  contraction  of  the  sphincter. 
The  opinion  held  by  some  observers  that  the  convergence  pupillary  re- 
flex depends  on   an   impulse  originating  in   a   cortical  centre  siraul- 


Deutsch.  Zeitsehr.  f.  Nervenheilk.,  1903,  Bd.  xxiv.,  S.  274. 


THE  INTRA-OCULAR  MUSCLES  297 

taneously  with  the  voluntary  impulse  for  convergence  operating  through 
the  intermediate  nucleus  of  the  third  nerve  may  be  correct.  Loss  of 
convergence  pupillary  reflex  in  one  or  both  eyes  has  been  observed 
without  loss  of  convergence.^^  This  fact  implies  a  centre  acting 
either  directly  to  cause  contraction  of  the  sphincter  pupilla^  or  indi- 
rectly to  inhibit  (Marian)  the  action  of  the  sympathetic  on  the  dilatator 
pupill?e. 

In  affections  of  convergence  light  reflex,  the  following  condition  may 
obtain : 

(a)  Paralysis  of  convergence  light  reflex  on  one  side  without  other 
paralyses ;  retention  of  pupillary  light  reflex  direct  and  consensual,  on 
both  sides.  The  pupil  on  the  affected  side  is  usually  larger  than  normal 
by  strong  illumination. 

(b)  Paralysis  of  convergence  light  reflex  on  both  sides  without  other 
paralyses  (other  reflexes  remaining  intact). 

Lesions  causing  paralysis  of  the  third  in  any  part  of  its  course  cause 
loss  of  the  convergence  pupillary  reflex. 

Sympathetic  Dilator  Tract. — The  fibres  which  innervate  the  dila- 
tator pupillae  have  their  origin  in  the  ciliospinal  centre  of  the  spinal 
cord,  situated  in  the  anterior  cornu  ^^  in  the  lower  part  of  the 
cervical  and  upper  part  of  the  dorsal  cord.  According  to  Bach, 
this  centre  is  connected  with  the  sphincter  nucleus  of  the  third.  A 
centre  in  the  medulla  is  supposed  by  Bach  to  be  inhibitor^'  to  both  of  the 
centres  with  which  it  is  connected,  namely,  with  the  ciliospinal  centre 
and  the  sphincter  nucleus  of  the  third.  Ruge  ^^  repeated  the  experi- 
ments of  Bach  on  rabbits  and  found  that  after  complete  division  of  the 
medulla  oblongata  perceptible  pupillary  reaction  could  be  obtained, 
apparently  conclusively  disproving  Bach's  idea  of  an  inhibitory  pupil- 
lary reflex  centre  in  the  medulla. 

Fibres  from  the  dilator  centre  in  the  cord  pass  by  the  rami  com- 
municantes  which  pass  out  of  the  spinal  canal  with  the  seventh  and 
eighth  cervical  and  first  and  second  dorsal  nerves,  and  enter  the  cervical 
sympathetic.     The  motor  fibres  reach  the  superior  cervical  ganglion, 

'"  The  writer  has  recently  observed  a  case  in  which,  with  double  choked  disk 
accompanied  by  headache,  there  was  preservation  of  the  pupillary  light  reflex,  both 
direct  and  consensual,  no  paralysis  of  accommodation  or  of  the  power  of  convergence, 
but  complete  loss  of  convergence  pupillary  reflex.  At  the  same  time  there  was 
paresis  of  the  external  rectus  of  the  right  eye.  Under  mixed  treatment  the  eyes 
resumed  the  normal  condition.  This  would  point  to  a  convergence  centre  entirely 
separate  from  the  sphincter  or  accommodation  nucleus. 

"  Coppez,  Revue  gener.,  February  28,  1903. 

**Graefe's  Archiv,  Bd.  liv.,  H.  iii.,  S.  483. 


298  THE    EYE    AND    NERVOUS    SYSTEM 

from  which  similar  fibres  pass  to  the  internal  carotid  plexus ;  thence  to 
the  Gasserian  ganglion  and  in  the  superior  division  of  the  fifth  into  the 
nasal  branch  and  by  way  of  the  long  ciliary  nerves  to  the  globe.  These 
nerve  filaments  pierce  the  sclera  near  the  optic  nerve  and  pass  between 
sclera  and  chorioid  forward  into  the  ciliary  body,  the  outer  layers  of 
which  they  pierce,  reaching  the  iris. 

It  is  probable  that  an  interruption  of  the  pupillo-dilator  fibres  takes 
place  in  the  superior  cervical  ganglion,  as  stimulation  by  non-electrical 
agents  produces  dilatation  of  the  pupil,  which  does  not  occur  on  irrita- 
tion of  non-sensitive  tissues  unless  the  faradic  current  is  employed. 

Anderson,^^  experimenting  on  cats,  after  excision  of  the  ciliary 
ganglion,  also  after  section  of  the  short  ciliary  nerves  which  he  con- 
siders equivalent  to  section  of  the  third  nerve  (motor  oculi),  could  not 
obtain  a  contraction  of  the  pupil  by  light  or  by  any  form  of  stimulus,  but 
obtained  marked  dilatation  of  the  pupil  (1)  by  stimulating  the  proximal 
end  of  the  sciatic  nerve  by  means  of  the  faradic  current;  (2)  by  pinch- 
ing the  skin;    (3)  by  stroking  the  hair. 

The  cord  was  divided  at  the  level  of  the  first  cervical  vertebra 
and  the  medulla  and  brain  destroyed.  Faradic  stimulation  of  the 
proximal  end  of  the  sciatic  nerve  produced  distinct  dilatation  after  a 
one  per  cent,  solution  of  eserin  had  been  instilled,  demonstrating  the 
fact  that  the  dilatation  could  not  be  due  to  inhibition  of  the  pupillo-con- 
strictor  tone.  Section  of  the  cord  below  the  second  dorsal  nerves 
abolishes  all  dilatation  reaction  to  stimuli  applied  below  this  point. 
These  experiments,  which  have  also  been  corroborated  by  others,  prove 
that  the  dilator  nerve  impulses  pass  through  the  centre  in  the  cervico- 
dorsal  cord  and  through  the  upper  cervical  ganglion.  Parsons  "^  has 
shown  that  stimulation  of  the  cortex  of  the  brain  has  no  effect  on  the 
pupil  after  division  of  the  third  and  sympathetic  nerves,  but,  if  the 
sympathetic  alone  is  divided,  dilatation  may  be  obtained,  which  Parsons 
explains  as  being  due  to  an  inhibitory  effect  on  the  third  nerve. 

The  experiments  of  Budge,^"*  ITensen  and  Volkers  (1868),  O.  Lan- 
gendorf  (1864),  and  others  prove  that  stimulation  of  the  ciliary  gang- 
lion and  short  ciliary  nerves  causes  contraction  of  the  pupil,  and  that 
stimulation  of  the  long  ciliary  nerves  causes  dilatation  of  the  pupil. 
Piltz's  experiments  demonstrate  the  fact  that  the  contraction  of  the 
pupil  resulting  from  stimulation  of  the  short  ciliary  nerves  comes  on 
suddenly  and  subsides  suddenly  on  removal  of  the  stimulus;    that  on 


Journal  of  Physiol.,  1903,  .30. 
Ibid.,   1901,  xxvi.  p.   .300. 
'Die  beweg.  d.  Iris,  Braunschweig,  1855. 


THE   INTRA-OCrLAR  MUSCLES  299 

stimulation  of  the  long  ciliary  nerves  the  dilatation  comes  on  in  about 
two  seconds  and  subsides  very  slowly  on  removal  of  the  stimulus. 

The  effect  on  the  pupil  by  stimulation  of  the  sympathetic  is  to 
produce  marked  dilatation  of  the  pupil  on  the  side  affected,  which  is 
greater  than  the  dilatation  after  abolition  of  the  function  of  the  third 
nerve,  but  is  not  so  great  as  is  obtained  by  the  use  of  potent  mydriatics. 
The  dilatation  after  division  of  the  third  on  stimulation  of  the  sympa- 
thetic reaches  almost,  if  not  quite,  maximum  dilatation,  showing  the 
effect  of  the  contraction  tone  of  the  sphincter  under  normal  conditions 
of  the  third.  Section  of  the  cervical  sympathetic,  removal  of  the 
superior  cervical  ganglion  or  section  of  the  long  ciliary  nerves  produces 
contraction  of  the  pupil  on  the  side  affected  without  entirely  abolishing 
the  pupillary  light  reflex.     The  miosis  is  maximal. 

Braunstein's  experiments  ^^  caused  him  to  conclude  as  follows : 

1.  Stimulation  of  the  outer  of  the  two  long  ciliary  nerves  causes  a 
dilatation  of  the  pupil  in  the  outer  upper  half. 

2.  Stimulation  of  the  ocular  end  of  the  divided  cervical  sympathetic 
nerve  produces  an  even  dilatation  of  the  pupil,  not  maximal. 

3.  Stimulation  of  the  divided  cervical  sympathetic  after  division  of 
the  outer  of  the  two  long  ciliary  nerves  produces  dilatation  of  the  inner 
lower  half  of  the  pupil  (Compare  Chapter  I,  pp.  5,  8). 

Examination  of  the  Light  Reflex. 

If  the  eye,  after  being  covered,  is  quickly  uncovered  and  exposed  to 
moderately  bright  illumination,  the  pupil  rapidly  contracts  and  reaches 
its  minimum  size  (primary  narrowing).  There  is  then  a  slight  dilata- 
tion (secondary  dilatation)  and  perhaps  a  second  slight  contraction  be- 
fore the  size  becomes  fixed.  This  phenomenon  may  be  even  more  plainly 
manifest  in  the  change  from  parallelism  of  the  visual  axes  (distance 
vision)  to  strong  convergence  (near  vision).  The  oscillation  of  the 
iris  under  these  normal  conditions  resembles  in  small  degree  the  phe- 
nomenon know4i  as  "  hippus."  The  primary  contraction  may  occur 
very  quickly;  indeed,  so  quickly  that  it  may  not  be  noticed,  the  second 
dilatation  being  the  first  movement  seen.  Under  these  circumstances 
the  movement  may  be  readily  mistaken  for  the  "  paradoxical"  pupillary 
reaction. 

Examination  for  the  Direct  Light  Reflex. — If  there  is  any  doubt 
regarding  the  reaction  of  the  pupil  to  light,  the  following  method  should 
be  employed.  The  patient  should  be  placed  in  a  dark  room  facing  and 
''  fixing"  a  single  source  of  light.     The  light  should  be  not  more  than 

"  Ein  Beitriige  z.  Kent.  d.  Innervation  d.  Irisbevvegungen,  Charkow,  1893. 


300  THE    EYE    AND    NERVOUS    SYSTEM 

one  metre  from  the  patient.  One  eye  of  the  patient  is  excluded  from 
the  light  by  covering  it  with  the  hand  or  some  opaque  object.  By  means 
of  a  convex  lens  (15  to  20  D.)  the  light  is  now  focussed  in  the  exposed 
eye,  the  patient  not  being  permitted  to  look  in  an}'  other  direction  than 
in  a  direct  line  to  the  light.  The  light  may  be  thrown  into  the  eye  and 
shut  out  of  the  eye  at  the  will  of  the  examiner.  The  position  and 
direction  of  regard  eliminate  any  influence  upon  the  pupil  by  accom- 
modation or  by  convergence.  The  strong  illumination  of  the  eye  thus 
obtained  makes  it  possible  to  determine  with  certainty  the  presence  or 
absence  of  the  pupillary  light  reflex.    Points  to  determine  are : 

(1)  Are  the  pupils  of  normal  size  when  the  eyes  are  in  shadow  and  in 
light? 

(See  normal  pupil.)  The  pupils  may  be  larger  than  normal  and  of 
equal  size  in  shadow  if  under  the  influence  of  a  mydriatic.  They  may 
be  smaller  than  normal  and  equal  in  size  in  light  (a)  if  the  irides  are 
equally  inflamed;  (b)  if  they  are  atrophic;  (c)  if  there  is  disease  of 
the  sympathetic  affecting  both  sides;  (d)  in  senile  atrophy;  (e)  if 
under  the  influence  of  a  miotic. 

The  pupils  may  be  larger  than  normal  when  exposed  to  hright  light 

(a)  in  loss  of  central  acuity  of  vision  and  in  total  blindness  due  to  a 
lesion  peripheral  to  the  corpora  quadrigemina ;  (b)  when  there  is  a 
lesion  involving  the  centripetal  fibres  of  the  pupillary  light  reflex  arc 
(page  292);  (c)  in  cases  of  glaucoma  w^ith  increased  tension;  (d) 
when  belladonna  or  other  mydriatics  are  being  taken  internally  in 
sufficient  quantity  or  when  mydriatics  have  been  instilled  into  the  eyes ; 
(e)  when  paralysis  of  the  pupillary  constrictor  fibres  exists,  or  (f)  when 
there  is  a  lesion  of  the  pupillary  light  reflex  arc  at  any  point. 

The  pupils  may  be  smaller  tlian  normal  when  exposed  to  bright 
light  (a)  when  miosis  has  been  induced  by  a  miotic  remedy,  as  by 
eserin,  pilocarpin,  etc, ;  (b)  when  the  sympathetic  on  one  or  both  sides 
is  paralyzed ;  (c)  in  some  cases  of  inflammation  affecting  both  irides. 

(2)  Are  the  pupils  of  equal  size  in  dilation  and  in  contraction? 

The  pupils  will  be  of  unequal  size  in  shadow,  the  pupil  of  the  affected 
eye  being  larger  (a)  when  a  mydriatic  has  been  used  in  one  eye  only; 

(b)  when  there  is  paralysis  of  the  centripetal  fibres  of  the  pupillary 
light  reflex  arc  (Fig.  G) ;  (c)  in  disease  affecting  the  tissues  of 
the  irides  unequally.  In  paralysis  of  the  dilator  tract  (see  Fig.  6) 
affecting  one  side  the  pupil  of  the  non-affected  eye  will  be  the  larger. 

The  pupils  will  be  of  unequal  size  in  contraction  by  bright  light, 
being  larger  (a)  when  a  mydriatic  has  been  used  in  one  eye;  (b)  when 
the  centripetal  light  reflex  tract  on  one  side  has  been  interrupted;    (c) 


THE  IXTRA-OCULAR  MUSCLES  301 

in  glaucoma  with  plus  tension,  affecting  one  side  more  than  the  other ; 
(d)  temporarily,  following  a  blow  on  the  eye  which  has  produced 
paresis  of  the  sphincter  pupillae. 

The  pupil  will  be  smaller  than  normal  (a)  when  a  miotic  has  been 
used  in  one  eye ;  (b)  in  paralysis  of  the  centripetal  dilator  tract  affect- 
ing one  eye  more  than  the  other ;  (c)  in  some  cases  of  congestion  of  the 
iris  of  one  eye. 

Examination  for  the  Indirect  or  Consensual  Light  Reflex. — The 
patient  may  be  placed  facing  a  window,  one  eye  being  screened  in  such  a 
manner  that  it  will  be  in  shadow,  but  so  that  the  observer  may  see  the 
iris  sufficiently  distinctly  to  note  its  movements.  The  exposed  eye  is  then 
alternately  covered  and  uncovered,  the  movements  of  the  iris  of  the 
screened  eye  being  observed.  On  excluding  the  light  from  the  unscreened 
eye,  the  pupil  of  the  screened  eye  should  dilate.  On  re-admitting  the 
light  to  the  unscreened  eye  the  pupil  of  the  screened  eye  should  contract. 
The  indirect  or  consensual  reaction  should  equal  the  direct  reaction. 

Instead  of  using  daylight,  the  patient  may  be  placed  in  a  moderately- 
lighted  room  with  both  eyes  exposed  and  the  direct  reaction  be  excited 
by  reflecting  light  into  one  eye  by  means  of  a  mirror  from  a  source  of 
light  placed  behind  the  plane  of  the  patient's  face. 

The  consensual  light  reflex  may  be  diminished  in  both  eyes  (a)  when 
the  visual  acuteness  of  both  eyes  is  much  diminished  by  disease  affecting 
retina,  oj)tic  nerve,  chiasm,  or  tractus ;  (b)  when  the  centrifugal  dilator 
tracts  are  partly,  but  not  completely,  paralyzed;  (c)  when  there  is 
disease  of  the  parenchyma  of  the  irides;  (d)  when  mild  mydriatic  or 
miotic  remedies  are  instilled  into  the  eye  or  taken  internally  in  sufficient 
quantity;  (e)  when  the  tension  of  both  eyes  is  slightly  elevated. 

The  consensual  light  reflex  will  be  lost  in  both  eyes  (a)  when  there 
is  complete  blindness  due  to  disease  affecting  any  part  of  the  optic  tract 
distal  to  the  point  where  the  centrij)etal  fibres  of  the  light  reflex  arc  leave 
the  tract  (see  Light  Reflex  Arc)  ;  ^^  (b)  when  there  is  paralysis  of  the 
centrifugal  dilator  tract  on  both  sides  at  any  point;  (c)  when  the 
sphincter  pupillse  or  dilatator  pupillse  muscles  are  paralyzed  by  the 
use  of  mydriatics  or  miotics;  (d)  when  the  tension  is  above  normal  in 
both  eyes. 

The  consensual  light  reflex  may  be  diminished  in  one  eye  under 
the  same  conditions  as  in  diminished  light  reflex  of  both  eyes  when  one 
eye  or  the  tracts  on  one  side  are  affected. 


*'  In  complete  blindness  due  to  double  cortical  homonymous  hemianopsia  the 
pupillary  light  reflex  is  preserved. 


302  THE    EYE    AND    NERVOUS    SYSTEM 

The  consensual  light  reflex  will  be  diminished  in  the  normal  eye 
(a)  when  impairment  of  vision  is  due  to  a  lesion  of  the  opposite  tract 
distal  to  the  point  where  the  centripetal  light  reflex  fasciculus  leaves  the 
optic  tract.  In  the  affected  eye  (a)  when  there  is  a  mild  effect  from 
the  instillation  of  mydriatics  or  miotics;  (b)  when  partial  paralysis  of 
the  dilator  tract  exists  at  any  part  of  its  course ;  (c)  when  paresis  of  the 
sphincter  nucleus  or  sphincter  fasciculus  of  the  third  nerve  exists;  (d) 
when  the  tension  of  the  eye  is  greatly  increased. 

The  consensual  light  reflex  may  be  lost  in  one  eye  (a)  when  com- 
plete paralysis  of  the  sphincter  pupilla?  from  mydriatics  or  a  blow 
occurs;  (b)  when  the  sphincter  nucleus  or  sphincter  fasciculus  of  the 
third  nerve  is  paralyzed  and  when  great  increase  of  tension  affects 
one  eye. 

In  some  cases  examination  by  means  of  photography  is  desirable,  par- 
ticularly, as  Schaefer  ^'  has  pointed  out,  in  the  cases  of  anisocoria  with- 
out iridoplegia,  when  almost  maximum  dilation  of  both  irides  simul- 
taneously is  recorded. 

The  Light  Reflex,  Direct  and  Indirect  (Consensual),  Excited  by 
Illuminating  the  Various  Parts  of  the  Retina. — To  test  the  light  reflex 
of  various  parts  of  the  retina,  it  is  necessary  to  arrange  the  illumination 
so  that  with  the  patient  in  diffuse  light  of  sufficient  brightness  to  permit 
the  observer  to  discern  the  movements  of  the  pupils,  a  pencil  of  light  of 
sufficient  intensity  may  be  thrown  into  the  pupil,  so  directed  that  only 
the  part  of  the  retina  to  be  tested  will  be  illuminated.  It  is  difficult  to 
illuminate  a  particular  part  of  the  retina  without  more  or  less  diffusely 
illuminating  other  parts,  thus  making  the  results  of  the  test  unreliable. 
The  rays  of  light  should  be  so  directed  that  they  will  come  to  an  approxi- 
mate focus  on  the  retina,  the  refractive  error  of  the  jjatient  being  taken 
into  consideration.  The  size  of  the  incident  cylinder  of  rays  should  be 
a  little  smaller  than  the  pupil.  The  instrument  of  von  Fragstein  and 
Kempner  (Fig.  7)  may  be  employed  and  if  properly  made  is  very 
satisfactory. 

Heddaeus  advises  the  use  of  a  very  weak  source  of  light,  as  weak  as 
can  be  employed  and  enable  the  observer  to  see  the  reaction  (a  candle- 
or  lamp-flame),  reflecting  the  light  into  the  eye  by  means  of  the  plane 
mirror.  In  hemianopsia  the  light  should  be  thrown  on  the  retina  as 
peripherally  as  possible,  having  the  patient  rotate  the  eyes  to  favor  this, 
in  order  that  the  sensitive  part  of  the  retina  may  not  be  illuminated  and 
the  reflex  excited.     If  the  rays  of  light  as  they  enter  the  eye  are  parallel 


"Dissertation.  Gieasen,  1899. 


THE  INTRA-OCULAK  MUSCLES 


303 


or  nearly  so,  the  part  of  the  retina  most  intensely  illuminated  will  be 
very  small  and  the  best  results  will  be  obtained.  The  Haab  reflex 
(page  304)  may  be  employed  as  a  control  measure. 

The  direct  pupillary  reflex  will  fail  when  light  is  thrown  on  that 
part  of  the  retina  corresponding  to  an  absolute  scotoma  in  the  field  of 
vision,  occasioned  by  disease  of  the  retina  or  disease  of  the  visual  tract 
distal  to  the  cerebral  end  of  the  pupillary  light  reflex  arc ;  that  is,  in 
cases  of  disease  of  the  optic  nerve,  chiasm,  or  tractus.  The  pupillary 
light  reflex  may  be  diminished  in  cases  in  which  the  blindness  is  not 
quite  complete  (the  scotoma  not  being  absolute). 

In  cases  in  which  the  scotoma  is  absolute,  but  the  lesion  in  the  visual 
tract  is  situated  proximal  to  the  cerebral  end  of  the  pupillary  light  reflex 
arc,  other  conditions  being  normal,  the  illumination  of  the  corresponding 


Fig.  7.— Von  Fragstein  and  Kempner's  apparatus  for  testing  the  pupillary  reaction. 


blind  part  of  the  retina  will  elicit  the  direct  and  consensual  pupillary 
light  reflex  (Wernicke  pupillary  reaction). 

Method  of  Testing  the  Convergence  Reaction. — The  individual  is  first 
directed  to  look  at  some  object  at  a  distance,  when  the  size  of  the  pupil 
is  noted.  He  is  then  requested  to  look  at  a  point  at  a  distance  of  ten  or 
twelve  inches  from  the  eyes  and  in  the  median  plane  of  the  head,  the 
illumination  remaining  unchanged.  When  the  gaze  is  changed  from 
the  distant  to  the  near-point  a  contraction  of  the  pupils  will  occur.  If 
now  the  gaze  is  again  directed  to  the  distant  object,  a  dilatation  of  the 
pupils  will  take  place.  This  reaction  will  take  place  whatever  the  degree 
of  illumination.  If  a  card  of  test-type  is  held  before  the  eyes  and 
removed  and  approached  to  the  eyes,  an  excellent  convergence  pupillary 
reflex  may  be  obtained.  Individuals  who  cannot  converge  and  accommo- 
date (small  children,  the  blind,  and  the  insane)  do  not  show  the  con- 
vergence pupillary  reflex. 

Sensory  Reflex  (Pain  Reflex). — Dilatation  of  the  pupil  is  excited  under 
normal  conditions  by  irritating  sensory  parts  in  almost  any  part  of  the 
body.  The  reflex  path  is  by  way  of  the  sensory  nerves  to  the  ciliospinal 
centre  in  the  cord  and  along  the  dilator  tract  to  tlie  iris.  If  the  skin  of 
the  neck,  particularly  the  back  of  the  neck,  is  pinched,  pricked,  or  pulled, 


304  THE    EYE    AND    NERVOUS    SYSTEM 

the  palm  of  the  hand  or  sole  of  the  foot  tickled,  dilatation  of  the  pupil 
follows. 

The  diameter  of  the  pupil  changes  (dilates)  as  a  result  of  every  emo- 
tion— fright,  fear,  sudden  joy  or  sorrow,  amusement  Bumke  ^*  attrib- 
utes this  to  excitation  of  the  cells  of  the  cortex  cerebri. 

Irritation  of  the  skin  by  sudden  applications  of  cold  or  heat  will 
cause  the  pupil  to  dilate. 

It  has  been  observed  ^®  that  irritation  or  stimulation  of  the  pneumo- 
gastric,  hypoglossus  and  laryngeal  nerves  causes  dilatation  of  the  pupil. 

Oculo-pupillary  reflex  is  a  term  given  by  von  Varady  and  others  to 
the  pupillary  reflex  due  to  exciting  sensory  surfaces,  particularly  of  the 
skin  and  mucous  membrane  of  the  lids.  Sensory  mydriasis  is  probably 
always  followed  by  comparative  miosis.  If  the  excitation  is  continued, 
mydriasis  is  again  induced,  followed  by  miosis.  Von  Varady  found 
that  the  most  practical  method  was  to  prick  the  skin  of  the  eyelid  with  a 
needle  and  then  observe  the  pupillary  reflex,  which  is  that  of  dilatation 
followed  by  miosis.  As  studied  by  von  Varady,  the  reflex  differed  in 
different  individuals.  It  might  be  absent  in  apparently  healthy  indi- 
viduals, present  or  absent  in  functional  disturbances  or  sensibility, 
namely,  hysterical  anajsthesia  or  hyperoBsthesia,  but  it  was  never  present 
in  cases  of  organscsthesia,  affording  a  diagnostic  sign  in  this  condition. 

Haab's  Cortical  Pupillary  Reflex  (Attention  Reflex). — In  1885  Haab 
(Sitzungbericht  d.  Ges.  d.  Aerzte  in  Zurich,  Nov.  21)  described  a  reac- 
tion of  the  pupil  observed  under  the  following  conditions :  If,  when  the 
individual  is  in  a  dark  room,  his  line  of  vision  being  directed  forward 
into  space  or  against  a  dark  wall,  a  lamp  or  candle  is  placed  at  a  few 
feet  or  yards  distance  (1  metre)  at  an  angle  of  45°  to  the  patient's  line 
of  vision  and  on  the  horizontal  plane,  the  reflex  will  be  excited,  provided 
the  attention  (not  the  line  of  vision)  can"  be  directed  to  and  held  upon 
the  flame.  The  reaction  is  a  marked  contraction  of  the  pupil  which  is 
maintained  as  long  as  the  attention  remains  fixed  on  the  flame,  but  is 
relaxed  whenever  the  attention  is  relaxed.  The  reflex  is  marked  in 
individuals  whose  irides  are  freely  responsive  to  the  stimulus  of  light 
Some  practice  is  often  necessary  to  elicit  the  reflex.  When  the  power  to 
fix  the  attention  as  described  is  acquired,  other  luminous  sources  beside 
the  lamp  (small  window,  slit  in  a  blind,  etc.)  may  be  substituted  if 
desired.     Auto-examination  may  be  conducted.^'*     The  degree  of  con- 

=»  Centralbl.  f.  Nerv.  u.  Psych.,  October,  1903,  p.  40 

"  Kowalewsky,  Investigations  on  the  Innervation  in  Dilatation  of  the  Pupil, 
Kassan,  1885. 

"  Haab,  Archiv  f.  Augenheilk.,  Bd.  xlvi. 


THE  IXTKA-OCULAK  MUSCLES  305 

traction  of  the  pupil  is  in  direct  proportion  to  the  degree  of  the  illumina- 
tion. If  a  piece  of  white  paper,  illuminated  by  a  source  of  light  not 
visible  to  the  patient,  be  substituted  for  tlie  lamp,  the  contraction  of  the 
pupil  is  less.  If  a  piece  of  black  paper  or  other  black  object  be  sub- 
stituted, there  is  no  contraction. 

Variation  in  the  position  of  the  source  of  illumination  causes  varia- 
tion in  the  degree  of  the  contraction  of  the  pupil.  If  the  flame  is  carried 
backward  beyond  45°  on  the  horizontal  plane,  letting  the  point  of  fixa- 
tion on  the  line  of  vision  be  represented  by  zero,  the  contraction  becomes 
less;  if  in  advance  of  45°,  greater.  The  reaction  also  becomes  less 
marked  if  the  source  of  illumination  is  above  or  below  the  horizontal 
plane. 

Both  pupils  are  affected  equally  with  the  light  falling  in  one  eye. 

The  reflex  apparently  depends  on  the  normal  condition  of  the  visual 
fibres  throughout  corresponding  to  the  side  of  the  retina  on  which  the 
light  falls  and  on  the  normal  condition  of  the  centrifugal  fibres  from 
the  cortex  that  are  concerned  in  this  reflex.  According  to  Haab,  the  cen- 
tripetal pupillary  light  reflex  fibres,  central  from  the  chiasm  at  least,  are 
not  necessary  (may  be  destroyed)  for  the  production  of  this  reflex. 

Cortex  Reflex. — Stimulation  of  the  cortex  has  been  found  to  produce 
an  influence  on  the  pupil,  causing  moderate  dilatation.  Many  investi- 
gators have  noted  the  fact  that  stimulation  of  the  cortex  of  the  brain, 
particularly  of  the  occipital  lobe,  would  cause  dilatation  of  the  pupil. 
Stimulation  of  the  anterior  four-fifths  of  the  cortex  of  the  cerebellum 
has  caused  dilatation; 

Parsons,^  ^  experimenting  on  cats,  dogs,  and  monkeys,  found  that 
electrical  stimulation  of  the  cortex  of  the  brain  ''  in  the  neighborhood  of 
the  crucial  sulcus  and  over  a  considerable  area  of  the  occipital  region" 
produced  dilatation  of  the  pupil.  When  the  cervical  sympathetic  was 
intact  the  pupils  became  widely  dilated,  producing  '"  all  the  usual  effects 
of  excitation  of  the  nerves  themselves."  \\Tien  the  sympathetic  was 
destroyed  the  dilatation  was  not  abolished,  but  was  only  half  or  two- 
thirds  as  great  as  when  the  sympathetic  was  intact.  This  fact  was 
strongly  brought  out  when  only  one  sympathetic  was  destroyed,  the  dif- 
ference in  the  degree  of  dilatation  of  both  pupils  being  very  pronounced. 
With  division  of  the  sympathetic  and  of  the  third  nerve,  stimulation  of 
the  cortex  fails  to  affect  the  pupils ;  they  are  immobile  and  remain  one- 
half  to  two-thirds  dilated.  These  experiments  lead  Parsons  to  the  con- 
clusion that  cortical  stimulation  produces  dilatation  of  the  pupil  both  by 

"  Royal  London  Hospital  Reports.  June,  1904,  p.  20. 

20 


306  THE    EYE    AND    NERVOUS    SYSTEM 

stimulation  of  the  sympathetic  and  by  inhibition  of  the  sphincter  centre 
when  the  cervical  sympathetic  is  intact,  and  by  inhibition  of  the  sphinc- 
ter centre  when  the  cervical  sympathetic  is  destroyed. 

Braunstein  '^  obtained  similar  results,  but  attributed  them  wholly  to 
inhibition  of  the  third  nerve. 

Evidence  of  obtaining  contraction  of  the  pupil  by  stimulating  cer- 
tain parts  of  the  cortex  is  not  wanting.  Schaefer  induced  strong  con- 
traction of  the  pupil  by  stimulating  the  quadrate  lobule  in  monkeys,  and 
Terrier  by  stimulating  the  middle  of  the  convexity  of  the  hemispheres 
of  the  brains  of  pigeons. 

Changes  in  the  size  of  the  pupil  by  the  will  is  claimed  by  some  to  be 
possible.  I  have  myself  observed  a  patient  who,  while  standing  before 
a  window,  looking  forward  into  space,  caused  his  pupils  to  dilate  and 
contract.  However,  it  is  so  difficult  to  dissociate  accommodative  effort 
in  these  cases  that  it  is  not  possible  to  state  definitely  that  these  move- 
ments of  the  pupil  are  purely  voluntary. 

Orbicularis  or  Lid  Reaction  (Gifford-Galassi ;  Westphal-Piltz). — This 
was  first  described  by  Von  Graefe  ^^  as  an  accompaniment  of  a  general 
contraction  of  ocular  muscles  excited  by  contraction  of  the  orbicularis. 
The  reaction  has  since  been  described  by  many  writers.  By  forcibly 
closing  the  eye  it  often  occurs  that  the  pupil  contracts.  If  the  lids  are 
held  open  by  a  speculum  or  by  other  means  and  an  attempt  is  made  to 
close  the  lids,  the  pupil  in  many  individuals  will  contract. 

Harold  Gifford,  in  writing  of  this  reflex,  expresses  the  opinion  that 
it  is  the  result  of  an  overflow  stimulus  passing  from  the  nucleus  of  the 
facial  to  the  nucleus  of  the  third  nerve,  along  the  longitudinal  fasciculus. 
This  view  is  also  held  by  other  writers.  The  reflex  may  be  obtained  in 
normal  individuals,  but  often  fails.  As  a  rule,  in  cases  of  tabes  with 
failure  of  reaction  to  light  but  with  retention  of  convergence  and  ac- 
commodation reflex,  the  orbicularis  reflex  is  present  and  is  often  more 
energetic  than  the  convergence  reflex  (Westphal  and  others).  Uhthoff 
has  observed  cases  in  which,  with  preserved  convergence  reflex,  the 
orbicularis  reflex  has  failfed,  and,  conversely,  cases  with  loss  of  con- 
vergence reflex  in  which  the  orbicularis  reflex  was  preserved.  Piltz 
found  the  orbicularis  reflex  preserved  in  41  to  43  per  cent,  of  the  cases 
of  tabes.  Similar  observations  have  been  made  in  cases  of  progressive 
paralysis. 

Gifford  ^*  writes  that  the  reflex  is  most  marked  in  individuals  whose 


°  Zurm  Lehre  v.  d.  Inner,  d.  Pupillenbewegung,  Braunschweig,  1894. 
"Graefe's  Arehiv,  Bd.  i.,  H.  1,  p.  318. 
"Archives  of  Ophthal.,  vol.  xxiv.  p.  402. 


THE  INTRA-OCULAK  MUSCLES  307 

vision  is  poor  and  in  eyes  that  are  almost  blind.  While  he  does  not 
ascribe  much  of  diagnostic  value  to  the  reflex,  he  suggests  that  it  may  be 
of  use  to  determine  whether  the  sphincter  is  paralyzed  as  in  cases  of 
disease  of  the  nucleus  of  the  third  nerve  or  in  brain  tumor ;  also  possibly 
to  determine  whether  a  dilatation  of  the  pupil  is  due  to  a  mydriatic  in 
doubtful  cases.  Gifford  is  of  the  opinion  that  the  reaction  depends 
chiefly  on  the  palpebral  portion  of  the  orbicularis  palpebrarum  muscle 
and  that  if,  as  Mendel  believes,  the  nerve  supply  to  this  part  of  the 
orbicularis  comes  from  the  posterior  part  of  the  third  nucleus,  the 
influence  on  the  sphincter  pupillse  is  easily  explained. 

Pupillary  Reflex  on  Movement  of  the  Eyes. — The  size  of  the  pupil  is 
reduced  in  many  cases  by  forcible  abduction.  Uhthoff  ^^  has  observed 
this  reaction  in  some  cases  of  Argyll-Robertson  pupil  when  the  patient 
rotated  the  eye  outward  forcibly.  Contraction  of  the  pupil  by  forcible 
rotation  outward  has  been  observed  by  other  writers.  'No  pathological 
or  diagnostic  importance  has  been  attributed  to  it. 

PATHOLOGICAL    VARIATIONS    IN    THE    PUPIL. 

The  possible  pathological  variations  in  the  pupil  may  be  stated  as 
follows  (Uhthoff)  : 

1.  Loss  of  the  pupillary  light  reflex  with  retention  of  the  con- 
vergence and  accommodation  reflex.     (Argyll-Robertson  pupil.) 

2.  Loss  of  convergence  and  accommodation  reflex  and  retention  of 
light  reflex. 

3.  Loss  of  the  pupillary  reflex  for  light,  convergence,  and  accommo- 
dation. 

4.  Ophthalmoplegia  interna,  loss  of  all  reflex  movements  of  the 
pupil. 

5.  Loss  of  direct  light  reflex  in  an  amaurotic  eye  and  consensual 
light  reflex  in  the  fellow-eye,  due  to  loss  of  vision  from  a  lesion  periph- 
eral to  the  chiasm  ("  reflextaub,"  Ileddaeus). 

6.  Loss  of  sensory  or  psychical  reflex  action. 

7.  Abnormal  miosis  with  retention  of  light  and  convergence  reflexes, 
either  from  abnormal  stimulation  of  the  sphincter  pupillae,  which  may 
include  the  so-called  myotonic  pupillary  reaction,  or  from  paralysis  of 
the  dilatator  pupilla?. 

8.  Abnormal  dilatation  of  the  pupil  with  retention  of  the  light 
and  convergence  reflex,  stimulation  of  the  dilatator  pupillse  (spastic 
mydriasis). 


"Graefe  Saemisch.  2  Aufl.,  Bd.  xi.,  H.  ii..  p.  242. 


308  THE    EYE    AND    NERVOUS    SYSTEM 

9.  Anisocoria  (difference  in  size  of  pupils). 

10.  Changing  anisocoria. 

11.  Irregular  form  of  the  pupils. 

12.  Hippus. 

13.  Paradoxical  pupillary  reflex. 

14.  Hemianopic  pupillary  reaction. 

Argyll-Robertson  Pupil. — Loss  of  the  pupillary  light  reflex,  either  with 
miosis  (si)iual  miosis,  paralysis  of  the  sympathetic,  absence  of  dilator 
nerve  impulse)  or  without  miosis  (sympathetic  not  paralyzed,  skin  re- 
flex retained),  constitutes  Argyll-Robertson  pupil.  Both  irides  are 
similarly  affected  in  the  greater  number  of  cases,  but  in  some  cases  (13 
per  cent.,  Rochon-Divignand  and  J.  Hettz)  it  is  unilateral  for  a  long 
time.  In  quite  a  large  percentage  of  cases  the  pupillary  light  reflex  is 
lost  in  one  eye  and  only  partly  lost  in  the  other. 

Miosis  is  present  in  almost  all  tabetic  cases. 

Paralysis  of  accommodation  of  one  or  both  eyes  may  accompany  the 
Argyll-Robertson  pupil. 

The  convergence  pupillary  reflex  is  absent  in  a  small  percentage  of 
the  cases  of  loss  of  pupillary  light  reflex.  Moeli  puts  it  at  1  per  cent. ; 
Ulithoff  states  that  the  proportion  between  the  cases  with  loss  of  pupillary 
light  reflex  only  and  those  with  loss  of  convergence  light  reflex  also,  is 
3.5  to  1.  Argyll-Robertson  pupil  has  been  observed  most  frequently  in 
tabes,  where,  according  to  Gowers,  it  is  present  in  80  per  cent. ;  Stem, 
90  per  cent. ;  Uhthoff,  G7  per  cent,  of  the  cases.  The  pupils  are  of  the 
same  size  in  both  eyes  as  a  rule.  Of  the  cases  observed  by  Uhthoff  the 
pupils  were  unlike  in  size  in  25  per  cent. 

The  Arg^'ll-Robertson  pupil  never  occurs  in  healthy  individuals.  In 
166  cases  of  loss  of  pupillary  light  reflex,  Uhthoff  ^^  found  67.6  per 
cent,  associated  with  tabes,  8.8  per  cent,  with  dementia  paralytica,  8.17 
per  cent,  with  syphilis  without  tabes,  5.87  per  cent,  with  other  cerebral 
diseases,  1.4  per  cent,  with  multiple  sclerosis,  2.0  per  cent,  with  railway 
spine  and  injury  of  the  head,  0.7  per  cent,  with  congenital  feebleminded- 
ness, 0.7  per  cent,  with  hystero-epilepsy,  0.7  per  cent,  with  abuse  of 
tobacco,  0.7  per  cent,  with  hemiansesthesia  dextram,  3.6  per  cent,  with 
no  assignable  cause.  It  has  been  observed  in  hypertrophic  peripheral 
neuritis  (Dejerine),  in  polyencephalitis  haemorrhagica,  and  in  poly- 
neuritis alcoholica. 

Uhthoff''^   has  collected  the  statistics  of  Moeli,   Siemerling,   and 


»•  Berlin,  klin.  Woch.,  1886,  No.  .3. 

"  Graefe  Saemisch,  Handbucli,  2d  ed.,  chap.  xxii.  p.  223. 


THE  IXTRA-OCULAR  MUSCLES  309 

Thomsen,  embracing  4000  cases  of  diseases  of  the  nervous  system  (in- 
mates of  asylums).  Absence  of  pupillary  light  reflex  was  found  in  492, 
distributed  as  follows:  Progressive  paralysis,  85.5  per  cent. ;  tabes,  4.25 
per  cent. ;  syphilis,  1.83  per  cent. ;  multiple  sclerosis,  1.2  per  cent. ; 
injury  to  head,  0.6  per  cent. ;   paranoia  and  epilepsy,  2  per  cent. 

Loss  of  pupillary  light  reflex  is,  as  a  rule,  permanent,  since  it  occurs 
the  greater  number  of  times  as  an  accompaniment  of  diseases  of  the 
nervous  system  that  are  progressive.  It  is  sometimes  present  in  cases 
of  disease  of  the  nervous  system  which  tend  to  recover ;  under  these 
circumstances  the  pupillary  light  reflex  may  be  re-established.  Sulzer  ^^ 
observed  loss  of  pupillary  light  reflex  in  the  early  stage  of  syphilis  in 
11  of  53  cases  examined.  He  followed  the  pupillary  anomaly  in  some 
of  the  cases,  ascertaining  that  the  condition  may  develop  within  three 
months  after  the  primary  sore.  He  states  that  in  general  the  loss  of 
pupillary  light  reflex  in  such  cases  is  transitory. 

It  is  held  by  some  writers  that  the  ArgAdl-Robertson  pupil  is  inter- 
mittent in  some  cases.  Tanzi  ^^  reports  such  a  condition  in  a  case  of 
progressive  paralysis.  Uhthoff  is  of  the  opinion  that  it  does  not  occur 
in  tabes,  but  thinks  that  it  may  occur  in  less  confirmed  pathological 
conditions  of  the  nervous  system. 

Loss  of  pupillary  light  reflex  is  recognized  as  often  being  a  very 
early  symptom  of  tabes. 

Dejerine,^*'  Dufour,^^  and  LTithoff  are  of  the  opinion  that  all  cases 
of  Argyll-Eobertson  are  due  to  syphilis.  This  view  is  combated  by 
others.  Jaifray  has  observed  it  in  aortic  disease.  P.  Maria  regards  this 
form  of  pupil  as  an  evidence  of  disease  of  the  posterior  columns  of  the 
cord  which  may  or  may  not  be  syphilitic. 

Seat  of  the  Lesion. — It  has  been  stated  that  one-sided  loss  of  pupil- 
lary light  reflex  occurs  and  that  in  certain  cases  loss  of  convergence  reflex 
and  loss  of  the  power  of  accommodation  occur ;  also  that  miosis  is  most 
frequently  present  in  loss  of  pupillary  light  reflex.  Heddaeus  is  of  the 
opinion  that  the  lesion  in  eases  of  one-sided  loss  of  pupillary  light  reflex 
affects  the  centrifugal  fasciculus  of  nerve-fibres  which  proceed  from  the 
sphincter  nucleus,  the  fasciculus  for  the  accommodation  reflex  remaining 
uninjured.  If  the  fasciculus  from  the  accommodation  nucleus  is  in- 
volved, there  is  total  one-sided  iridoplegia.  The  consensual  reaction  of 
the  fellow-eye  is  not  affected.     Others  regard  the  one-sided  loss  of  pupil- 

'*  Ann.  de  Dermatol,  et  de  Syph.,  March,  1901. 

^'  Riv.  di  patol.  nerv.  e.  ment.,  iv.  S.  386. 

"  Soc.  de  Xeurologie  de  Paris,  June  15,  1902. 

"Bull,  de  la  Soc.  med.  des  H6pitaux  de  Paris,  January  1.3.  1902. 


310  THE    EYE    AND    NERVOUS    SYSTEM 

lary  light  reflex  as  a  nuclear  affection.  Uhthoff  is  of  the  opinion  that 
lesion  of  the  portion  of  the  centripetal  pupillary  light  reflex  fibres  lying 
between  the  tractus  or  external  geniculate  body  and  the  sphincter  nucleus 
has  more  in  its  favor.  If  tliere  is  a  lesion  of  this  bundle  on  both  sides 
at  this  point,  the  light  reflex  will  be  abolished.  Miosis  might  be  pro- 
duced by  irritation  of  the  sphincter  nucleus,  an  extension  of  the  process 
to  this  centre,  but  Uhthoff  believes  that  the  miosis  is  due  to  a  lesion  of 
the  cervical  sympathetic. 

Pathological  research  in  cases  of  loss  of  pupillary  reaction  has 
afforded  but  little  light  on  the  subject.  Pineles,  Zeri,  and  others,  in 
examining  microscopically  the  brains  of  tabetics,  found  pathological 
changes  in  the  floor  of  the  aqueduct  of  Sylvius.  Kound-cell  infiltration 
was  found  in  this  locality  by  Kostenitsch.  In  the  examination  of  20 
cases  of  progressive  paralysis  with  Argyll-Robertson  pupil,  Schutz  *^ 
found  pathological  changes  in  the  nerve-fibres  in  the  gray  substance  in 
the  floor  of  the  aqueduct  (sclerosis)  to  which  he  attributed  the  loss  of 
the  pupillary  light  reflex ;  however,  he  was  not  able  to  demonstrate  any 
particular  bundle  of  nerve-fibres  as  being  concerned  in  this  degeneration. 
Siemerling  and  Boedeker  have  met  with  similar  changes,  also  with 
minute  hemorrhages  in  tliis  tissue.  The  cases  of  Moeli  (page  291) 
and  Monakow,  who  found  in  a  case  of  one-sided  loss  of  pupillary  light 
reflex  a  small  sclerosed  focus  in  the  tissue  between  the  sphincter  nucleus 
and  the  external  geniculate  body,  apparently  involving  the  centripetal 
pupillary  light  reflex  bundle  as  it  passes  from  the  tractus  to  the  sphincter 
nucleus,  support  Uhthoff's  position. 

A  number  of  investigators  refer  the  lesion  to  the  cervical  portion  of 
the  spinal  cord.  Wolf  ^^  found  that  in  cases  of  tabes  and  also  general 
paralysis  in  which  the  light  reflex  was  lost  and  the  knee-jerk  intact,  the 
lesion  affected  the  posterior  colunms  of  the  cervical  cord.  In  cases  in 
which  the  pupillary  light  reflex  was  retained  and  the  knee-jerk  was 
lost,  the  cord  was  affected  below  the  third  or  fourth  dorsal  vertebra.  In 
cases  in  which  both  pupillary  light  reflex  and  knee-jerk  were  lost,  the 
entire  cord  was  affected.  Others  have  made  similar  observations.  These 
investigators  have  failed  to  examine  the  brain  tissue  in  the  floor  of  the 
third  ventricle  to  determine  whether  it  was  or  was  not  involved.  Cases 
of  loss  of  pupillary  light  reflex  with  intact  cord  have  been  reported 
(Dejerine)  and  cases  of  cervical  tabes  with  retained  pupillary  light 
reflex  have  also  been  reported  (Buck). 


'  Archiv  f.  Psych.,  Bd.  xxii.,  H.  3,  S.  527. 
Ibid.,  xxxii.,  H.  i.,  S.  57. 


THE  IN^TRA-OCITLAR  MUSCLES  311 

Recently  Marina  *^  has  examined  the  ciliary  ganglion  and  ciliary 
nerves  in  cases  of  loss  of  pupillary  light  reflex  and  claims  to  have  found 
pathological  changes  in  all  of  the  cases.  He  is  of  the  opinion  that  the 
seat  of  the  lesion  is  in  these  structures. 

Ruge  advances  the  hypothesis  that  disease  of  the  ciliospinal  centre 
is  followed  by  atrophy  of  sympathetic  nervous  elements  in  the  ciliary 
ganglion,  causing  an  interruption  in  the  reflex  arc.  The  reflex  irido- 
plegia  of  tabes  is  brought  about  in  this  way,  making  a  double  lesion 
unnecessary  to  explain  the  phenomenon. 

Clinical  and  pathological  evidence  regarding  the  site  of  the  lesion 
in  loss  of  pupillary  light  reflex  to  the  present  time  fails  of  positive 
localization.  The  weight  of  evidence  indicates  a  lesion  in  the  vicinity 
of  the  nucleus  of  the  third  and  the  occasional  accompaniment  of  paralysis 
of  extrinsic  eye-muscles  as  well  as  the  ciliary  muscles  makes  the  supposi- 
tion plausible. 

Loss  of  Convergence  Accommodation  Reflex  and  Retention  of  Light  Reflex. 
— This  condition  is  rarely  met  with.  It  occurs  in  most  cases  of  con- 
genital absence  of  convergence,  in  some  cases  in  which  the  power  of 
convergence  is  lost ;  also  in  some  cases  in  which  the  convergence  is 
abandoned,  as  in  loss  of  the  vision  of  one  eye  or  in  conditions  in  which 
one  eye  is  not  used.  It  has  been  observed  in  tabes,  but  must  be  ex- 
tremely rare,  as  Uhthoff  writes  that  he  has  never  seen  it.  The  author 
has  observed  it  in  syphilitic  basal  meningitis,  and  it  has  been  seen  in 
progressive  paralysis,  myelitis,  and  tumor  of  the  corpora  quadrigemina. 

The  site  of  the  lesion  is  supposed  to  be  in  the  brain  tissue  imme- 
diately below  the  sphincter  and  accommodation  nucleus,  so  placed  as  to 
involve  the  fasciculus  of  fibres  which,  according  to  Heddaeus,  joins  the 
convergence  centre  with  the  oculomotor  nerve. 

Loss  of  convergence  pupillary  reaction  may  affect  one  pupil  alone  or 
both  pupils.  The  latter  is  by  far  the  more  common.  The  convergence 
reaction  is  seldom  absent  in  tabes,  but  it  occurs  in  some  cases.  Sulzer  *^ 
reports  two  cases  of  absence  of  convergence  and  light  reflex  in  53  cases 
of  early  syphilis.  Moeli  ^®  reports  it  in  one  per  cent,  of  500  cases  ex- 
amined. Uhthoff  ^"  found  absence  of  the  convergence  pupillary  reflex 
in  syphilis,  8  cases;  tabes,  3  cases;  progressive  paralysis,  2  cases; 
injury,  2  cases ;  "  cold,"  1  case ;  tuberculosis  of  the  brain,  1  case ; 
tumor  of  the  brain,  1  case ;  no  assignable  cause,  12  cases.    Convergence 

"Ann.  d.  Neurologie,  vol.  xix.  p.  209. 
**Ann.  de  Dermatol,  et  de  Syph.,  March,  1901. 
"  Centralbl.  f.  prakt.  Augenheilk.,  1885,  p.  272. 
^■Berlin,  klin.  Woch.,  1886,  No.  3. 


312  THE    EYE    AND    NERVOUS    SYSTEM 

pupillary  paralysis  has  been  observed  also  after  diphtheria,  alcohol 
intoxication,  tubercnlosis,  Basedow's  disease. 

In  some  of  the  cases  in  which  convergence  and  accommodation  pupil- 
lary reaction  is  lost,  mydriasis  is  present,  even  when  the  power  of 
accommodation  is  preserved. 

Ophthalmoplegia  interna  is  a  term  given  to  the  condition  of  isolated 
paralysis  of  the  sphincter  pupilUr  and  the  ciliary  muscles  without  in- 
volvement of  the  extrinsic  eye-muscles.  The  lesion  in  these  cases  is 
without  doubt  nuclear.  It  is  most  frequently  unilateral,  but  may  be 
bilateral.  Not  infrequently  the  sphincter  will  be  involved  first,  the 
ciliary  muscle  later ;  the  reverse  may  occur.  In  rare  cases  there  may  be 
isolated  paralysis  of  one  or  the  other  muscle. 

In  regard  to  the  cause,  Uhthoff  has  collected  30  cases.  Syphilis  was 
the  cause  in  23.3  per  cent. ;  tabes,  10  per  cent. ;  progressive  paralysis, 
G.Q  per  cent. ;  traumatism,  6,6  per  cent. ;  cold,  3.3  per  cent. ;  intra- 
cranial disease,  6.6  per  cent. ;  cause  unknown,  40  per  cent.  The  pro- 
portion of  unilateral  to  bilateral  was  approximately  2  to  1.  Syphilis  is 
by  far  the  most  frequent  cause.  In  six  cases  observed  by  the  writer  a 
history  of  syphilis  was  obtained  in  all,  and  in  all  the  primary  syphilitic 
lesion  had  occurred  many  years  before.  Post-diphtheritic  ophthalmo- 
plegia interna  has  been  observed. 

In  quite  a  high  percentage  of  cases  other  portions  of  the  nucleus  of 
the  third  nerve  and  the  nucleus  of  the  fourth  and  sixth  nerves  become 
involved.  The  condition  of  partial  or  complete  ophthalmoplegia  externa 
develops.  At  times  paralysis  of  the  intrinsic  muscles  follows  paralysis 
of  the  extrinsic  muscles. 

Prognosis. — Unilateral  ophthalmoplegia  interna  may  remain  for 
many  years  unchanged,  but  the  greater  number  of  cases  recover  partly 
or  wholly  or  are  complicated  by  involvement  of  other  parts  of  the  central 
nervous  system.  Double  ophthalmoplegia  interna  is  not  as  a  rule  so  apt 
to  remain  unchanged. 

Loss  of  sensory  or  psychical  reflex  action  will  occur  in  any  case  in 
which  there  is  a  lesion  affecting  the  dilator  pupillary  tract.  Destruction 
of  the  ciliospinal  centre,  M'ith  consequent  miosis ;  of  the  superior  gang- 
lion of  the  cervical  sympathetic,  which  produces  the  symptom  complex 
of  ptosis,  miosis,  enophthalmus,  and  reduction  in  the  tension  of  the 
globe;  of  the  centrifugal  dilator  tract  distal  to  the  ganglion  with  the 
consequent  miosis,  all  produce  loss  of  sensory  and  psychical  reflex,  with- 
out abolishing  the  light  reflexes. 

Tx)ss  of  the  sensory  reflex  occurs  in  all  cases  of  tabes  with  miosis  and 
Argyll-Robertson  pupil.    It  is  in  fact  absent  in  all  individuals  who  from 


THE  IXTRA-OCULAK  MUSCLES  313 

any  cause  present  miosis  with  loss  of  pupillary  light  reflex.  It  may  also 
be  absent  in  cases  of  miosis  with  retention  of  the  light  reflex.  When  this 
occurs,  lesion  of  the  dilator  tract  in  some  part  is  the  cause.  Aside  from 
tabes,  this  condition  occurs  in  progressive  paralysis,  some  cases  of 
syphilis,  multiple  sclerosis,  and  traumatism. 

This  reflex  is  retained  in  all  normal  individuals  and  in  those  with- 
out miosis  in  whom  the  pupillary  light  reflex  is  retained. 

In  cases  of  miosis  with  loss  of  sensory  reflex,  mydriatics  effect  a 
partial  dilatation  of  the  pupil,  probably  by  direct  effect  on  the  dilator 
muscle-fibres. 

Abnormal  miosis  with  retention  of  light  and  convergence  reflex  may 
occur  from  abnormal  stimulation  of  the  sphincter  pupillse  or  from 
paralysis  of  the  dilatator  pupilla?  (paralysis  of  the  sympathetic). 

Stimulation  miosis  (spastic  miosis)  is  frequently  accompanied  by 
spasm  of  accommodation.  This  may  be  brought  about  by  hemorrhagic 
encephalitis,  brain  tumor,  or  abscess  which  does  not  involve  the  sphincter 
nucleus,  but  simply  stimulates  or  irritates  the  neurons  of  this  group  of 
nerve  cells.  If  the  brain  disease  advances  and  destroys  the  sphincter 
nucleus,  dilatation  supersedes  miosis.  Spastic  miosis  may  be  due  to  the 
influence  of  light  in  individuals  with  hypersensitive  retinae,  as  in  neu- 
rasthenia and  in  hyj^eremia  of  the  retina  and  optic  nerve,  and,  accord- 
ing to  Heddaeus,  in  the  first  stages  of  alcoholic  amblyopia,  abnormal  use 
of  the  eyes  and  as  a  result  of  excessive  sexual  indulgence.  Excessive 
synergistic  contraction  of  the  pupils,  occurring  in  hypermetropes  who 
are  obliged  to  converge  excessively  in  order  to  accommodate  sufficiently, 
especially  in  the  early  stage  of  presbyopia,  may  produce  spastic  miosis. 
This  may  also  occur  in  emmetropic  eyes  in  cases  of  weakness  of  the 
interni. 

Except  that  produced  by  miotics,  spastic  miosis  results  in  narrowing 
the  pupil  to  a  greater  degree  than  anything  else.  The  jjupil  contracts 
very  slightly  to  light  and  accommodation  and  is  influenced  to  some  de- 
gree by  miotics.  It  differs  from  paralytic  miosis  in  that  mydriatics 
cause  dilatation  ad  maximum. 

Cases  of  alternating  spastic  miosis  and  mydriasis  have  been  reported 
by  Bielschowsky,^^  in  one  of  which  alternating  miosis  and  mydriasis 
occurred  in  the  right  eye  at  intervals  of  twenty  to  thirty  seconds.  There 
was  right  partial  oculomotor  paralysis.  When  mydriasis  was  present, 
there  was  slight  divergence.  When  miosis  came  on,  the  eye  turned 
inward  and  downward. 


*«Klin.  Monatsblatt  f.  Augenheilk.,  Bd.  xvi.  p.  308. 


314  THE    EYE    AND    NERVOUS    SYSTEM 

The  myotonic  pupillary  reaction  of  Saenger  *^  is  closely  allied  to  spas- 
tic miosis.  This  reaction  is  in  the  nature  of  a  tonic  spasm  and  may  be 
excited  by  light,  convergence  and  accommodation,  and  by  the  orbicu- 
laris reflexes.  It  is  characterized  by  a  very  slow  recovery  from  the  mio- 
sis occasioned  by  the  stimuli  mentioned.  When  the  stimulus  is  removed, 
the  dilatation  of  the  pupil,  which  normally  follows  the  reflex  miosis  very 
rapidly,  takes  place  slowly ;  that  is,  in  from  one-half  to  ten  minutes. 

The  phenomenon  has  been  observed  only  in  patients  with  disease  of 
the  nervous  system.  Saenger  observed  it  in  a  case  of  suspected  tabes. 
The  pupillary  light  reflex  was  lost.  The  patient  was  conscious  of  the 
excessive  contraction  of  the  pupil,  he  experienced  a  strained  or  cramped 
feeling  in  the  temple  and  forehead.  Distance  objects  were  blurred  at 
this  time,  indicating  spasm  of  accommodation.  Piltz  has  observed  the 
myotonic  pupillary  reaction  in  general  paresis  with  loss  of  pupillary 
light  reflex. 

Saenger  is  of  the  opinion  that  the  site  of  the  lesion  is  in  the  iris  and 
that  the  condition  is  analogous  to  Thomsen's  disease. 

Abnormal  dilatation  of  the  pupil  with  retention  of  the  light  and  conver- 
gence reflexes  (spastic  mydriasis)  is  met  with  in  cases  of  stimulation  of 
the  cervical  sympathetic,  whether  due  to  inflammatory  processes  affect- 
ing the  ciliospinal  centre  or  superior  cervical  ganglion  or  by  sensory  or 
psychical  influences.  It  may  accompany  intracranial  effusions  and 
intracranial  tumor.^"  Abnormal  dilatation  has  been  observed  after  epi- 
leptic seizures,  premonitory  to  locomotor  ataxia,  in  acute  mania,  and  in 
melancholia.  Bumke*"^^  observed  excessive  dilatation  of  the  pupil  in 
fifteen  cases  of  catalepsy,  with  retention  of  light  and  convergence  re- 
flexes, in  marked  anaemia,  and  sometimes  in  imbeciles.  It  is  often 
present  in  cases  of  intestinal  irritation,  as  from  helminths.  The  pupils 
are  dilated  and  fixed  in  amaurosis,  in  complete  paralysis  of  both  third 
nerves,  and  when  mydriatics  have  been  used. 

Anisocoria. — Difference  in  the  size  of  the  pupils  is  frequently  ob- 
served. Slight  differences  may  exist  under  perfectly  normal  conditions 
(page  288).  Pronounced  differences  in  the  size  of  the  pupils  is 
jiathological  almost  without  exception.  Difference  in  the  size  of  the 
pupils,  the  abnormal  pupil  being  smaller  than  its  fellow,  will  occur  in 

*Neurolog.  Contralbl.,  vol.  xxi.  p.  837. 

*°  When  in  the  course  of  intracranial  disease  of  any  kind,  miosis  gives  way  to 
mydriasis,  the  symptom  is  one  of  grave  import,  as  it  signifies  partial  or  complete 
paralysis  of  the  sphincter  nucleus  either  by  direct  involvement  or  by  excessive 
pressure ;  paralyses  of  other  branches  of  the  third  nerve  often  accompany  or  follow 
this  condition. 

"Centralbl.  f.  Neur.  u.  Psych..  October,  1903,  p.  41. 


THE  TNTEA-OCULAR  MUSCLES  315 

paralysis  of  the  sympathetic  affecting  one  side,  when  a  miotic  has  been 
used,  when  there  is  congestion  of  the  iris. 

The  abnormal  pupil  will  be  larger  than  its  fellow  when  there  is 
stimulation  of  the  sympathetic  on  one  side,  when  a  mydriatic  has  been 
used  in  one  eye  only,  when  there  is  paralysis  of  the  centripetal  fibres  of 
the  pupillary  light  reflex  arc,  after  a  blow  that  has  produced  paresis  of 
the  sphincter  pupilla^,  in  increase  of  intra-ocular  tension  affecting  one 
eye  more  than  the  other,  in  paralysis  of  the  motor  oculi,  in  disease  of 
the  nucleus  of  the  third  nerve  affecting  the  sphincter  portion. 

Inequality  of  the  pupils  "  may  lead  one  to  suspect  locomotor  ataxia, 
general  paralysis  of  the  insane,  unilateral  lesion  of  the  third  or  of  the 
sympathetic  nerve,  pain  in  a  branch  of  the  fifth  nerve,  carotid  aneurism 
or  tumor  of  the  neck,  unilateral  cranial  lesion,  unilateral  acute  glau- 
coma." 

A  difference  in  the  size  of  the  pupils  is  common  in  tabes.  Of  166 
cases  examined  by  Uhthoff,  25  per  cent,  presented  a  difference  in  the 
size  of  the  pupils.  In  25  per  cent,  of  the  cases  of  syringomyelia  the 
pupils  are  uneven,  due  to  involvement  of  the  ciliospinal  centre.  Schau- 
man  ^^  has  noted  a  difference  in  the  pupils  of  723  subjects.  The  num- 
ber includes  401  subjects,  or  55.5  per  cent,  with  no  organic  lesions 
but  some  neurosis,  including  11  with  exophthalmic  goitre,  4  epileptics, 
and  3  with  chorea.  In  the  remaining  322,  or  44.5  per  cent.,  there 
were  organic  lesions,  including  tuberculosis  in  6.8  per  cent.,  and  some 
vascular  affection  in  5.5  per  cent.  When  anisocoria  is  not  due  to  an 
organic  lesion,  it  is  the  expression  of  an  asthenic  diathesis  or  a  sign 
of  hereditary  degeneracy. 

Irregularity  of  the  Pupils. — Piltz  ^^  observed  in  a  new-born  child  that 
the  pupil  was  not  circular,  but  that  it  soon  became  so.  He  concludes 
that  synergetic  action  of  the  various  parts  of  the  iris  does  not  obtain 
at  birth,  but  soon  asserts  itself. 

When  irregularity  of  the  pupil  is  observed,  it  is  advisable  to  use  a 
mydriatic  for  the  purpose  of  excluding  synechiae.  Irregularity  of  the 
pupil  consists  in  irregularities  in  the  margin  of  the  pupil  due  to  de- 
fective innervation  of  the  various  parts  of  the  muscles  of  the  iris. 
Thus  lesion  of  the  outer  branch  of  the  long  ciliary  nerves  causes  con- 
tr notion  of  the  upper  outer  half  or  quadrant  of  the  iris  and  a  flattening 
of  the  pupil  in  that  part.  The  inner  branch  of  these  nerves  supplies 
the  lower  inner  half  of  the  iris.  The  various  nerve-fibres  from  the 
sphincter  nucleus  are  distributed  to  the  various  parts  of  the  sphincter 


"Abstract,  Journal  Am.  Med.  Asso.,  July  11,  1903,  p.  143. 
"  Neurol.  Centralbl..  1903,  vol.  xxii.  p.  662. 


316  THE    EYE    AND    NERVOUS    SYSTEM 

pupilla?  and  lesion  of  a  part  of  these  fibres  would  cause  paralysis  of 
portions  of  the  sphincter  pupillac  only. 

Piltz  divides  irregularities  of  the  pupil  not  due  to  synechia}  into 
two  classes — (1)  changing;    (2)  constant. 

Changing  irregularities  present  the  following  conditions.  When 
the  pupillary  light  reflex  is  tested,  some  parts  of  the  iris  contract  while 
other  parts  do  not.  In  a  few  days  the  part  that  was  stationary  is 
active,  the  other  parts  becoming  stationary.  Piltz  has  observed  this 
form  in  progressive  paralysis  and  in  katatonia.  lie  observed  a  case 
in  which  on  the  21st  of  January,  1899,  the  right  pupil  was  eccentrically 
displaced  doA\Tiward  and  outward ;  on  the  24th  of  January  the  same 
pupil  was  displaced  upward  and  outward.  The  pupillary  light  reflex 
was  lost  in  both  eyes  and  convergence  reflex  reduced.  The  orbicularis 
reflex  was  preserved  to  some  degree.  Piltz  is  of  the  opinion  that 
changing  paralysis  is  due  to  a  shifting  of  the  lesion  in  the  nucleus. 

Fixed  irregularities  are  due  to  lesions  in  the  nerves  that  innervate 
the  iris  that  are  more  or  less  constant.  Piltz  observed  seven  cases  of 
progressive  paralysis,  with  irregular  pupils  in  six.  The  inmates  of  a 
Swiss  asylum  for  the  insane  showed  irregular  pupils  in  42  per  cent. 
Of  62  cases  of  tabes,  29  per  cent,  presented  irregular  pupils.  Intra- 
ocular conditions  not  infrequently  cause  irregularity  of  the  pupil,  and 
intraorbital  conditions  may  cause  injury  of  a  long  or  short  ciliary 
nerve  or  destruction  of  the  ciliary  ganglion,  but  these  cases  are  very 
rare.  Joffroy^^  states  that  within  the  last  ten  months  preceding  the 
writing  of  his  article  he  had  not  seen  a  case  of  Argyll-Robertson  pupil 
that  did  not  show  some  irregularity  of  the  margin  of  the  pupil.  He 
regards  the  condition  as  often  the  first  sign  of  advancing  disease  of  the 
innervation  of  the  iris  which  eventually  goes  on  to  complete  loss  of  the 
pupillary  light  reflex  (Argj'll-Robertson  pupil)  and  in  some  cases  to 
ophthalmoplegia  interna.  Piltz  has  observed  that  irregularities  in  the 
pupillary  margin  are  of  frequent  occurrence  in  progressive  paralysis; 
that  in  incipient  progressive  paralysis  it  is  at  times  the  first  and  only 
symptom ;  and  it  is  sometimes  the  very  first  sign  of  the  development  of 
Argj'll-Robertson  pupil.  The  changes  obsen-ed  indicate  that  they  are 
due  to  disease  of  the  nerves  supplying  the  iris  or  to  their  nuclei. 

Hippus. — Under  certain  conditions,  especially  when  both  eyes  are 
shaded  from  the  light  and  then  quickly  uncovered,  the  patient  being 
placed  before  a  window  or  other  source  of  light,  the  pupils  will  alter- 
nately dilate  and  contract.     This  change  in  size  of  the  pupil  under 

"Rev.  neurologique.  1902,  p.  275. 


THE   IXTRA-OCULAR  MUSCLES  317 

uniform  illumination  and  without  the  influence  of  accommodation  or 
convergence  is  known  as  hippus.  It  is  a  clonic  spasm  of  the  sphincter 
puj)illa3  (Fick).  It  may  not  be  associated  with  impaired  vision,  but 
is  occasionally  observed  in  developing  cataract.  It  is  sometimes  asso- 
ciated with  nystagmus.  It  is  also  seen  in  neurasthenia,  hysteria,  epi- 
lepsy, disseminated  sclerosis,  cerebrosj)inal  sclerosis,  in  acute  menin- 
gitis, and  in  some  cases  of  brain  tumor.  In  observing  the  changes  in 
the  size  of  the  pupil,  it  must  be  remembered  that  the  normal  oscillation 
differs  much  in  different  individuals.  In  patients  with  irides  that  react 
very  readily  to  the  stimulus  of  light,  oscillation  is  more  pronounced 
than  in  irides  that  react  slowly.  It  is  difficult  to  determine  where 
normal  oscillation  leaves  off  and  abnormal  oscillation  begins. 

According  to  Schrameck,^^  hippus  occurs  in  cases  in  which  there 
is  paresis  of  the  extrinsic  ocular  muscles,  particularly  when  improve- 
ment is  taking  place.  It  has  been  observed  in  a  case  of  absence  of 
pupillary  light  reflex  (Uhthoff),  also  in  a  case  of  double  amaurosis 
(Ewitzky). 

Paradoxical  Reflex. — Piltz  ^^  classifies  the  paradoxical  reflexes  as 
follows:  (1)  Paradoxical  reflex  (a)  on  near  vision,  convergence  dila- 
tion of  the  pupil;  (h)  contraction  of  the  pupil  in  distance  vision. 
(2)  True  jjaradoxical  pupillary  reflex,  (a)  dilatation  by  bright  light; 
(b)  contraction  of  the  pupil  when  the  light  is  withdrawn,  with  sub- 
divisions (aa)  rapid  dilation  without  preceding  contraction  or  (hh) 
pupil  contracts  without  preceding  dilation. 

Experiments  on  rabbits  and  other  animals  have  shown  that  section 
of  the  cervical  sympathetic  on  one  side  causes  constriction  of  the  pupil, 
and  removal  of  the  superior  cervical  ganglion  on  the  opposite  side 
causes  slightly  greater  constriction  of  the  pupil  on  the  same  side,  but 
under  certain  conditions,  excitements,  anaesthesia,  dyspnoea,  the  pupil 
on  the  side  of  the  excised  superior  cervical  ganglion  becomes  more  dilated 
than  on  the  side  of  the  divided  nerve.  This  phenomenon  has  been 
termed  paradoxical  pupil-dilatation.  It  has  been  observed  that  the 
pupil  on  the  side  of  the  excised  superior  cervical  sympathetic  may 
become  larger  than  its  fellow  even  when  the  cervical  sympathetic  on 
the  other  side  has  not  been  divided. 

Lodato  ^^  writes  of  the  paradoxical  pupillary  reaction  following 
sympathectomy  and  states  that  when  chloroform  is  given,  the  pupil  on 


■^Klin.  Moiiatsblt.  f.  Auorenheilk.,  May,  1902,  pp.  45,  46. 
»« Neurol.  Centralbl.,  20,  22,  1902. 
"Arch.  di.  ottal.,  vol.  x.,  3,  4,  1902. 


318  THE    EYE    AND    NERVOUS    SYSTEM 

the  operated  side  dilates  more  considerably  than  on  the  non-operated 
side. 

The  dilatation  must  be  the  result  of  an  increase  in  the  tone  of  the 
dilatator  pupilla}  muscle  due  to  the  excitation,  independent  of  the  influ- 
ence of  the  dilator  nerve-fibres  in  their  relation  to  the  superior  cervical 
sympathetic  ganglion. 

Paradoxical  pupillary  reflex  is  rare.  It  has  been  observed  only 
in  patients  affected  with  grave  lesions  of  the  central  nervous  system. 
Bock  ^^  mentions  two  cases ;  both  were  tabetic.  In  one  patient  there 
were  grave  lesions  of  the  sympathetic  with  increased  redness  and  sweat- 
ing of  one  side  of  the  face.  The  paradoxical  reaction  was  more  marked 
on  the  side  of  greater  redness  and  sweating  of  the  face.  The  vision  in 
this  eye  was  greatly  reduced.  The  reaction  was  obtained  from  the  fel- 
low-eye which  possessed  %  vision  but  to  less  degree.  The  reaction  in 
this  case  was  obtained  by  subjecting  the  eyes  to  the  influence  of  bright 
light,  dilatation  instead  of  contraction  following. 

Accommodative  paradoxical  pupillary  reaction  sometimes  occurs 
in  patients  with  grave  disease  of  the  central  nervous  system.  Piltz 
mentions  the  rarity  of  true  paradoxical  pupillary  reaction  and  cites 
but  five  cases,  all  occurring  in  patients  with  advanced  disease  of  the 
nervous  system. 

Paradoxical  pupillary  reaction  may  be  confounded  with  hippus, 
the  changes  in  the  pupil  which  occur  in  convergence  and  divergence, 
the  effect  of  heat,  and  the  relaxation  of  the  pupil  after  forcible  con- 
traction of  the  orbicularis  and  the  production  of  the  so-called  lid  reflex. 

More  than  half  of  the  reported  cases  of  paradoxical  pupillary  re- 
action have  been  reported  as  occurring  in  tabes,^^  but  Uhthoff  writes  ^^ 
that  in  all  of  the  vast  number  of  patients  observed  by  him  he  has  never 
seen  a  true  case  of  paradoxical  pupillary  reaction.  Cases  have  been 
observed  occurring  in  progressive  paralysis,  multiple  sclerosis,  brain 
syphilis. 

The  Hemianopic  Pupillary  Reflex.si — Lesions  of  the  brain  situated 
centrally  from  the  cerebral  end  of  the  pupillary  light  reflex  arc,  inter- 
fering with  vision,  do  not  interfere  with  the  pupillary  light  reflex.  A 
lesion  situated  in  the  internal  capsule  affecting  the  visual  fibres  on  their 
way  to  the  cortical  visual  area,  or  a  lesion  of  the  visual  area,  will  pro- 
duce blindness  on  one  or  both  sides  without  abolishing  the  pupillary 

"  Graefe's  Archiv,  Bd.  xvii.,  H.  2,  S.  244. 

"Muchin  Money,  Lancet,  1889,  p.  173;    Abadie  and  others. 

~Graefe  Saemisch,  2d,  Bd.  xi.,  T.  ii.  p.  240, 

"  Wernicke,  Fortschrift  d.  Med.,  January  15,  1883,  Bd.  i..  No.  2. 


THE   INTRAOCULAR  MUSCLES  319 

light  reflex.  This  is  therefore  a  valuable  symptom  in  determining  the 
location  of  a  lesion  affecting  the  vision.  It  has  been  suggested  that  the 
Haab  reflex  (page  304)  may  interfere  with  the  observation  of  this 
reflex.  In  regard  to  this,  Haab  writes :  "  In  hemianopsia  due  to  cor- 
tical lesions  my  reflex  is  not  a  disturbing  factor,  as  the  lesion  destroys 
the  power  of  fixing  the  attention." 

The  relation  of  lesions  of  the  cortical  visual  areas  and  the  optic 
radiations  to  the  pupillary  light  reflex  may  be  stated  as  follows: 

1.  Lesion  of  the  cortical  visual  area  on  one  side  gives  homonymous 
hemianopsia  of  the  opposite  half  of  the  visual  fields,  with  fully  pre- 
served pupillary  light  reflex. 

2.  Lesion  of  the  cortical  visual  area  on  both  sides  gives  total  blind- 
ness with  fully  preserved  pupillary  light  reflex. 

8.  Lesion  of  the  tractus  on  one  side  and  the  cortical  visual  area 
on  the  other  side  gives  total  blindness  with  pupillary  light  reflex  pre- 
served on  the  half  of  the  retina  corresponding  to  the  side  of  the  affected 
visual  area. 

4.  Lesion  of  both  tracts  causes  complete  blindness  with  complete 
loss  of  pupillary  light  reflex.®^ 

"For  cuts  of  the  pupillary  light  reflex  in  hemianopsiaj  see  Plate  II 
facing  page  290. 


CHAPTER    VII. 

PEEIPHERAL  AFFECTIONS  OF  THE  FIFTH,  SEVENTH,  AND 
CERVICAL  SYMPATHETIC  NEE\"ES :  OCULAR  LESIONS 
CAUSED   BY   THEM;    TREATMENT. 

By  EDWARD  JACKSON,  M.  D. 

THE    FIFTH    NERVE. 

The  differentiation  between  central  and  ■peripheral  lesions  of  the 
fifth  nerve  will  often  depend  on  the  location  of  the  sensations,  or  the 
lack  of  sensation,  experienced.  Sensations  referred  to  the  whole  dis- 
tribution of  the  nerve  or  to  widely  separated  portions  are  probably  cen- 
tral in  origin.  Those  confined  to  the  region  of  a  single  branch  are  prob- 
ably peripheral. 

Anatomy. — The  portions  of  the  fifth  nerve  having  close  relations 
with  the  eye  and  its  appendages  are  the  ophthalmic  and  superior  maxil- 
lary divisions,  particularly  the  former. 

Before  leaving  the  skull  the  ophthalmic  division  itself  divides  into 
lachrymal,  frontal,  and  nasal  branches,  which  enter  the  orbit  through 
the  sphenoidal  fissure.  The  lachrymal  branch,  entering  the  orbit 
towards  the  temporal  end  of  the  fissure,  passes  to  the  region  of  the  lach- 
rymal gland,  to  which  it  supplies  several  filaments ;  and  is  distributed 
to  the  conjunctiva  and  portions  of  the  skin  indicated  in  Fig.  1.  The 
frontal  branch,  entering  through  the  upper  middle  portion  of  the 
sphenoidal  fissure,  passes  forward  near  the  roof  of  the  orbit  to  the 
supra-orbital  foramen  or  notch,  througli  which  a  portion  emerges  as  the 
supra-orbital  branch,  the  supratrochlear  having  been  previously  given 
off  to  emerge  from  the  uj)pcr  inner  angle  of  the  orbit.  The  nasal  branch, 
entering  the  orbit  slightly  to  the  nasal  side  of  the  frontal  branch,  passes 
along  the  nasal  wall  of  the  orbit,  enters  the  ethmoid  through  the  anterior 
ethmoidal  foramen,  and  is  distributed  chiefly  to  the  nose  (See  Page  15). 

The  superior  maxillary  division  of  the  nerve,  passing  through  the  fora- 
men rotundum  across  the  sphenomaxillary  fossa  and  through  the  infra- 
orbital canal  emerges  from  the  infra-orbital  foramen.  Its  temporal 
and  malar  branches,  given  off  in  the  sphenomaxillary  fossa,  pass  througli 
the  malar  bone;  and  the  palpebral  and  nasal  branches,  given  off  after 
it  emerges  from  the  foramen,  are  distributed  to  the  lids,  conjunctiva, 
and  region  of  the  lachrymal  sac. 
320 


THE  FIFTH  NERVE 


321 


The  general  distribution  of  the  branches  of  the  fifth  nerve  to  the 
skin  of  the  head  and  face  is  shown  in  Fig.  1. 

The  most  important  anastomoses  of  the  trigeminus  are  those  with  the 
sympathetic  nerves,  especially  its  connection  with  the  ciliary  ganglion. 
It  should  be  borne  in  mind  that  beside  the  superficial  distribution  iudi- 


Supra-trochlear 
l.V 


■Infra-trocMear 
l.V 


yasal  I'V 


Fig.  1.— Nonnal  distribution  of  the  fifth  nerve  to  the  face.  1.  T'— ophthalmic  division  ;  2.  F— su- 
perior maxillary  ;  S.  T— inferior  maxillary.  The  names  on  the  different  areas  indicate  the  branches 
supplying  them.    (Flower.) 


cated  in  Fig.  1  the  fifth  is  also  the  nerve  of  general  sensation  for  the 
anterior  part  of  the  dura  mater,  and  the  mucous  membrane  of  the  ear, 
nose,  throat,  accessory  sinuses,  the  mouth,  and  the  upper  portion  of  the 
pharynx. 

Physiology. — Besides  serving  to  transmit  the  impressions  of  touch, 
pressure,  and  temperature,  and  to  excite  pain,  the  fifth  nerve  is  con- 
cerned in  very  important  reflexes.  Hyperaemia  in  response  to  corneal 
irritation  may  involve  the  vessels  of  the  optic  disk  and  retina  as  well 
as  those  of  the  pericorneal  zone.  Contraction  of  the  pupil  in  response 
to  such  irritation  is  often  very  striking.  The  increase  of  the  lachrymal 
secretion  may  be  a  reflex  response  to  irritation  of  any  portion  of  the 
mucous  membrane  supj^lied  by  this  nerve.  Its  fibres  may  also  carry 
important  efferent  impulses  governing  secretion,  or  the  nutrition  of  the 
various  tissues  (trophic). 

Anaesthesia. — In  rare  cases  absence  of  sensibility  over  parts  or  the 
whole  of  the  distribution  of  the  fifth  nerve  appears  to  be  due  to  con- 
genital defect.  More  frequently  it  arises  from  lesions  due  to  pressure, 
inflammation,  or  toxic  disturbance' of  the  nerve  trunk  or  its  branches. 
Chief  among  these  toxic  influences  are  malaria,  lead,  and  auto-intoxi- 
21 


322  THE    EYE    AND    NERVOUS    SYSTEM 

cation  of  intestinal  origin.  The  location  of  the  ana'sthesia  will  be  deter- 
mined by  the  nerve  distribution.  Thus,  small  areas  indicate  that  the 
course  of  the  disturbance  is  acting  upon  correspondingly  few  peripheral 
filaments.  Anaesthesia  involving  the  whole  distribution  of  an  important 
branch,  such  as  the  supraorbital,  indicates  that  this  nerve  is  affected 
before  breaking  up  into  its  terminal  filaments.  AMien  the  anaesthetic 
region  involves  the  distribution  of  all  the  branches  of  one  of  the  great 
divisions  of  the  nerve — the  ophthalmic  or  the  infraorbital — the  cause 
must  act  close  to  where  such  divisions  emerge  from  the  skull.  The 
anaesthesias  of  more  peripheral  origin,  and  therefore  involving  smaller 
areas,  are  very  much  more  likely  to  be  overlooked.  This  may  be  the 
reason  that  the  extensive  ana'sthesias  of  intracranial  origin  appear  to 
be  the  more  common. 

Corneal  ancestlwsia  is  of  especial  interest.  The  sensibility  of  the 
cornea  is  commonly  tested  by  touching  it  with  a  thread  or  a  few 
fibres  of  absorbent  cotton  rolled  into  a  point.  For  purposes  of  com- 
parison, tlie  sound  cornea  may  be  similarly  tested  in  alternation  with 
the  cornea  suspected  of  anaesthesia  or  hyperaesthesia.  The  sensitiveness 
is  to  be  judged  by  the  apparent  reaction  produced,  as  well  as  by  the 
patient's  statements. 

The  relative  ease  with  which  anaesthesia  of  the  cornea  is  produced 
by  the  use  of  cocaine,  holocaine,  and  other  local  anaesthetics  seems  to 
indicate  that  there  is  an  especial  liability  of  the  corneal  nerves  to  such 
functional  impairment.  Tn  malarial  disease  anaesthesia  of  the  cornea 
may  be  demonstrated  when  no  other  evidence  of  trigeminal  anaesthesia 
can  ha  discovered.  The  writer  has  observed  it  confined  to  a  certain 
segment  of  the  cornea,  the  sensibility  of  other  parts  being  normal.  The 
corneal  anaesthesia  of  glaucoma  is  ascribed  to  pressure  on  the  nerve- 
trunks  as  they  pass  forward  within  the  sclera.  It  is  proportioned  to 
the  increase  of  the  intra-ocular  tension,  and  to  the  rapidity  of  that 
increase. 

Paraesthesia. — Perverted,  abnormal,  and  usually  disagreeable  sen- 
sations— like  burning,  pricking,  tingling,  numbness,  or  fulness  of  the 
parts  supplied  by  the  fifth  nerve — are  more  frequently  recorded  as  due 
to  central  causes.  But  they  may  arise  from  abnormal  states,  as  neuritis 
of  the  peripheral  branches.  After  injuries — particularly  those  causing 
bruising  of  the  soft  tissues,  or  fracture  of  the  bones  about  the  orbit — 
such  sensations  may  persist  for  many  months  or  even  for  several  years. 
Gradually  they  become  less  constant  and  less  annoying  until  they  en- 
tirely disappear.  After  some  ocular  inflammations,  especially  inflam- 
mations of  the  iris  and  cornea,  sensations  of  the  kind  may  be  complained 


THE  FIFTH  NERVE  323 

of  for  considerable  periods.  Some  of  the  discomforts  which  persist 
after  the  removal  of  the  eye-strain  that  had  been  attended  by  chronic 
congestion,  may  be  of  this  character. 

In  overcoming  these  disagreeable  sensations,  time  is  the  most  im- 
portant factor,  but  something  may  be  done  by  avoiding,  so  far  as  pos- 
sible, any  source  of  irritation  or  strain,  or  any  observed  exciting  cause. 

Neuralgia  and  Neuritis. — Xeuralgic  pain  of  peripheral  origin  is 
probably  generally  due  to  neuritis.  At  least,  in  practice  we  are  unable  to 
distinguish  between  pain  which  is  due  to  neuritis  and  that  which  is  not. 
When  such  pain  is  confined  to  a  small  area  of  the  skin  distribution  of 
the  fifth  nerve,  its  character  may  be  readily  recognized.  But  when  it  is 
referred  to  the  eyeball  itself,  its  significance  becomes  more  difficult  to 
estimate.  The  latter  will  be  considered  under  the  next  heading.  Neu- 
ralgic pain  attended  with  facial  spasm  (prosopalgia  or  tic  douloureux) 
is  discussed  under  facial  spasm. 

Ocular  Pain. — It  is  very  important,  but  often  difficult,  to  deter- 
mine the  exact  character  of  pain  referred  to  the  eye.  The  two  most 
important  varieties  are  the  pain  such  as  is  produced  by  irritation  of 
the  conjunctival  or  corneal  nerve-endings,  and  such  as  would  arise  from 
deeper  lesions.  The  former  is  described  as  scratching,  smarting,  or 
the  feeling  of  a  foreign  body;  the  latter  rather  as  aching.  Burning 
and  itching  may  arise  from  either  set  of  causes,  but  these  words  arc 
more  commonly  used  with  reference  to  pain  of  superficial  origin. 
Boring,  shooting,  or  stinging  pains  are  usually  of  deep  origin,  although 
the  latter  terms  may  be  applied  to  sensations  better  referred  to  as 
scratching. 

The  exact  location  to  which  pain  is  referred  may  be  of  important 
significance  but  it  cannot  be  wholly  relied  on.  By  the  scratching  felt 
in  a  certain  position,  the  location  of  a  minute  foreign  body  or  super- 
ficial lesion  may  be  ascertained.  But  with  regard  to  pain  of  deeper 
origin,  its  referred  location  is  likely  to  prove  misleading.  The  pain 
of  iritis  or  glaucoma,  if  severe,  will  always  be  felt  more  or  less  in  the 
brow  or  cheek.  Often  it  is  complained  of  chiefly  or  entirely  in  these 
regions.  The  mistaking  of  these  diseases  for  trigeminal  neuralgia  is 
extremely  common  among  practitioners  whose  attention  is  not  especially 
fixed  upon  the  eye.  Pain  referred  to  the  distribution  of  the  ophthalmic 
or  superior  maxillary  divisions  of  the  fifth  nerve  should  always  lead 
to  a  careful  examination  of  the  eye-ball ;  and  pain  of  ocular  origin  may 
be  even  more  remotely  referred. 

The  persistence  of  para?sthesia  after  injury  has  been  referred  to. 
Pain  may  be  equally  persistent.     Even  though  no  organic  disease  may 


324  THE    EYE    A:^D    NERVOUS    SYSTEM 

be  discoverable,  pain  may  continue  as  a  sort  of  morbid  habit.  This  is 
noticed  especially  after  the  relief  of  eye-strain  that  has  long  caused 
headache.  But  in  such  cases  the  suffering  gradually  diminishes.  Some- 
times after  an  operation  on  the  eyeball  or  after  accidental  injury  or 
severe  inflammation,  pain  out  of  all  proportion  to  the  severity  of  other 
symptoms  may  persist  for  a  long  time.  In  these  cases  it  might  reason- 
ably be  supposed  that  a  neuritis  of  the  nerve-endings  of  the  fifth  nerve 
had  arisen;  and  treatment  directed  in  accordance  with  this  supposi- 
tion gives,  after  the  necessary  lapse  of  time,  satisfactory  results. 

Neurotic  CEdema. — Localized  oedema  of  the  conjunctiva  or  lids, 
or  both,  apparently  dependent  on  nerve  influence,  is  not  very  rare. 
Sometimes  it  is  accompanied  by  vascular  engorgement  of  the  part.  But 
in  many  cases  this  is  not  noticeable.  The  acute  attack  may  be  ushered 
in  by  burning,  stinging,  or  itching  of  the  parts,  followed  by  very  rapid 
swelling,  which  may  cause  complete  closure  of  the  eye.  'Usually  within 
twelve  to  forty-eight  hours  the  swelling  begins  to  diminish,  and  in  three 
or  four  days  the  parts  are  restored  to  their  normal  appearance.  Com- 
monly but  one  eye  is  affected,  but  the  swelling  may  extend  from  one 
side  to  the  other,  or  both  may  be  originally  attacked.  Often  there  is 
similar  acute  oedema  of  other  parts.  But  the  ocular  symptoms  may  be 
the  only  ones  complained  of. 

Some  of  these  attacks  closely  resemble  urticaria  in  their  origin  and 
nature.  They  may  be  provoked  by  use  of  certain  articles  of  diet,  as 
fish  or  shell-fish,  or  the  drinking  of  alcoholic  beverages.  In  a  few 
persons  large  doses  of  potassium  iodide  will  produce  enormous  con- 
junctival swelling  of  this  character. 

Chronic  oedema  of  the  lids,  liable  to  increase  from  errors  of  diet, 
exposure  to  wind  or  dust,  use  of  the  eyes,  or  loss  of  sleep,  is  sometimes 
a  very  annoying  and  obstinate  condition.  It  may  continue  to  give 
trouble  for  many  years.  The  treatment  includes  protection  of  these 
parts  from  local  irritation ,  and  careful  avoidance  of  the  exciting  cause, 
when  this  can  be  discovered. 

Trophic  Disorders. — It  is  uncertain  whether  trophic  disorders  in 
the  region  of  the  fifth-nerve  distribution  are  often  of  extracranial  origin. 
But,  as  their  peripheral  manifestations  are  well  kno%vn  and  the  causa- 
tive lesions  unknown  or  extremely  obscure,  it  is  convenient  to  consider 
them  in  this  connection. 

Facial  hemiatrophy  involves  to  some  extent  the  lids  and  the  bones 
of  the  orbit,  but  produces  no  evident  alterations  in  the  eyeball  itself. 
Unilateral  hypertrophy  of  the  face  may  involve  the  same  parts.  Whiten- 
ing or  falling  of  the  lashes  or  hairs  of  the  eyebrow  on  one  side,  or  a 


THE  FIFTH  [NERVE  325 

portion  of  them,  is  ascribed  to  morbid  nerve  influence.  Disorders  of 
secretion,  hvperidrosis,  or  chromidrosis,  localized  erythema,  itching, 
and  perhaps  even  gangrene,  may  have  a  similar  origin. 

Neuropathic  Keratitis. —  Magendie  observed  that  section  of  the  tri- 
geminus in  rabbits  was  followed  by  inflammation  and  destruction  of 
the  cornea.  He  assumed  that  this  depended  upon  the  loss  of  a  trophic 
influence  necessary  to  sustain  the  normal  corneal  nutrition.  Snellen 
suggested  that  lack  of  sensibility  in  the  cornea  permitted  injuries  to 
occur  to  it;  and  foreign  bodies  to  remain  in  contact  with  it,  by  sus- 
pending reflex  movements  of  the  lids  and  lachrymation ;  and  it  was 
shown  that  after  section  of  the  trigeminus,  careful  protection  of  the 
eye  prevented  keratitis.  Keratitis  can  be  prevented  by  fully  protecting 
the  eye  for  a  few  weeks  after  operative  removal  or  destruction  of  the 
Gasserian  ganglion.  Claude  Bernard  called  attention  to  the  share  which 
vasomotor  changes  might  have  in  producing  this  form  of  corneal  inflam- 
mation. Turner  and  Ferrier,  by  experimental  divisions  of  the  fifth 
nerve,  its  roots  and  branches,  with  protection  of  the  eye  from  injuries, 
reached  the  conclusion  that  keratitis  from  injury  or  disease  of  the  tri- 
geminus was  the  result  of  an  irritation  rather  than  a  paralysis. 

Many  recent  writers,  apparently  from  an  incomplete  survey  of  the 
subject,  have  accepted  the  explanation  of  Snellen,  that  it  was  a  kera- 
titis of  traumatic  origin,  which  might  be  rendered  grave  by  intercur- 
rent infection.  But,  as  pointed  out  by  Harlan,^  such  an  explanation 
does  not  meet  all  the  known  facts.  The  clinical  appearance  and  course 
of  the  disease  differ  radically  from  those  of  an  ordinary  traumatic 
infective  keratitis.  Harlan  reported  a  case  in  which  the  cornea  re- 
mained clear  under  prolonged  exposure  due  to  facial  paralysis,  and  sub- 
sequently sloughed  when  it  was  protected  by  paralysis  of  the  elevator  of 
the  upper  lid.  In  a  case  reported  by  Xorris  the  sloughing  of  the  cornea 
occurred  in  spite  of  careful  protection  against  injury.  It  must  also 
be  borne  in  mind  that  neuropathic  keratitis  occurs  in  connection  with 
trigeminal  disease  without  anaesthesia  of  the  cornea,  and  that  corneal 
anaesthesia  due  to  glaucoma  is  not  attended  with  any  such  inflammation. 

In  the  present  state  of  our  knowledge  a  rational  view  seems  to  be, 
that  disease  or  injury  involving  the  trunk  or  ganglion  of  the  fifth  nerve 
causes  or  predisposes  to  corneal  disease,  partly  by  an  obscure  irritative 
influence  and  partly  by  favoring  traumatism  and  infection.  But  the 
different  hypotheses  above  mentioned  should  all  be  borne  in  mind,  since 
trophic  influence,  injury,  vasomotor  disturbances,  and  central  irritation 


'  Transactions  of  the  American  Ophthalmological  Society,   1897. 


326  THE    EYE    AND    NERVOUS    SYSTEM 

may  all  be  factors  in  the  case  and  may  require  to  be  met  by  different 
therapeutic  influences. 

The  clinical  course  of  the  disease  may  be  that  of  a  general  soften- 
ing or  sloughing  of  the  cornea.  More  commonly,  as  the  writer  has  seen 
it,  it  begins  with  clouding  of  the  central  portion  of  the  cornea.  This 
clouding  impairs  vision.  It  is  accompanied  by  some  general  hyper- 
a?mia  of  the  eyeball,  but  with  comparatively  little  inflammatory  pain 
or  reaction.  Lachrymation  is  not  increased,  but  the  corneal  surface  is 
not  especially  dry.  The  sensibility  of  the  cornea  is  usually  markedly 
diminished.  But  this  may  not  be  the  case.  Ulceration  is  liable  to 
occur  first  in  the  central  portion  of  the  cornea. 

The  ulcer  may  become  infected,  hypopion  form,  and  the  cornea 
slough.  In  a  fair  proportion  of  cases  the  process  does  not  go  on  to 
perforation;  but  the  cornea  heals  with  permanent  opacity.  Central 
vision  is  thus  greatly  impaired  or  destroyed,  but  the  eye  may  still  pre- 
serve a  useful  peripheral  field.  The  corneal  disease  may  be  attended 
from  an  early  stage  with  evidences  of  iritis,  including  adhesions,  swell- 
ing, and  discoloration  of  the  lids. 

Some  writers  have  applied  the  description  of  keratitis  due  to  ex- 
posure and  desiccation  of  the  cornea — lagophthalmic  keratitis — to  this 
disease.  The  two  conditions,  however,  differ  markedly  in  important 
clinical  features.  Keratitis  from  exposure  begins  with  dryness,  ulcera- 
tion, and  infection  in  the  lower  part  of  the  cornea,  while  in  true  neu- 
ropathic keratitis  it  is  the  central  region  of  the  cornea  that  is  first  and 
most  severely  affected.  Exposure  keratitis  has  the  appearance  and  runs 
the  course  of  an  infective  ulcer.  Neuropathic  keratitis  shows  a  deep 
opacity  of  the  central  part  of  the  cornea  preceding  or  without  ulcera- 
tion. 

Treatment. — The  local  treatment  of  neuropathic  keratitis  includes 
careful  protection  of  the  cornea  by  union  of  the  lids  or  the  occlusive 
bandage,  the  cautious  application  of  hot  stupes  or  dry  heat,  careful 
cleansing  of  the  eye  with  physiologic  salt  solution  or  very  weak  anti- 
septic solutions,  and  the  instillation  of  atropine.  The  prognosis,  how- 
ever, must  depend  largely  upon  the  nerve  element  in  the  ease.  If  this 
can  be  improved  by  general  regimen,  tonics,  or  the  very  cautious  appli- 
cation of  electricity,  such  measures  must  not  be  neglected. 

Herpes  Zoster. — Numerous  cases  of  corneal  leucoma,  associated 
with  scars  on  the  face  and  scalp  in  the  area  of  distribution  of  the  oph- 
thalmic division  of  the  trigeminus,  with  the  history  that  this  corneal 
disease  occurred  with  an  attack  of  what  was  regarded  as  erysipelas, 
called  the  attention  of  Jonathan  Hutchinson,  Sr.,  to  tlie  importance  of 


THE  FIFTH  NERVE  327 

tlie  eye  lesions  of  herpes  zoster  affecting  this  region.  An  acute  clinician 
ought  to  be  able  at  any  stage  to  recognize  that  he  has  to  deal  with  zoster 
rather  than  erysipelas;  and  yet  the  diagnosis  is  easier  after  the  case 
has  run  its  course  than  it  is  in  the  early  stages. 

Herpes,  unlike  erysipelas,  is  preceded  by  severe  burning,  neuralgic 
pain  in  the  affected  part,  and  is  not  preceded  by  a  chill,  high  fever, 
marked  headache  and  other  evidences  of  severe  general  sickness.  The 
eruption  of  herpes  zoster  begins  with  isolated  spots,  upon  which  vesicles 
develop,  instead  of  having  the  redness  and  swelling  general  from  ihe 
start.  After  the  eruption  begins  to  appear,  there  is  no  extension  of  the 
region  affected,  and  there  is  but  little  involvement  of  the  lymphatic 
glands. 

The  disease  is  unilateral,  the  eruption  being  sharply  limited  near, 
but  often  not  exactly  at,  the  median  line.  The  distribution  corresponds 
to  the  more  or  less  variable  areas  of  nerve  distribution.  The  skin  of 
the  whole  affected  region  becomes  inflamed,  causing  severe  swelling  of 
the  lids  and  the  appearance  which  gives  rise  to  the  diagnosis  of  ery- 
sipelas. The  eruption  runs  its  course  in  from  one  to  three  weeks,  each 
vesicle  giving  rise  to  a  scab  which  falls  off  leaving  a  permanent  depres- 
sion. The  neuralgic  pain  may  disappear  with  a  full  development  of 
the  eruption,  but  often  it  persists,  especially  in  elderly  people,  after 
all  inflammatory  symptoms  have  disappeared ,  sometimes  causing  great 
suffering  for  many  weeks  or  months. 

The  ocular  lesions  attending  ophthalmic  herpes  are  quite  varied. 
Wilbrand  and  Saenger  have  brought  together  cases  of  involvement  of 
the  iris,  ciliary  body,  chorioid,  retina,  and  optic  nerve,  increase  and 
diminution  of  intra-ocular  tension,  and  paralysis  of  the  internal  and 
external  muscles  of  the  eye.  In  a  few  cases  it  has  been  followed  by 
paralysis  of  the  facial  nerve.  These  complications  appear  to  present 
the  usual  characters  of  inflammation,  degeneration,  or  paralysis  of  these 
respective  parts. 

The  common,  dangerous,  and  characteristic  ocular  lesion  is  pre- 
sented by  the  cornea.  With  the  development  of  the  skin  eruption  vesi- 
cles may  appear  on  the  conjunctiva  and  cornea,  giving  rise  in  the  latter 
situation  to  corneal  ulceration.  The  corneal  ulcer  may  extend  and 
perforate.  But  without  perforation  and  even  without  ulceration,  while 
the  eruption  is  at  its  height,  a  dense  opacity  may  develop  in  the  depth 
of  the  cornea,  leaving  a  permanent  leucoma  or  dense  nebula.  The  cor- 
neal inflammation  is  apt  to  last  much  longer  than  the  skin  eruption 
with  which  it  begins;  and  such  clearing  of  the  opacity  as  does  occur 
takes  place  very  slowly,  and  may  not  begin  until  after  the  persistence 


328  THE    EYE    AND    NERVOUS    SYSTEM 

of  the  neuralgic  pain  has  ceased  to  give  serious  annoyance.  The  cornea 
will  often  be  found  quite  ana?sthetic,  the  anaesthesia  continuing  through 
the  period  of  pain  and  maximum  opacity.  In  many  respects  the  cor- 
neal disease  has  the  characteristics  of  neuropathic  keratitis. 

Causes. — The  pathology  of  herpes  zoster  is  not  fully  worked  out. 
But  tlie  ocular  and  skin  lesions  seem  to  depend  upon  inflammatory 
changes,  hemorrhage,  and  subsequent  degeneration  in  the  Gasserian 
ganglion.  The  inflammation  may  extend  to  the  nerve-trunk,  and  the 
degenerative  changes  through  the  roots  of  the  nerve  into  the  central 
nervous  system.  Head  and  Campbell^  believe  that  herpes  zoster  is  a 
specific  disease  with  a  definite  period  of  incubation,  and  occurring  in 
epidemics.  More  generally  it  has  been  ascribed  to  cold  or  to  some 
toxic  influence.  Arsenic  and  exposure  to  charcoal  fumes  have  been 
regarded  as  especially  liable  to  cause  it. 

Treatment.— The  local  treatment  should  be  the  same  as  for  neuro- 
pathic keratitis,  protective  and  non-irritative  (see  page  848).  When 
all  pain  has  disappeared  and  the  sensibility  of  the  cornea  has  been 
largely  restored  the  usual  remedies  for  corneal  opacity  may  be  resorted 
to.  But  the  permanent  opacity  is  likely  to  be  out  of  proportion  to  the 
loss  of  corneal  tissue. 

Prognosis. — The  prognosis  varies  with  the  severity  of  the  attack, 
the  presence  of  ulceration,  deeper  opacity  of  the  cornea,  and  the  occur- 
rence of  iritis,  or  involvement  of  other  deeper  structures  of  the  eye. 
In  general  the  danger  of  a  prolonged  or  permanent  disability  of  the 
eye  increases  with  the  age  of  the  patient.  It  has  been  noted  that  the 
cornea  is  more  likely  to  suffer  in  those  cases  in  which  vesicles  appear 
on  the  side  of  the  nose.  But  severe  corneal  lesions  may  occur  when 
the  skin  of  the  nose  is  not  at  all  involved,  and  the  cornea  may  escape, 
although  well-marked  vesicles  or  scars  are  found  upon  the  nose.  In 
all  cases  the  prognosis  should  be  guarded,  especially  as  to  the  time  of 
recovery.  Some  eyes  remain  irritable  and  painful  many  months  and 
years  after  the  attack,  arid  there  is  no  way  of  judging  in  the  early  stages 
which  cases  will  run  such  a  course. 

Other  Forms  of  Herpes. — It  must  not  be  forgotten  that  other  forma 
of  herpes  occur  in  and  about  the  eye.  In  rare  cases  the  ordinary  febrile 
herpes  has  been  observed  occurring  on  the  lids  as  it  generally  occurs 
upon  the  lips.  Herpes  of  the  cornea  occurs  in  connection  with  various 
febrile  diseases,  especially  affecting  the  respiratory  tract.  Symptoms 
of  severe  corneal  irritation  attend  the  development  of  the  vesicles,  and 


Brain,  1900,  No.  91. 


THE  FIFTH  XERVE  329 

their  rupture  leaves  a  number  of  superficial  ulcers.  But  these  ulcers 
commonly  heal  without  leaving  any  penuanent  impairment  of  vision. 
Phlyctenular  disease  of  the  conjunctiva  and  cornea  also  exhibits  many 
of  the  characteristics  of  an  herpetic  eruption.  There  can  be  little  doubt 
that  the  peripheral  branches  of  the  fifth  nerve  perform  an  essential 
part  in  the  production  of  all  these  lesions.  In  febrile  herpes  the  dis- 
turbance may  be  due  to  poisoning  or  impaired  nutrition  of  the  nerve. 
In  phlyctenular  disease  it  has  been  ascribed  to  the  local  action  of  various 
bacterial  toxines. 

Recurring  Erosion  of  the  Cornea. — After  a  superficial  injury,  as  a 
finger-nail  scratch  of  the  cornea,  attacks  of  severe  pain  with  or  without 
loss  of  epithelium  may  recur  for  months  or  even  years.  Injury  to  the 
peripheral  nerve-endings  of  the  cornea  is  probably  an  e'ssential  factor  in 
producing  this  condition. 

Dendritic  ulcer  of  the  cornea,  as  has  been  pointed  out  by  Charles,^ 
probably  owes  its  peculiar  character  to  lesions  affecting  the  terminal 
filaments  of  the  corneal  nerves  between  Bowman's  membrane  and  the 
epithelium. 

Supposed  Reflex  Blindness  and  Palsies. — From  the  time  of  Hip- 
pocrates it  has  been  known  that  various  injuries  in  the  region  supplied 
by  the  fifth  nerve  were  liable  to  be  followed  by  blindness  or  by  paralysis 
of  one  or  more  of  the  ocular  muscles.  It  is  now  known  that  in  many 
of  these  cases  the  blindness  or  paralysis  is  due  to  direct  injury  to  the 
optic  nerve  or  to  the  motor  nerve  supplying  the  affected  muscles. 
Wounds  about  the  orbit  often  penetrate  more  deeply  than  is  suspected, 
and  hemorrhage  or  thrombosis  may  carry  their  effects  still  deeper. 
Post-mortem  examination  has  demonstrated  that  of  fatal  cases  of  frac- 
ture of  the  cranium  from  blows  upon  the  region  supplied  by  the  fifth 
nerve  the  fracture  involved  the  orbit  in  68  per  cent.,  and  in  51  per 
cent,  extended  into  the  optic  foramen. 

Xevertheless,  the  view  that  optic  nerve  atrophy  and  paralysis  of 
the  motor  nerves  of  the  eye  might  be  produced  by  trophic  influences 
set  up  by  injury  to  the  peripheral  branches  of  the  fifth  nerve  has  been 
accepted  by  many  excellent  observers,  even  in  recent  years.  Hubbell,^ 
who  has  recently  supported  this  hypothesis,  cites  a  long  list  of  famous 
clinical  observers  who  are  inclined  to  accept  it.  To  the  writer  it  seems 
more  reasonable  to  suppose  that  all  cases  of  the  class  are  caused  in  the 
same  or  closely  similar  manner  in  which  we  know  that  a  large  propor- 


*  American  Journal  of  Ophthalmology,  April,  1904. 

*  Transactions  of  the  Section  on  Ophthalmology  of  the  A.  M.  A.,  1904. 


;J30  THE    EYE    AND    NERVOUS    SYSTEM 

tion  are  caused,  than  to  Lave  recourse  to  an  hypothesis  so  indefinite  and 
unsupported  as  that  of  a  harmful  trophic  influence  exerted  by  the  fifth 
nerve. 

Photophobia. — Although  the  term  photophobia  (fear  of  light) 
might  be  expected  to  indicate  only  symptoms  arising  from  the  retina 
and  optic  nerve,  it  is  applied  to  phenomena  more  generally  connected 
with  irritations  acting  upon  the  peripheral  branches  of  the  fifth  nerve. 
It  is  easy  to  see  how  light  falling  upon  an  eye  with  an  inflamed  iris 
may  cause  disturbance  of  the  nerves  of  general  sensation  distributed 
to  the  iris  by  the  reaction  it  provokes  causing  movements  in  the  inflamed 
tissue.  But  in  some  of  the  most  marked  cases  of  photophobia  it  is 
evident  that  light  lias  little  or  nothing  to  do  with  the  production 
of  the  symptonft.  The  child  with  phlyctenular  keratitis  buries  its 
head  in  the  pillow  as  strenuously  and  persistently  at  night  or  in  the 
darkened  room  as  in  full  daylight.  And  its  fear  of  light  is  removed 
by  measures  that  diminish  the  hypersensitiveness  of  the  fifth-nerve 
endings,  but  which  have  no  appreciable  effect  upon  the  retina  and  optic 
nerve. 

There  is  a  form  of  discomfort  about  the  eyes,  and  aching  often 
becoming  a  general  headache,  which  seems  directly  connected  with 
exposure  to  light.  This  may  be  experienced  by  most  persons  who  are 
much  indoors  with  feeble  illumination.  When  they  go  directly  into 
strong  sunlight,  the  pain  may  arise  instantly,  and  may  have  the  char- 
acter of  a  sharp  cramp,  probably  connected  with  excessive  contraction 
of  the  sphincter  of  the  pupil.  The  contrast  between  bright  lights  and 
comparative  darkness,  produced  by  looking  towards  sources  of  artificial 
illumination  at  night,  is  quite  unpleasant  to  most  people.  Excessive 
liability  to  similar  unpleasant  sensations  is  noted  in  patients  suffering 
from  acute  or  chronic  wasting  disease,  attended  with  toxaemia,  especially 
after  typhoid  fever  and  in  the  course  of  general  tuberculosis.  Photo- 
phobia of  the  kinds  above  mentioned  may  have  little  connection  with 
the  fifth  nerve,  and  that  only  through  the  nerve-centres. 

But  the  photophobia  which  attends  the  presence  of  a  foreign  body 
in  the  eye,  or  inflammatory  irritation  of  the  conjunctiva,  cornea  or  iris, 
is  clearly  a  fifth-nerve  symptom.  It  is  associated  with  no  characteristic 
anatomical  changes  in  the  nerve.  Indeed,  the  production  of  this  form 
of  pain  can  hardly  be  regarded  as  an  abnormality  of  the  nerve  function, 
since  it  seems  necessary  to  secure  rest  and  protection  or  relief  from 
the  irritation  that  causes  the  photophobia.  In  this  form  of  photophobia 
there  is  a  tendency  to  spasmodic  closure  of  the  lids,  excessive  lachry- 
mation,  and  general  hypera?mia  of  the  eyeball.     In  the  more  severe 


THE  FIFTH  NERVE  331 

•cases  the  face  may  be  continuously  buried  in  a  pillow,  cushion,  or  hand- 
kerchief, or  the  eyes  exposed  to  the  air  and  light  only  with  great  diffi- 
culty, and  for  a  short  time. 

Treatment.— Photophobia  which  is  rarely  connected  with  exposure 
to  light  should  be  met  by  measures  that  will  lessen  the  general  erethism 
of  the  nervous  system,  and  by  avoiding  contrasts  of  illumination,  either 
successive,  by  going  from  darkness  suddenly  to  light,  or  simultaneous, 
as  by  facing  sources  of  artificial  light  at  night,  or  the  narrow  streaks 
of  light  about  a  shaded  window.  But  it  is  important  not  to  have  the 
patient  give  way  too  much  to  the  desire  to  avoid  diffuse  daylight.  If 
■dark  glasses  are  worn  they  should  be  resorted  to  only  at  times  of  ex- 
posure to  the  brightest  light,  so  as  to  lessen  the  contrast.  They  should 
not  be  used  continuously.  Habitual  exposure  to  moderate  light  is  the 
physiologic  and  most  efficient  method  of  keeping  down  the  hypersensi- 
tiveness  of  the  retina. 

Photophobia  dependent  on  corneal  irritation  demands  chiefly  the 
removal  of  the  irritation.  The  avoidance  of  exposure  to  light  and  air 
may,  however,  tend  to  increase  the  photophobia.  Often  the  dressings 
worn  upon  the  eye  aggravate  and  continue  the  symptom  when  it  would 
otherwise  have  subsided.  Photophobia  is  usually  an  indication  for 
removal  of  bandages  and  avoidance  of  anything  that  will  keep  up  the 
heat  and  moisture  of  the  parts.  It  is  often  wise  to  substitute  dark 
glasses  for  a  close  dressing,  simply  because  they  permit  the  free  access  of 
air,  and  continuous  cooling  of  the  parts  by  evaporation.  Strongly 
astringent  applications,  as  silver  nitrate,  boroglyceride,  etc.,  do  good 
by  removing  the  hypersensitiveness  of  the  nerve-endings. 

In  photophobia  attended  by  lachrymation  small  fissures  in  the  skin 
are  apt  to  appear  about  the  outer  canthus  (rhagades).  The  treatment 
of  these,  as  by  touching  with  a  strong  solution  of  silver  nitrate,  will 
^eatly  lessen  the  photophobia.  The  local  application  of  cocaine  or 
holocaine  within  the  conjunctival  sac  temporarily  relieves  photophobia. 
But  with  the  passing  of  the  anaesthetic  influence  the  symptom  returns 
with  renewed  force,  and  frequent  reapplications  of  cocaine  to  keep 
up  the  anaesthetic  influence  are  extremely  dangerous. 

In  corneal  disease  the  influence  of  atropine  upon  the  nerve-endings 
in  the  cornea  gives  a  decided  and  much  more  permanent  relief,  and 
usually  proves  beneficial  also  for  the  condition  causing  the  photophobia. 
Wliere  photophobia  is  attended  with  spasmodic  closure  of  the  lids  and 
does  not  yield  to  other  measures,  it  will  usually  be  greatly  lessened  by 
canthotomy  or  canthoplasty.  The  old  plan  of  suddenly  plunging  the 
head  into  cold  water  is  efficient,  but  not  often  practical. 


332 


THE    EYE    AXD    :N^ERV0US    SYSTEM 


THE   SEVENTH    NERVE. 

The  course  of  the  seventh,  or  facial,  nerve  through  the  temporal 
bone,  and  its  numerous  anastomoses  and  chief  branches  of  distribution, 
are  shown  in  Fig.  2  (Compare  Page  17). 

Entering  the  internal  auditory  meatus  it  lies  in  close  relation  with 
the  auditory  nerve,  so  that  any  gross  lesion  of  this  portion  will  be 
attended  by  vertigo,  abnormal  movements,  and  deafness  of  the  same 
side.     But  there  is  no  deafness  or  hemiplegia  of  the  opposite  side, 


Fig.  2 — Seventh  nerve  (facial)  and  its  branches  and  anastomoses.    (Leube.) 


or  abducens  paralysis  of  the  same  side,  which  would  attend  an  intra- 
cranial lesion.  Leaving  the  auditory  nerve,  it  enters  the  Fallopian 
canal.  At  the  geniculate  ganglion,  where  the  nerve  and  canal  bend 
sharply  downward,  are  given  off  the  petrosal  branches,  communicating 
with  the  spheno-palatine  ganglion  and  the  otic  ganglion.  Clinical 
evidence  of  the  involvement  of  these  branches  has  not  been  recognized. 
A  little  lower  a  branch  is  given  off  to  the  stapedius  muscle,  and 
paralysis  of  this  muscle  may  cause  abnormal  acuteness  of  hearing. 
Hence  a  lesion  occurring  in  the  Fallopian  canal,  but  not  involving  its 
bony  wall  or  the  structures  contiguous  to  them  may  be  attended  by 
hyperacusis  instead  of  by  deafness.  A  little  lower  still  the  chorda 
tympani  is  given  off.  Lesions  centered  at  this  point  are  attended  with 
disturbance  of  the  salivary  secretion  and  taste.  More  peripheral  lesions 
leave  taste  undisturbed,  while  affecting  all  the  external  muscles  inner- 
vated by  the  facial  nerve.  At  the  stylomastoid  foramen  the  posterior 
auricular,  digastric,  and  stylohyoid  branches  are  given  off,  so  that  extra- 


THE  SEVEXTH  XERVE 


333 


cranial  lesions  leave  them  unaffected.  There  are  also  minute  twigs 
sent  to  the  sympathetic  and  glossopharyngeal  and  pneumogastric  nerves. 

Emerging  from  the  stylomastoid  foramen,  the  facial  nerve  runs 
within  the  parotid  gland  across  the  external  carotid  artery.  Behind 
the  ramus  of  the  lower  jaw  it  divides  into  the  cervicofacial  branch, 
distributed  to  the  neck  and  lower  part  of  the  face,  and  the  temporo- 
facial,  which  splits  up  into  the  temporal,  malar,  and  infra-orbital 
branches.  The  temporal  and  malar  branches  supply  the  oculofacial 
muscles ;  that  is,  the  frontal  portion  of  the  occipitofrontalis,  the  orbicu- 
laris palpebrarum,  and  the  corrugator  supercilii. 

Paralysis  of  the  Seventh  Nerve. — Facial  paralysis  of  interosseous 
or  external  origin  (Bell's  palsy)  is  attended  with  loss  of  faradic  irri- 
tability in  the  affected  muscles  which  occurs  in  the  first  week,  and 


\ 


Fig.  3.— Peripheral  facial  paralysis,  right  side. 
(Case  of  Dr.  Spiller.) 


Fig.  4. — Bilateral  peripheral  facial  pa- 
ralysis ;  attempt  to  close  both  eyes  and 
draw  up  the  corners  of  the  mouth.  (Case 
of  Dr.  Spiller.) 


later  with  wasting  of  the  muscles.  The  loss  of  function  is  shown  by  a 
general  relaxation  of  all  the  muscles  of  one  side  of  the  face,  as  illus- 
trated in  Fig.  3.  The  "  smoothing  out"  of  the  face  produced  by  this 
relaxation  varies  with  the  amount  of  subcutaneous  fat  and  the  habitual 
wrinkling  of  the  skin.  It  is  less  noticeable  in  women  and  children, 
more  pronounced  in  the  aged  and  those  with  deeply  furrowed  faces. 

There  is  also  inability  to  execute  the  usual  movements  of  the  face, 
as  to  elevate  the  brow  or  close  the  eye  (Fig.  4).  It  is  generally 
advised  to  test  the  power  of  closing  the  eyes  by  having  the  patient  attempt 
to  close  each  eye  separately.  But  this  may  prove  misleading,  for  some 
persons  cannot  close  either  eye  separately  and  others  can  close  one  eye 


334 


THE    EYE    AND    NERVOUS    SYSTEM 


alone  but  not  the  other.  The  latter  is  commonly  tlie  case  where  one  eye 
has  distinctly  l)etter  vision  than  its  fellow.  It  is  better  to  have  the  patient 
try  to  close  both  eyes  at  once  or  to  elevate  both  brows  as  nearly  alike  as 
possible.  One  may  also  detect  the  lessened  power  of  the  muscles  by 
oflFering  resistance  to  their  action,  as  by  resisting  closure  of  the  lids. 
This  may  be  made  a  rough  test  of  recovery. 

While  in  paralysis  of  central  origin  the  oculofacial  muscles  are 
usually  but  slightly  involved  or  may  quite  escape,  it  is  possible  to  have 
a  jx)ripheral  j)aralysis  confined  to  these  muscles.  Thus  ^lills  mentions 
a  case  in  which  from  a  stab  in  front  of  the  ear  the  frontalis  was  para- 


FiQ.  5. — Contracture  of  the  orbicularis 
palpebrarum  and  of  other  muscles  of  the 
right  side  of  the  face  in  facial  jwralysis  of 
central  oriprin.    (Case  of  Dr.  Spiller.) 


Fig.  6.— Total  peripheral  facial  paralysis 
of  right  side.  Secondary  contracture.  In- 
voluntary closure  of  eye  and  elevation  of 
brow  in  laughing.    (Wilbrand  and  Saenger.) 


lyzed  and  the  orbicularis  palpebrarum  was  weakened,  alth<jiigh  the  other 
facial  muscles  were  unaffected.  In  another  case,  following  a  fall  on 
the  temple,  the  loss  of  power  was  confined  to  the  frontalis  (page  439). 
Paralysis  of  the  frontalis  destroys  the  power  of  elevating  tlie  eye- 
brow. This  leaves  the  brow  drooping  on  the  affected  side,  narrowing 
somewhat  the  upper  part  of  the  field  of  vision.  Paralysis  of  the  orbicu- 
laris prevents  closure  of  the  lids.  The  consequences  of  exposure  of 
the  eye  will  be  discussed  under  lagophthalmos.  It  also  permits  the  lower 
lid  to  hang  away  from  the  eyeball,  interfering  with  the  lachrymal 
function.  (See  Lachrymation.)  After  three  or  four  months  secondary 
contractures  may  occur  in  the  paralyzed  muscles.  These  are  less  fre- 
quently exhibited  in  the  oculofacial  muscles  than  in  those  acting  on 
the  moutli.  But  they  may  obscure  the  diagnosis,  and  even  give  the 
impression    that   the    paralysis    affects    the    opposite    side    (Fig.    5). 


THE  SEVENTH  NEKVE  335 

The  contracture  of  the  orbicularis  causes  narrowing  of  the  palpebral 
opening  and  wrinkling  of  the  neighboring  skin.  The  contracture  of  the 
frontalis  causes  an  undue  elevation  of  the  eyebrow  of  the  affected  side. 
They  are  increased  during  voluntary  action  of  the  affected  muscles,  as 
in  laughing.     The  effects  of  such  contracture  are  well  shown  in  Eig.  6. 

Etiology. — A  large  proportion  of  ca^s  give  a  clear  history  of  recent 
special  exposure  to  cold,  which  seems  to  act  by  setting  up  a  neuritis 
or  perineuritis.  Less  frequently  the  neuritis  seems  to  be  of  toxic  origin. 
Syphilis,  rheumatism,  diabetes,  gout,  leukemia,  diphtheria,  and  other 
specific  infections  are  credited  with  causing  it.  It  has  also  been 
ascribed  to  an  acute  specific  infectious  process.  A  considerable  num-- 
ber  of  cases  are  secondary  to  aural  disease,  particularly  suppuration 
of  the  middle  ear,  and  local  injury  to  the  nerve  or  its  branches  may 
cause  it.  It  may  occur  as  a  part  of  a  polyneuritis.  Shumway  ^  has 
collected  eight  cases  in  which  it  has  been  accompanied  by  optic  neuritis 
and  subsequent  optic  atrophy.  In  two  cases  he  has  seen  it  followed 
by  marked  flattening  of  the  face  on  the  affected  side  and  ptosis. 

Treatment. — This  must  first  combat  the  cause.  In  neuritis  due  to 
cold  it  is  imjjortant  that  active  treatment  should  begin  promptly.  A 
general  purgative,  leeches  below  the  ear,  salicylates  and  later  iodides 
are  to  be  tried.  Counterirritation  by  a  blister  or  by  milder  irritants 
may  prove  useful.  At  a  later  stage  strychnia  may  be  given.  Elec- 
tricity is  to  be  used  with  caution,  and  its  application  immediately  sus- 
pended if  there  arises  evidence  of  secondary  contracture  (page  885). 

Prognosis. — The  prognosis  depends  upon  the  cause  and,  with  neuritis 
due  to  cold,  somewhat  on  the  promptness  with  which  effective  treatment 
is  begun.  Probably  half  of  all  cases  make  a  fair  recovery,  but  a  con- 
siderable proportion  show  permanent  loss  of  power  in  the  affected  mus- 
cles, and  in  not  a  few  the  paralysis  continues  complete.  The  rapid  and 
complete  loss  of  faradic  irritability  is  unfavorable,  and  complete  re- 
covery cannot  be  expected  where  secondary  contractures  have  occurred. 
Long  duration  of  the  paralysis  is  also  a  ground  for  an  unfavorable 
prognosis,  although  cases  may  recover  after  several  months. 

Facial  Spasm. — This  is  more  frequently  of  cerebral  or  nuclear  than 
of  peripheral  origin.  But  it  may  be  caused  by  a  gross  irritative  lesion 
in  any  part  of  the  nerve-trunk  or  its  branches.  Meige  -  classes  as 
"  spasms"  those  motor  reactions  arising  from  actual  irritation,  and  as 
"  tics"  those  disorders  which  have  a  psychomotor  origin,  and  which  are 


^  Trans.  See.  on  Ophthalmology,  A.  M.  A.,  1904. 
^  Annales  d'Oculistique,  March,  1903. 


336  THE    EYE    AND    NERVOUS    SYSTEM 

therefore  not  discussed  in  this  chapter.  Certain  forms  of  spasm  are 
discussed  in  connection  with  the  palpebral  opening.  We  are  here  con- 
cerned with  spasm  of  the  frontalis,  the  corrugator  supercilii  and  the 
orbicularis  palpebrarum  muscle^,  and  only  the  last,  blepharospasm,  is 
of  much  practical  importance. 

Blepharospasm.  —  Blepharospasm  varies  from  slight  fibrillary 
twitchings  of  the  orbicularis  muscle,  to  painful  contraction  of  the  mus- 
cle, which  resists  strong  mechanical  effort  to  separate  the  lids,  and 
entails  periods  of  practical  blindness.  The  fibrillary  twitchings  may 
be  observed  shortly  after  the  instillation  of  eserine.  Many  persons 
experience  them  after  loss  of  sleep  or  moderate  overexertion  of  the 


Fig.  7.— Case  of  tic  douloureux:  appearance  of  patient's  face  during  severe  paroxysm.    (Mills). 

eyes.  They  are  often  quite  annoying  to  the  patient,  although  scarcely 
perceptible  to  anyone  else.  They  have  no  grave  significance,  and  can 
be  relieved  by  sleep,  removal  of  any  local  irritation  or  of  eye-strain. 
But  they  are  liable  to  recur  in  persons  predisposed  to  them. 

Excessively  frequent,  somewhat  prolonged  acts  of  winking  are  often 
noticed  in  children.  They  call  for  careful  search  for  some  local  irri- 
tation or  eye-strain.  Older  persons,  who  have  usually  suffered  from 
prolonged  conjunctival  irritation,  acquire  a  habit  of  exaggerated  wink- 


THE  SEVENTH  NEKVE  337 

ing,  a  rather  prolonged,  forcible  closure  of  the  lids.  This  habit  be- 
comes a  serious  complication  when  certain  operations,  especially  cataract 
extraction,  are  required. 

More  acute  and  often  uncontrollable  blepharospasm  is  a  symptom 
of  conjunctival,  and  especially  of  corneal,  irritation.  It  is  usually  asso- 
ciated with  photophobia  and  excessive  lachrymation.  This  is  particu- 
larly marked  as  a  symptom  of  phlyctenular  disease.  The  pulling  of  a 
tooth  will  cause  a  sudden  reflex  spasm  of  all  the  muscles  of  that  side 
of  the  face.  Forcible  uncontrollable  spasm  of  the  lids  occurs  in  tic 
douloureux  or  prosopalgia  (Fig.  7).  Hysterical  blepharospasm  is  dis- 
cussed in  the  chapter  on  hysteria  (page  651).  In  a  few  cases  attacks  of 
spasmodic  closure  of  the  eyes  without  discoverable  cause  occur  with  grad- 
ually increasing  frequency  and  prolongation  of  the  attacks.  Mills  sug- 
gests that  these  are  due  to  nuclear  instability, — a  manifestation  of 
chronic  degenerative  changes. 

Diagnosis. — All  forms  of  blepharospasm  tend  to  cause  narrowing 
or  closure  of  the  palpebral  opening,  and  wrinkling  of  the  skin  of  the 
lids  and  adjoining  parts.  Ptosis  causes  narrowing  of  the  palpebral 
opening,  but  is  rather  attended  with  smoothness  of  the  lids  from  re- 
laxation or  distention  of  the  parts.  But,  besides  recognizing  the  con- 
dition present  as  blepharospasm,  the  diagnosis  must  include  the 
determination  of  its  cause,  if  possible.  Careful  search  should  be  made 
for  a  source  of  irritation,  as  inflammation  or  the  presence  of  a  foreign 
body.  Beginning  with  the  cornea  and  conjunctiva  this  search  must 
extend  to  the  regions  supplied  by  other  branches  of  the  fifth  nerve, 
including  investigation  of  possible  disease  of  the  maxillary,  nasal,  and 
other  adjoining  sinuses,  and  of  the  teeth. 

Treatment. — The  treatment  of  blepharospasm  is  first,  and  usually 
wholly,  the  removal  of  its  cause.  This  must  often  include  the  correction 
of  ametropia  or  other  possible  cause  of  eye-strain,  as  well  as  the  re- 
moval of  mechanical  or  inflammatory  irritation.  In  a  few  chronic  cases 
in  which  the  spasm  causes  severe  pain  or  temporary  disability  by  blind- 
ness, it  is  right  to  resort  to  stretching,  division,  or  excision  of  the  nerve- 
branches  concerned.  These  measures  will  give  relief  for  the  time,  some- 
times for  many  months,  but  their  effect  is  not  often  permanent  (p.  894). 

Prognosis. — This  is  favorable  in  proportioiji  as  the  spasm  is  due  to 
an  evident  and  remediable  irritation.  Cases  not  so  caused,  and  espe- 
cially those  due  to  chronic  degenerative  changes,  are  well-nigh  hopeless. 
Certain  tics  of  central  or  psychomotor  origin  have  a  better  prognosis, 
being  amenable  to  educative  treatment. 

Spasmodic  Entropion. — The  tendency  to  exaggerated  winking  pro- 

22 


338  THE    EYE    AND    NERVOUS    SYSTEM 

duced  by  any  irritation  in  the  conjunctival  sac,  with  the  chronic  inflam- 
matory changes  that  produce  it,  are  the  causes  of  spasmodic  entropion. 
Entropion  caused  by  relaxation  or  swelling  of  the  skin  of  tlie  lids  is 
often  classed  with  this.  But  the  true  spasmodic  form  depends  on  the 
action  of  the  orbicularis.  When  this  muscle  is  strongly  contracted,  the 
palpebral  opening  is  narrowed,  and  the  skin  around  is  dragged  in 
towards  it.  When  this  dragging  is  kept  up,  or  repeated  at  short  inter- 
vals over  a  considerable  length  of  time,  the  skin  at  the  lid  margin 
becomes  more  or  less  pushed  over  tlie  edge  of  the  cartilage  toward  the 
inner  side  of  the  lid,  carrying  with  it  the  lashes,  which  then  rub  against 
the  eyeball,  and  still  further  increase  the  irritation  and  tendency  to 
spasmodic  contraction  of  the  orbicularis.  Entropion  most  frequently 
occurs  in  the  course  of  trachoma,  in  which  there  are,  with  conjunctival 
irritation,  cicatricial  changes  in  the  lids  leading  to  incurving  of  the 
tarsus  and  organic  entropion.  In  these  cases  it  is  hard  to  separate  the 
spasmodic  from  the  cicatricial  causes.  But  the  orbicularis  spasm  may 
be  an  important  factor  in  the  development  of  organic  entropion. 

Treatment. — This  must  include,  first,  the  removal  of  the  irritation 
which  is  provoking  the  orbicularis  to  spasmodic  contraction,  as  the 
treatment  of  the  trachoma.  In  some  cases  the  habit  of  spasmodic 
closure  of  the  lids  is  itself  an  important  cause  of  irritation,  and  can- 
thotomy  or  canthoplasty,  preventing  the  excessive  pressure  of  the  lids 
upon  the  eyeball,  will  be  followed  by  great  improvement.  In  severe 
chronic  cases,  however,  an  entropion  operation  is  indicated,  especially 
that  of  Hotz,  which  fastens  the  skin  of  the  lid  securely  to  the  upper 
margin  of  the  tarsus. 

THE   SYMPATHETIC    NERVE. 

Anatomy. — The  sympathetic  nerve  supply  of  the  eye  and  its  ap- 
pendages comes  through  the  superior  cervical  ganglion  of  the  sympa- 
thetic, which  Langley  calls  "  a  relay  station  for  the  sympathetic  nerve 
supply  for  the  whole  of  the  head."  From  this  ganglion  branches  form 
plexuses  which  accompany  all  the  arteries  distributed  to  the  orbit  and 
surrounding  regions,  and  other  branches,  mostly  from  the  larger  plex- 
uses, join  the  cranial  nerves.  The  third  receives  such  a  branch  at  its 
point  of  division ;  the  fourth  as  it  lies  in  the  wall  of  the  cavernous 
sinus ;  the  fifth  in  the  Gasserian  ganglion ;  the  sixth  where  it  lies  in 
close  apposition  with  the  carotid  plexus;  the  seventh  at  the  stylo- 
mastoid foramen. 

In  the  orbit  is  situated  the  ciliary  ganglion.     This  is  about  2  milli- 


THE   SYMPATHETIC   NERVE  339 

metres  long  and  1  millimetre  thick.  It  lies  slightly  to  the  temporal 
side,  and  slightly  lower  than  the  optic  nerve,  8  to  11  millimetres  from 
the  optic  foramen,  and  14  to  17  millimetres  from  the  entrance  of  the 
optic  nerve  through  the  sclera.  Its  roots  are  derived  from  the  fifth 
nerve,  usually  from  a  branch  of  the  nasal  just  after  entering  the 
orbit,  a  filament  of  the  carotid  plexus,  and  a  filament  from  the  oculo- 
motor, usually  from  the  branch  supplying  the  inferior  oblique.  It 
gives  off  the  short  ciliary  nerves,  which  enter  the  posterior  part  of  the 
sclera,  and  run  forward  between  it  and  the  chorioid  to  the  ciliary 
muscle  and  iris.  We  thus  have  the  sympathetic  reaching  all  parts  of 
the  eye  and  its  appendages,  and  a  special  mechanism  supplying  the 
iris  and  ciliary  body,  the  function  of  which  is  discussed  in  Chapter  VII. 
on  "  The  Intra-ocular  Muscles." 

Vasomotor  Disturbances. — The  most  general  and  best  understood 
function  of  the  sympathetic  nerve  is  its  vasomotor  function.  Uni- 
lateral flushing  of  the  face  and  neck  occurs,  and  may  be  attended  with 
noticeable  hyperaemia  of  the  conjunctiva  and  narrowing  of  the  pal- 
pebral opening.  Such  an  attack  may  last  but  a  few  minutes  or  it  may 
continue  for  hours.  A  more  permanent  impairment  of  this  function 
may  occur.  Following  influenza,  the  writer  has  seen  hyperaemia  of 
one  eye,  especially  of  the  conjunctiva,  with  narrowing  of  the  palpebral 
opening  and  slight  tonic  ptosis.  Cases  of  this  kind  have  not  received 
the  attention  they  deserve.  It  is  very  easy  to  confuse  such  a  condition 
with  unilateral  inflammation  of  the  conjunctiva  and  partial  closure  of 
the  lids  on  account  of  irritation.  But  instead  of  active  local  treatment 
these  are  to  be  benefited  chiefly  by  improving  the  general  condition. 
Pulsating  exophthalmos  not  due  to  gross  lesions  of  the  orbital  vessels 
may  be  regarded  as  a  vasomotor  disorder. 

The  connection  of  the  general  blood-pressure  with  the  intra-ocular 
tension  has  been  the  subject  of  various  theoretic  discussions  and  physio- 
logic experiments.  The  conclusions  drawn  from  the  latest  of  these  by 
Parsons  and  Snowball  ^  are  that  the  intra-ocular  tension  responds  pas- 
sively to  all  variations  in  the  general  blood-pressure,  whether  these  be 
produced'  by  vasomotor  actions  or  by  such  mechanical  means  as  the  tying 
of  a  large  artery  or  by  the  inhibition  of  the  heart's  action.  The  in- 
crease of  intra-ocular  tension  caused  by  stimulation  of  the  peripheral 
portion  of  the  cervical  sympathetic  they  ascribe,  however,  not  to  vaso- 
motor action  but  to  contraction  of  the  unstriped  muscles  of  the  orbit. 

Influence  of  Unstriated  Muscles.— Stimulation  of  the  sympathetic 


'  Royal  London  Ophthal.  Hosp.  Reports,  vol.  xv.  Part  3,  p.  275. 


340  THE    EYE    AND    NERVOUS    SYSTEM 

produces  contraction  of  the  unstriated  muscle-cells  with  which  it  is 
connected.  The  intra-ocular  muscles  and  their  action  are  discussed  in 
the  preceding  chapter.  The  principal  extra-ocular  muscles  of  this  char- 
acter are  those  acting  upon  the  upper  and  lower  lids;  and  the  fibres 
stretched  across  the  sphenoidal  and  sphenomaxillary  fissures.  These 
latter  are  better  developed  in  some  of  the  lower  animals;  and  it  is 
doubtful  if  they  have  much  practical  importance  in  the  human  orbit. 

Contraction  of  Mueller's  muscles  connected  with  the  lids  tends  to 
cause  retraction  of  the  lids.  Their  relaxation  produces  narrowing  of 
the  palpebral  opening.  Contraction  of  fibres  stretching  across  the  back 
of  the  orbit  has  been  suggested  as  the  explanation  of  the  protrusion 
of  the  eyes  produced  by  stimulation  of  the  sympathetic,  and  relaxation 
of  these  fibres  has  been  supposed  to  account  for  exophthalmos.  But  it 
is  doubtful  whether  the  protrusion  and  sinking,  produced  by  stimula- 
tion or  paralysis  of  the  sympathetic  in  man,  are  not  merely  apparent 
rather  than  real,  the  appearance  being  produced  by  the  widening  or 
narrowing  of  the  palpebral  opening.  The  detailed  effects  produced 
by  these  muscles  are  discussed  in  connection  with  alterations  of  the 
lids  and  the  palpebral  opening. 

Secretion. — One-sided  sweating  with  flushing  of  the  face  occurs 
from  defective  sympathetic  innervation.  In  these  cases  the  eyelids 
show  slight  increase  of  secretion.  The  influence  of  the  sympathetic 
upon  the  lachrymal  secretion  is  discussed  under  that  heading.  What 
share  its  influence  on  secretion  has  in  affecting  the  intra-ocular  tension 
will  be  considered  under  the  next  heading. 

Intra-ocular  Tension. — Softening  of  the  eye-ball  after  section  of 
the  cervical  sympathetic  was  one  of  the  earliest  observations  regarding 
the  physiology  of  this  nerve.  Increase  of  intra-ocular  tension  by  stimu- 
lation of  the  peripheral  portion  of  the  nerve  is  another  common  obser- 
vation. How  these  changes  of  intra-ocular  tension  are  produced  is  still 
in  dispute.  Hippel  and  Gruenhagen  ascribed  them  to  influences  pro- 
duced upon  the  orbital  muscles  and  the  vessels  of  the  eye.  Adamtik 
attributed  them  to  changes  in  the  intra-ocular  muscles,  especially  to  the 
unstriped  fibres  of  the  choroid.  Homer  and  Nicati  concluded  that  the 
change  was  independent  of  the  ocular  vessels.  Abadie  suggested  that 
excitation  of  vasodilator  fibres  was  the  cause  of  increased  tension. 
Lodato  causing,  by  insertion  of  a  foreign  body,  irritation  of  the  superior 
cervical  ganglion,  produced  elevated  intra-ocular  tension  lasting  for  some 
months.  But  Weeks,  among  reported  cases  of  irritation  of  the  cervical 
sympathetic  in  man,  found  no  mention  of  glaucoma  occurring  as  a 
complication.    Selenkowski  and  Rosenberg,  Ilertel  and  Levinsohn  found 


THE  SYMPATHETIC  NERVE  341 

the  changes  of  tension  produced  bj  disturbance  of  the  cervical  sympa- 
thetic were  but  brief.  De  Schweinitz  ^  concludes  that  the  fall  of  the  intra 
ocular  tension  produced  by  section  of  the  sympathetic  or  extirpation  of 
the  sympathetic  ganglion  probably  depends  on  vascular  or  perhaps  mus- 
cular changes. 

It  is  quite  possible  that  the  sjTnpathetic  exerts,  apart  from  its  vaso- 
motor function,  a  direct  control  over  secretion  within  the  eye.  But 
no  one  has  yet  been  ingenious  enough  to  devise  appropriate  experiments 
or  to  so  marshal  clinical  facts  as  to  clearly  demonstrate  this 
condition. 

Within  the  last  ten  years  excision  of  the  superior  cervical  ganglion 
of  the  sympathetic  has  been  done  many  times  for  exophthalmic  goitre, 
epilepsy,  and  glaucoma.  From  the  clinical  observations  on  these  cases 
and  those  in  which  the  sympathetic  has  been  paralyzed,  as  by  pressure 
from  enlarged  cervical  glands,  it  seems  clear  that  loss  of  function  of 
the  cortical  sympathetic  is  followed  by  diminished  intra-ocular  tension. 
In  healthy  eyes  this  diminution  seems  to  be  brief  and  unimportant. 
In  glaucomatous  eyes  it  may  be  more  prolonged. 

Wilder  ^  concludes  that  "  sympathectomy  for  glaucoma  is  not  an 
operation  to  be  condemned  too  hastily.  .  .  .  The  statistics  up  to  date 
seem  to  indicate  that  the  simple  chronic  form  is  the  one  most  suited 
for  it,  next  to  the  hemorrhagic  form,  if  that  can  be  determined." 
WTiat  is  yet  known  with  reference  to  the  influence  of  the  sympathetic 
upon  intra-ocular  tension  does  not  justify  neglect  of  older  established 
methods  of  controlling  this  condition.  But  it  does  justify  resort  to 
sympathectomy  as  an  adjunct  to  other  treatment  or  when  such  treat- 
ment has  proven  insufficient. 

Excision  of  the  ciliary  ganglion  practised  by  Rohmer  ^  is  a  difficult 
operation  on  account  of  the  smallness  and  position  of  the  ganglion; 
and  it  has  not  proven  so  efficient  as  excision  of  the  superior  cervical 
ganglion  for  the  reduction  of  intra-ocular  tension.  It  should  be  con- 
sidered not  as  an  alternative  of  iridectomy  or  even  of  sympathectomy, 
but  rather  in  comparison  with  opticociliary  neurotomy  or  excision  of 
the  eyeball. 

Pathology. — While  the  effects  of  increased  or  diminished  func- 
tional activity  in  the  sympathetic  nerve  are  well  understood,  the  re- 
corded observations  as  to  its  pathological  histology  are  so  inconclusive 
as  to  furnish  no  basis  for  a  classification  of  its  diseases.     Indeed,  they 

'Trans.  Sec.  on  Ophthalmology,  A.  M.  A.,  1903. 

^  Ibid. 

^  Annales  d'Oculistique,  July,  1902. 


342  THE    EYE    AND    NERVOUS    SYSTEM 

here  require  but  little  notice.  Weeks  *  finds  regarding  glaucoma : 
"  The  testimony  in  our  possession  is  not  sufficiently  conclusive  to  enable 
us  to  say  that  there  is  any  constant  change  in  the  cervical  sympathetic 
peculiar  to  glaucoma.  Xor  is  it  sufficiently  conclusive  to  exclude  the 
possibility  of  such  constant  change."  The  most  constant  change  found 
in  the  ganglia  removed  has  been  an  increase  of  pigment  in  the  neuron 
cell-bodies.  But  this  may  be  a  senile  change.  Increased  connective 
tissue,  or  sclerosis,  which  has  been  reported  by  several  observers,  has 
not  been  found  at  all  by  others  equally  competent. 

THE    SECRETION    AND    REMOVAL    OF   TEARS. 

Secretion. — The  control  of  nerve  impulses,  either  of  reflex  or  of 
central  psychic  origin,  over  the  secretion  of  tears  is  one  of  the  com- 
monest observations  in  ocular  physiolog}'.  It  is  also  certain  that  reflex 
lachrymation  is  especially  likely  to  arise  from  impressions  made  through 
the  fifth  nerve,  as  by  the  irritation  of  wind  or  dust  in  the  eye,  pungent 
gases,  etc.  But  the  exact  channels  by  which  the  efferent  nerve  impulses 
governing  its  action  reach  the  lachrymal  gland  have  not  been  determined. 

While  fibres  of  the  sympathetic  nerve  enter  the  gland  along  each 
of  its  nutrient  vessels,  its  most  evident  nerve-supply  is  through  branches 
of  the  fifth  nerve.  Chiefly  it  is  supplied  through  the  lachrymal  branch 
of  the  ophthalmic  division,  which  runs  forward  near  the  roof  of  the 
orbit  to  be  distributed  to  the  gland.  But  anastomosing  with  this  is  a 
twig  coming  irom  the  orbital  branch  of  the  second  division  of  the  tri- 
geminus ;  and  this  anastomotic  twig  may  have  special  importance  with 
reference  to  the  lachrymal  secretion.  A  large  part  of  the  lachrymal 
branch  of  the  first  division  passes  through  the  gland  to  be  distributed 
to  the  conjunctiva  and  skin  adjoining.      (See  Fig.  8.) 

The  results  of  physiologic  experiments  are  so  contradictory,  pos- 
sibly on  account  of  the  variations  of  function  of  the  different  animals 
experimented  upon,  that  they  throw  no  certain  light  upon  the  neurology 
of  the  lachrymal  secretion.  They  need  not  be  here  discussed,  except 
to  point  out  that  increased  secretion  of  tears  after  section  of  the  sym- 
pathetic or  after  removal  of  the  superior  cervical  ganglion  is  usually 
quite  temporary,  and  probably  depends  upon  vasomotor  paralysis. 

When  it  comes  to  actual  disease  of  the  nerves,  lesions  of  the  fifth 
nerve  seem  less  frequently  to  cause  disturbance  of  lachrymation  than 
those  of  the  seventh.  In  trigeminal  neuralgia  there  is  no  constant  or 
characteristic  alteration  of  the  lachrymal  secretion.     Nor  is  any  lach- 


*  Trans.  Sec.  on  Ophthalmology,  A.  M.  A.,  1903. 


SECRETION  AXD  REMOVAL  OF  TEARS 


343 


rymal  disturbance  more  certain  to  occur,  with  neuralgia  involving  the 
first  division  of  the  nerve,  than  when  the  second  or  third  division  is 
affected,  without  the  first.  The  intense  pain  may  cause  the  increased 
lachrymation,  very  much  as  pain  elsewhere,  or  irritation  of  the  eyes 
or  nasal  mucous  membrane  may  cause  it.  A  more  specific  disturbance 
of  the  lachrymal  function  has,  however,  been  noticed  by  Uhthoff  in 
one  case  of  peripheral  neuritis  affecting  the  first  and  second  divisions 
of  the  fifth  nerve. 

Paralysis  of  the  seventh  nerve,  by  relaxation  of  the  orbicularis, 
allowing  the  lid  to  fall  away  from  the  eye-ball,  commonly  disturbs 


Fig.  8.— Nerve  supply  of  lachrymal  gland.  1,  lachrymal  branch;  2,  lachrymal  gland;  3,  malar 
branch ;  4,  second  division  of  fifth  nerve ;  5,  anastomotic  branch  connecting  gland  with  second  division. 
(Merkel.) 

the  removal  of  tears.  But  in  a  considerable  number  of  reported  cases, 
facial  paralysis  has  clearly  been  attended  with  diminution,  if  not  com- 
plete cessation,  of  the  secretion  of  tears  on  the  affected  side.  Probably 
the  symptom  would  be  more  frequently  noticed  if  the  diminished  re- 
moval of  tears  did  not  mask  the  diminished  secretion.  The  interference 
with  secretion,  and  the  interference  with  removal,  t)  some  extent  neu- 
tralize each  other ;  and  render  the  lachrymal  disturbances  attending  the 
seventh  nerve  lesions  less  noticeable  than  they  otherwise  would  be. 

While  the  neurology  of  lachrymation  cannot  be  regarded  as  settled, 
the   share  of  the  seventh  nerve  in  the  control  of  the  gland  might  be 


344  THE    EYE    AND    NERVOUS    SYSTEM 

explained  in  this  way:  As  has  already  been  pointed  out  (page  333), 
the  seventh  nerve  gives  off  at  the  geniculate  ganglion  the  petrosal 
branches ;  th^  superior,  or  greater,  petrosal  passes  to  the  sphenopalatine 
ganglion,  which  gives  branches  to  the  second  division  of  the  trigeminus ; 
and  this,  as  we  have  seen,  supplies  an  anastomotic  branch  to  the  lach- 
rymal nerve.  Through  tliis  channel  efferent  impulses,  emerging  from 
the  nerve-centres  by  way  of  the  seventh  nerve,  may  reach  the  lachrymal 
gland.  The  similarity  of  the  arrangement  with  that  of  the  chorda 
tympani,  given  off  from  the  seventh  more  peripherally  than  the  great 
petrosal,  and  joining  the  third  division  of  the  trigeminus,  is  quite 
striking.  That  the  chorda  tympani  exerts  an  important  influence  over 
the  secretion  of  the  salivary  glands  is  well  known,  and  this  view  of 
the  nerve-control  of  the  lachrymal  secretion  adds  one  to  the  striking 
analogies  that  may  be  traced  between  the  functions  of  salivation  and 
lachrymation.  It  should  not  be  forgotten,  however,  that  definite  proof 
regarding  the  innervation  of  the  lachrymal  gland  is  yet  lacking. 

The  amount  of  lachrymal  secretion  formed  in  a  given  time  depends 
almost  wholly  upon  reflex  influences.  Thus,  Schirmer  points  out  that 
the  secretion  practically  ceases  during  sleep.  Under  psychic  influences 
or  peripheral  irritation,  it  is  capable  of  enormous  increase.  Even  in 
those  persons  in  whom  epiphora  is  most  constant  it  occurs  in  response 
to  some  especial  stimulation,  as  from  chronic  inflammation  of  the  lach- 
rymal sac,  eye-strain,  or  the  ordinary  exposures,  which  in  the  particular 
patient  cause  an  excessive  reflex.  It  is  a  significant  fact  that  after 
removal  of  the  lachrymal  sac  for  dacryocystitis,  the  patients  usually 
have  no  further  trouble  from  epiphora,  showing  that  it  has  been  a  reflex 
of  the  irritation  of  the  diseased  tear  passages. 

Increased  or  diminished  lachrymation  may  occur  in  organic  disease 
of  the  central  nervous  system,  as  locomotor  ataxia.  In  exophthalmic 
goitre  increased  lachrymation  has  usually  been  ascribed  to  exposure  of 
the  conjunctiva  by  retraction  of  the  lids  and  defective  winking.  But 
Berger  has  pointed  out  that  it  may  antedate  the  symptoms  of  Dal- 
rymple  and  Stellwag  (See  Pages  668  and  840). 

Increased  or  diminished  lachrymation  may  occur  in  organic  disease 
Schirmer,  from  his  very  careful  study  of  the  subject,  concludes  that 
in  the  act  of  winking,  tension  upon  the  palpebral  tendon  causes  a  dila- 
tation of  the  lachrymal  sac,  and  the  sucking  into  it  of  tears  from  the 
conjunctiva.  Wlien  the  eye  is  reopened  the  elasticity  of  tlie  sac  forces 
the  tears  on  into  the  nose.  Probably  an  important  part  in  the  process 
is  taken  by  the  minute  sphincter  muscles  which  surround  each  lachry- 
mal punctum.     Relaxation  of  these  sphincters  during  winking  favors 


THE  LIDS  AND  THEIE  MOVEMENTS  345 

the  passage  of  the  tears  into  the  canaliculi ;    and  their  contraction  at 
other  times  tends  to  prevent  regurgitation. 

The  abnormal,  continuous  relaxation  of  this  little  sphincter  muscle, 
either  by  chronic  disease  or  forcible  dilatation,  impairs  the  natural  ability 
to  remove  any  excess  of  lachrymal  secretion.  In  order  that  the  tears 
shall  enter  the  canaliculi  the  puncta  must  be  turned  in  towards  the 
eyeball,  and  the  lids  be  in'  contact  with  the  globe  of  the  eye,  so  that 
the  tears  shall  be  conducted  by  capillary  attraction  to  the  puncta. 
Paralysis  of  the  orbicularis  causes  interference  with  the  passage  of  the 
tears  by  the  inability  to  maintain  these  latter  conditions. 

THE    LIDS    AND    THEIR    MOVEMENTS. 

The  position  and  form  of  the  lids  and  palpebral  opening  respond 
so  clearly  to  nerve  impulses  and  to  such  varied  impulses  that  they  afford 
diagnostic  evidence  of  great  value.  But  they  are  subject  to  marked 
normal  and  pathologic  variations,  due  to  alterations  in  the  tissues  of 


Fig.  9.— Thickening  of  the  lids  produced  by  trachoma.    (Case  of  Dr.  Jackson.) 

the  lids  themselves  or  of  the  structures  that  they  cover,  and  these 
variations  must  be  thoroughly  understood  before  the  symptomatic  value 
of  the  appearances  presented  can  be  estimated. 

The  lids  form  a  freely  movable  curtain  stretched  across  the  orbital 
opening,  loosely  attached  in  all  directions  except  to  the  nasal  side  in 
the  region  of  the  internal  palpebral  ligament.  They  are  usually  com- 
paratively thin  and  flaccid  except  for  the  normal  elasticity  of  the  skin 
and  other  tissues  composing  them.  They  lie  upon  the  deeper  tissues 
from  which  they  largely  take  their  outline.     But  the  lids  may  become 


34C  THE    EYE    AND    NERVOUS    SYSTEM 

thickened  so  that  the  outline  of  the  deeper  structures  is  quite  masked. 
Normally  comparatively  free  from  fat  in  the  adult,  they  may  become 
the  seat  of  extensive  deposits  of  adipose  tissue,  and  usually  do  contain 
a  good  deal  of  it  in  early  childhood.  Their  loose  cellular  tissue  favors 
their  rapid  and  excessive  infiltration  with  serum,  and  they  may  become 
greatly  thickened  with  more  substantial  and  permanent  exudate. 

Still  more  confusing  are  the  changes  produced  by  inflammation. 
These  are  quite  common  as  a  result  of  trachoma.  In  the  chronic  cases 
the  deposits  of  connective  tissue,  besides  thickening,  bind  the  lid  rigidly 
to  adjoining  tissues,  greatly  interfering  with  all  of  its  movements. 
The  effect  produced  is  illustrated  in  Fig.  9. 

In  this  patient  the  alterations  produced  by  chronic  trachoma  were 
chiefly  confined  to  the  right  eye,  so  that  the  left  serves  as  a  standard 
of  comparison.  Syphilitic  tarsitis  causes  a  somewhat,  but  not  exactly, 
similar  alteration  of  appearance  and  function.     It  is  generally  bilateral. 

In  the  curtain  of  the  lids  the  features  especially  influenced  by 
nerve  impulses  are  the  palpebral  opening  and  the  superior  and  inferior 
orbitopalpebral  folds.     Of  these  folds  the  lower,  between  the  lower  lid 


Fio.  10.— Palpebral  folds,    n,  brow:  b,  oculo-orbital  fold,  superior;  c,  d,  /  inferior  folds ;  e,  cheek. 

(Wilbrand  and  Saenger.) 

and  margin  of  the  orbit,  is  most  marked  when  the  eye  is  turned  strongly 
down.  But  even  with  that  movement  it  may  be  quite  indistinguishable, 
esf)ecially  in  children  with  nuich  fat  in  the  lids.  On  looking  up  there 
remain  only  the  lines  or  creases,  usually  two,  which  mark  the  site  of 
the  fold.  Its  formation  evidently  depends  upon  traction  of  the  inferior 
rectus,  through  its  connections  with  the  lid  (Fig.  11).  These  folds  are 
shown  in  Fig.  10. 

The  upper  fold   h  is  well  marked,  although   it  varies  greatly  in 
appearance  and  l)eeomes  much  deeper  when  the  eye  is  rolled  upward. 


THE  LIDS  AXD  THEIR  MOVEMEIs^TS 


347 


When  the  orbit  is  well  filled  with  fat,  it  appears  merely  as  a  close  fold 
in  the  skin.  But  when  the  adipose  tissue  is  removed  by  age  or  wasting 
disease  it  becomes  a  broad  fossa,  which  is  the  chief  factor  in  producing 
the  appearance  of  hollow  or  sunken  eyes,  in  wasting  disease.  Below 
there  are  generally  several  smaller  creases,  c,  d,  f,  divided  into  a  tem- 
poral and  a  nasal  group,  which  become  emphasized  as  the  eye  is  turned 
down  and  from  or  toward  the  nose. 

For  a  clear  understanding  of  alterations  brought  about  in  the  lids 
and  palpebral  opening,  one  must  bear  in  mind  the  anatomic  and  physio- 


66        6c 


Fig.  11.— Section  of  orbit.  1,  skin  of  lid ;  2,  orbicularis  muscle ;  3,  tarso-orbital  fascia ;  4,  con- 
junctival cul  de  sac ;  5,  tarsus ;  6,  elevator  of  upper  lid ;  6a,  unstraited  muscle  connecting  elevator 
with  skin  of  lid ;  6b  and  6c,  connections  of  elevator  with  tarsus  and  conjunctiva ;  7,  skin  of  lower 
lid ;  8,  inferior  rectus ;  8a,  connection  of  lower  lid.    (Schwalbe). 


logic  relations  of  the  various  structures  of  the  orbit.  To  the  margin 
of  the  orbit  is  attached  a  sort  of  connective  tissue  hammock,  including 
part  of  the  capsule  of  Tenon,  denser  connective  tissue  bands,  and  looser 
meshes.  In  this  hammock  lies  the  eyeball,  drawn  firmly  back  by  the 
elastic  tension  of  the  recti  muscles,  and  rotated  or  swung  by  their 
individual  or  combined  contractions.  The  globe  of  the  eye  is  not  a 
ball  turning  in  a  fixed  socket.     The  well-known  fact  that  the  centre 


348  THE    EYE    AND    NERVOUS    SYSTEM 

of  rotation  is  back  of  the  centre  of  the  eyeball  means  that  the  eyeball 
as  a  whole  moves  somewhat  in  the  direction  toward  which  it  is  rotated. 
On  looking  up  it  rises;  on  looking  dow-n  it  is  depressed;  on  looking 
to  the  right  or  left  it  moves  perceptibly  in  these  directions.  All  such 
movements  of  the  eyeball  are  closely  followed  by  movements  of  the  lids. 
The  parts  concerned  are  illustrated  in  Fig.  11. 

The  harmony  of  movement  of  eyeball  and  lids  is  aided  by  the  numer- 
ous bands  that  pass  from  the  tendons  of  the  recti  and  inferior  oblique 
muscles  to  the  lids;  so  that  the  lid  movements  are  partly  due  to  the 
action  of  the  muscles  which  turn  the  eyeball. 

A  good  example  of  how  such  harmony  of  movement  is  brought  about 
is  revealed  by  a  careful  study  of  the  connections  of  the  elevator  of 
the  upper  lid  and  the  superior  rectus  muscles.  Besides  a  close  anatomic 
association  of  the  nuclei  governing  these  two  muscles  and  their  habitual 
physiologic  association  of  simultaneous  use,  slips  from  the  tendon  of 
insertion  of  each  pass  to  the  tendon  of  insertion  of  the  other. 

Harmony  of  movement  even  of  different  parts  of  the  upper  lid  is 
secured  by  such  an  arrangement  of  muscular  insertions.  The  part  of 
the  skin  covering  the  cartilage  of  the  upper  lid  is  normally  kept  rather 
closely  applied  to  it,  although  the  skin  and  cartilage  are  not  firmly 
connected  except  at  the  lid  margin.  This  close  apposition  of  the  skin 
to  the  cartilage  is  preserved,  whatever  the  position  of  the  lid  and  how- 
ever great  the  general  redundancy  of  the  skin.  This  has  been  ascribed 
to  the  elasticity  of  the  tissue  connecting  the  skin  with  the  tarsus.  But 
it  is  known  that  certain  anterior  fibres  of  Mueller's  muscle  in  the  upper 
lid  pass  through  the  orbicularis  to  be  inserted  in  the  skin  of  the  lid. 
Dwight  ^  points  out  that  their  function  is  to  draw  the  skin  to  the  fold 
above  the  tarsus  w^hen  the  lids  are  opened.  Failure  of  this  function 
causes  atonic  ptosis. 

The  Palpebral  Opening. — The  palpebral  opening  varies  in  size 
and  shape  from  a  mere  slit  separating  the  closed  lids  to  an  almost 
circular  aperture,  the  diameter  of  which  approaches  that  of  the  eye- 
ball. The  active  factors  in  producing  these  changes  are  the  constriction 
of  the  orbicularis  tending  to  diminish  the  opening,  and  the  contractions 
of  the  elevator  of  the  upper  lid  and  the  superior  and  inferior  recti 
muscles,  with  the  attached  unstriated  muscles,  which  tend  to  retract 
the  lids  and  so  enlarge  the  opening.  Passively,  narrowing  of  the  open- 
ing may  be  due  to  inflammatory  or  other  deposits  which  tend  to  oppose 
the  retractors  of  the  lids  or  to  weakness  or  paralysis  of  these  retractors. 


'  System  of  Ophthalmology,  edited  by  Norris  and  Oliver,  vol.  i.  p.  92. 


THE  LIDS  AND  THEIR  MOVEMENTS  349 

Widening  of  the  palpebral  aperture  is  passively  favored  by  paralysis 
of  the  orbicularis,  or  by  enlargement  of  the  eyeball,  or  its  being  pushed 
forward  by  pressure  from  behind,  or  by  cicatricial  adhesions  preventing 
the  normal  closure  of  the  lids. 

The  nerves  directly  concerned  in  the  changes  of  form  of  the  opening 
are  the  facial,  distributed  to  the  orbicularis ;  the  oculomotor,  supplying 
the  recti  and  the  elevator  of  the  upper  lid ;  and  the  sympathetic,  which 
is  supposed  to  innervate  the  unstriated  fibres  of  Mueller's  muscles  of 
the  lids.  The  palpebral  opening  is  generally  more  nearly  circular  and 
relatively  wider  in  childhood.  Excitement  and  probably  the  influence 
of  general  cold  tend  to  widen  the  aperture.  Stimulation  or  irritation 
of  the  cervical  sympathetic  and  the  local  application  of  cocaine  have 
the  same  influence.  On  the  other  hand,  age,  weariness,  stupor,  paraly- 
sis of  the  sympathetic  or  its  division  or  excision,  tend  to  diminish  the 
palpebral  opening. 

Exophthalmos  widens  and  enophthalmos  causes  narrowing  of  the 
aperture.  But  retraction  of  the  lids  and  widening  of  the  palpebral 
opening  causes  an  appearance  of  exophthalmos,  while  narrowing  of 
the  opening  causes  an  appearance  of  enophthalmos.  Unfortunately, 
the  prominence  of  the  eye-ball  in  the  orbit  and  the  actual  variations 
in  its  position  with  reference  to  the  plane  of  the  orbital  opening  have 
rarely  been  subjected  to  actual  measurement;  so  that  there  may  often 
have  been  retraction  of  the  lids  causing  an  appearance  of  exophthalmos, 
when  it  was  supposed  that  widening  of  the  palpebral  opening  had  been 
caused  by  exophthalmos.  Certainly,  eyes  that  appear  to  be  protruded 
or  retracted  often  prove  not  to  have  changed  their  relations  with  the 
orbital  margin. 

On  the  other  hand,  actual  protrusion  of  the  eyeball  may  be  accom- 
panied by  swelling  or  relaxation  of  the  lids  leading  to  narrowing  of 
the  lid  opening,  which  partly  masks  the  exophthalmos.  In  cases  of 
exophthalmos  slowly  developed  by  the  increase  of  an  orbital  tumor 
the  lids  may  be  enormously  enlarged. 

Winking. — Normal  winking  is  a  rhythmical  closure  of  the  palpe- 
bral opening  by  the  contraction  of  the  tarsal  portion  of  the  orbicularis, 
as  a  reflex  probably  to  a  need  for  renewed  moisture  of  the  corneal  sur- 
face, a  sort  of  thirst  of  the  part,  which  usually  does  not  affect  conscious- 
ness. The  afferent  impulse  passes  through  the  fifth,  the  efferent  through 
the  seventh  nerve.  Winking  is  repeated  from  two  to  five  times  a  min- 
ute.    Howe,^   who  studied  it  by  photography,  finds  that  the  closing 


^  Transactions  of  the  American  Ophthal.  Society,  1904. 


350  THE    EYE    AND    NERVOUS    SYSTEM 

of  the  lids  occupies  one-twentieth  to  one-tenth  of  a  second.  They 
remain  closed  two-  or  three-tenths  of  a  second,  and  the  lifting  of  the 
lid  again  takes  one-  or  two-tenths  of  a  second.  Slow  lifting,  he  thinks, 
may  be  a  valuable  sign  of  paresis  of  the  elevator  of  the  lid. 

Lid  Signs. — Altliough  the  normal  variations  in  the  palpebral  open- 
ing make  it  difficult  to  judge  at  first  sight  whether  or  not  the  lids  are 
abnormally  separated,  any  change  of  the  kind  is  likely  to  be  noticed, 
in  the  expression  of  the  eyes,  by  those  familiar  with  the  patient.  When 
the  lids  are  separated  too  widely  it  gives  the  eye  a  staring  expression. 
When  this  is  habitually  present  the  retraction  is  probably  due  to  spasm 
of  the  unstriated  muscle-fibres  of  the  lids  innervated  through  the  sym- 


Fio.  12.— <"iraefe'8  sign :  exposure  of  sclera  on  looking  down.    From  a  case  of  exopbthalmio  goitre. 

(Wilbrand  and  Saenger.) 

pathetic.  This  has  been  called  Dalrymple's  sign.  It  may  cause  ex- 
I)osure  of  the  sclera  both  above  and  below  the  cornea.  It  can  be  imi- 
tated by  the  application  to  the  eye  of  cocaine,  which  produces  a  similar 
spasm  of  Mueller's  muscle  that  lasts  considerably  longer  than  the  ansBS- 
thesia,  but  hardly  so  long  as  the  dilatation  of  the  pupil  produced  by 
this  drug.      (Compare  Page  835.) 

The  normal  movements  of  the  lids  with  the  eyeball  have  already 
been  referred  to.  The  movements  of  the  upper  lid,  upward  or  down- 
ward as  the  eye  is  turned  up  or  down,  is  by  far  the  most  extensive  of 
these  lid  movements,  and  in  health  is  very  accurately  co-ordinated  with 
the  movements  of  the  globe.  In  disease  the  downward  movement  of 
the  lid,  when  the  eye  is  turned  down,  may  become  very  defective  or 
altogether  wanting.     The  upper  lid  remaining  elevated  and  retracted 


THE  LIDS  Al^B  THEIR  MOVEMENTS  351 

when  the  eye  is  turned  downward  causes  an  appearance  that  is  strikingly 
abnormal.     This  is  sho^^^l  in  Fig.  12. 

This  is  named  for  its  discoverer,  the  Graefe  sign.  It  is  easily 
confused  with  the  Dalrymple  sign.  When  the  eyes  are  turned  down 
tliey  are  really  too  widely  open.  But  if  the  Graefe  sign  alone  be  present 
it  can  be  seen  that  there  is  no  special  retraction  of  the  lower  lid ;  and 
when  the  eyes  are  turned  up  there  is  no  abnormal  widening  of  the 
palpebral  opening.  In  practice  the  two  signs  are  very  likely  to  be 
seen  together,  there  being  both  a  widening  of  the  opening — Dalrymple's 
sign — and  a  failure  of  the  upper  lid  to  move  down  with  the  eyeball — 
Graefe's  sign.  The  disturbance  of  the  co-ordination  of  the  lid  move- 
ment and  eye  movement  is  probably  a  failure  of  function  in  the  nuclei 
controlling  these  movements ;  or  the  effect  of  some  abnormal  impulse 
or  lack  of  control  from  the  higher  centres.  (Compare  Page  833.) 

An  impairment  of  a  closely  related  function,  a  disturbance  of  the 
nervous  mechanism  concerned  in  the  reflex  movements  of  the  lids,  leads 
to  a  third  lid  sign  named  for  Stellwag.  Stellwag^s  sign  consists  of 
infrequent,  irregular,  and  incomplete  winking,  occurring  independently 
of  any  paresis  of  the  seventh  nerve  or  defective  sensibility  in  the  cornea 
or  conjunctiva.  The  eye  exhibiting  it  may  remain  a  minute  or  more 
without  winking.  Then  there  may  be  repeated  inperfect  winking  move- 
ments. Sometimes  the  movements  will  be  repeated  at  nearly  the  usual 
intervals  (two  to  five  times  a  minute).  But  most  of  the  closures  of 
the  eye  will  be  incomplete.  Where  Stellwag's  sign  is  well  marked  the 
lid  movement  of  winking  will  be  noticeably  hesitating  and  irregular 
in  character.  The  sign  of  Stellwag  is  commonly  associated  with  those 
of  Graefe  and  Dalrymple.     (Compare  Page  834.) 

Lid  Reflexes. — The  reflex  closure  of  the  lids  to  a  touch  of  a  foreign 
body  upon  the  cornea  or  conjunctiva  felt  through  the  fifth  nerve,  or 
to  the  impression  of  something  approaching  the  eye  transmitted  through 
the  retina  and  optic  nerve,  is  very  prompt,  certain,  and  quite  beyond 
the  control  of  the  will.  Any  one  who  frequently  has  to  instil  fluids 
into  the  eye  or  make  applications  to  the  conjunctiva  will  appreciate 
the  constancy  and  force  of  this  reflex.  Touching  or  striking  the  skin 
of  the  lids  or  neighboring  parts  of  the  face  will  produce  a  movement 
toward  closure  of  the  palpebral  opening. 

A  special  sensorimotor  reflex  of  the  lids  has  been  described  by 
McCarthy,  who  calls  it  the  supraorbital  reflex.  To  elicit  this  reflex 
the  patient  should  relax  the  muscles  about  the  eyes.  Then  a  slight 
tap  of  the  percussion  hammer  is  given  high  on  the  forehead.  This 
usually  excites  a  general  movement  of  closure  of  the  lids.     But  the 


352  THE    EYE    AND    NERVOUS    SYSTEM 

action  to  which  McCarthy  calls  attention  is  a  quick  fibrillary  contrac- 
tion of  the  lower  half  of  the  orbicularis  palpebrarum.  This  reflex  is 
absent  after  division,  or  disease  destroying  the  function  of  the  orbital 
nerve,  and  in  all  cases  of  peripheral  paralysis  of  the  facial  nerve.  (See 
Page  916.) 

Widening  of  the  Palpebral  Opening  by  Contracture  of  the  Ele- 
vator.— Besides  the  opening  of  the  eye  produced  by  the  healthy  exercise 
of  the  function  of  the  elevator  of  the  upper  lid  and  that  due  to  retraction 
of  the  lids  by  Mueller's  muscle,  which  is  discussed  elsewhere,  abnormal 
widening  of  tlie  palpebral  fissure  may  occur  as  a  reflex  or  choreiform 
symptom.  Posey  '  has  reported  a  case  of  rhythmical  widening  about 
fifteen  to  twenty  times  a  minute,  which  ceased  after  the  use  of  atropine 
for  the  measurement  of  ametropia.  Other  cases  of  similar  peculiar 
tremor  of  the  lids  have  been  reported  in  connection  with  exophthalmic 
goitre,  ophthalmoplegia  externa,  and  facial  paralysis.  Secondary  con- 
tracture of  the  elevator  of  the  upper  lid  sometimes  occurs  after  facial 
paralysis  of  either  peripheral  or  central  origin.  It  leads  to  abnormal 
widening  of  the  opening,  closely  resembling  the  Dalrymple  sign. 

Lagophthalmos. — Continuous  exposure  of  the  eyeball  by  inability 
to  close  the  lids  may  be  due  to  cicatricial  contraction  deforming  the  lids 
or  drawing  them  away  from  their  normal  position  and  binding  them 
down  so  as  to  interfere  with  their  function.  In  such  cases  the  scars 
responsible  for  the  deformity  are  immediately  evident  upon  inspection. 

In  elderly  people  with  great  relaxation  of  tissue  and  general  mus- 
cular weakness,  there  may  be  inability  to  close  the  eyes  fully.  In  cases 
of  this  kind  there  is  often  some  conjunctival  inflammation  and  swell- 
ing, so  that  ectropion  of  the  lower  lid  occurs  and  may  give  more  trouble 
than  the  exposure  of  the  eyeball. 

Complete  facial  paralysis  of  peripheral  origin  is  always  attended 
with  inability  to  close  the  eye  by  voluntary  contraction  of  the  orbicu- 
laris, although  the  lids  can  easily  be  drawn  together  with  the  fingers. 
With  attempts  at  forcible  closure  of  the  lids,  however,  the  eyeball  is 
normally  rolled  up  so  that  the  cornea  is  greatly  protected  from  injury ; 
and  in  many  cases  tlie  inability  to  close  the  lids  by  winking  is  replaced 
by  frequent  movements  of  the  eyeball  upward,  which  secures  the 
moistening  of  the  cornea  and  removal  of  foreign  bodies  from  its  sur- 
face. If,  however,-  these  movements  are  not  made  with  sufficient  fre- 
quency, or,  if  in  a  case  of  long  standing,  secondary  contracture  of  the 
upper  lid  increases  the  exposure  of  the  eye-ball,  there  is  great  danger 

'  Journal  of  Nervous  and  Mental  Disease,  July,  1902. 


THE  LIDS  AXD  THEIR  MOVEMENTS  353 

of  serious  disease  of  the  cornea.  This  is  particularly  likely  to  occur 
in  elderly  or  poorly  nourished  patients  or  when  the  sensibility  of  the 
cornea  is  impaired. 

Exposure  Keratitis. — Lagophthalraic  keratitis  arises  from  the  un- 
due drying  of  the  exposed  portion  of  the  cornea.  It  begins  with  dis- 
turbance of  the  epithelium  and  superficial  ulceration  near  the  lower 
corneal  margin.  Infection  occurring  through  the  channels  thus  opened 
to  it  leads  to  purulent  infiltration  of  the  subjacent  tissue.  The  infil- 
tration runs  a  comparatively  chronic  course  and  is  marked  by  slight 
inflammatory  reaction,  as  compared  with  that  which  usually  attends 
an  infective  keratitis  of  equal  extent  and  gravity.  If  unchecked,  the 
process  goes  on  to  perforation  of  the  cornea,  extensive  adherent  leu- 
coma,  and  practical  loss  of  usefulness  of  the  eye. 

The  treatment  of  this  condition  must  include  careful  protection  of 
the  eye  from  irritants,  as  by  dressings  that  will  close  the  lids  and  hold 
them  shut.  If  corneal  infection  requiring  active  treatment  has  not 
occurred  or  has  been  overcome,  the  most  efiicient  means  of  protecting 
the  eye  is  by  uniting  the  margins  of  the  lids.  These  may  be  freshened 
and  brought  together  by  sutures  throughout  a  part  of  the  palpebral 
fissure.  Usually  the  tarsorraphy  is  done  at  the  outer  canthus,  but  in 
some  cases  a  better  effect  will  be  obtained  by  uniting  the  lid  margins 
near  the  region  of  the  lachrymal  puncta.  In  this  way  we  secure  the 
proper  drainage  of  tears  and  leave  the  temporal  portion  of  the  palpebral 
opening  free,  preserving  part  of  the  field  of  vision.  The  exact  extent 
and  location  of  the  union  of  the  lids  must  be  determined  by  study  of 
the  individual  case.  If  union  of  the  lids  be  resorted  to  early,  before 
infection  has  occurred,  the  prognosis  is  quite  favorable.  And  if  at  a 
subsequent  time  recovery  of  the  power  of  the  orbicularis  occurs,  the 
connection  between  the  lids  can  readily  be  divided. 

Ptosis. — The  conditions  apart  from  abnormal  innervation  which 
cause  narrowing  of  the  palpebral  opening  have  already  been  referred 
to  (page  347).  Congenital  ptosis,  hysterical  ptosis,  and  ptosis  due 
to  disease  of  the  oculomotor  nerve,  or  of  the  central  nervous  system,  are 
discussed  in  Chapter  V.  It  remains  here  to  consider  the  general 
characteristics  of  ptosis,  a  few  forms  arising  from  local  conditions, 
and  a  brief  outline  of  the  treatment.  In  a  general  way  ptosis  is  due 
to  relaxation  of  the  elevator  of  the  lid  or  to  a  more  or  less  permanent 
contracture  of  the  orbicularis. 

Relaxation  of  the  elevator  is  attended  with  comparative  smooth- 
ness of  the  skin  of  the  lid  and  partial  obliteration  of  the  upper  oculo- 
orbital  fold  (Fig.  13).  Complete  paralysis  of  the  elevator  is  shown 
23 


364  THE    EYE    xYNU    NERVOUS    SYSTEM 

by  the  fact  that  on  looking  upward  there  is  no  deeping  of  this 
fold.  Contraction  of  tlie  orbicularis  tends  to  cause  wrinkling  of  the 
skin  of  the  lids  and  adjoining  parts. 

Temporary  Ptosis. — Any  inflammatory  disease  of  the  eye  which  con- 
tinues many  days  is  liable  to  cause  narrowing  of  the  palpebral  opening. 
This  occurs  quite  independently  of  any  exudation  into  the  lids  that 
might  mechanically  oppose  tlieir  full  opening.  It  is  at  first  an  effort 
to  protect  the  inflamed  eye  from  light  or  irritation.  It  is  most  notice- 
able in  connection  with  chronic  inflammation  affecting  but  one  eye. 
Such  a  ptosis  may  be  continued  indefinitely  to  diminish  the  light  fall- 
ing on  a  nebula  left  by  corneal  inflammation.     But  even  when  no  such 


Fig.  13.— Ptosis:  obliteration  of  palpebro-orbital  fold.    (Case  of  Dr.  Jackson.) 

sequel  remains  and  the  inflammation  has  entirely  passed  away,  nerve 
habit  may  continue  for  a  time  a  drooping  of  the  lid.  This  drooping, 
however,  will  slowly  diminish,  and  in  the  course  of  weeks  or  months 
will  entirely  disappear.  The  process  of  recovery  may  be  hastened  by 
massage  and  voluntary  efforts  to  open  the  eye  widely.  Possibly  electric 
stimulation  of  the  muscle  may  be  of  benefit.  But  such  ptosis  may  be 
due  to  contracture  of  the  orbicularis  rather  than  to  relaxation  of  the 
elevator,  and  injudicious  use  of  electricity  in  such  a  ease  would  be 
pretty  certain  to  do  more  harm  than  good. 

Another  form  of  temporary  ptosis  is  seen  in  persons  with  organic 
disease  of  the  central  nervous  system.  Sometimes  the  upper  lid  will 
droop  for  a  few  hours,  sometimes  for  days  or  weeks,  although  the  patient 
can  usually  open  one  eye  widely  by  special  effort  and  there  may  be 
no  other  evidence  of  oculomotor  paresis.  This  form  depends  on  relax- 
ation of  the  elevator.     The  prognosis  regarding  it  is  very  uncertain, 


THE  LIDS  AXD  THEIR  MOVEMENTS  355 

but  it  may  disappear  permanently,  even  after  it  has  been  troublesome 
for  a  long  time. 

Xot .  a  few  persons  complain  of  special  difficulty  in  opening  the 
eyes  after  particularly  sound  sleep.  Although  there  is  no  adhesion 
of  the  lids  and  the  patient  seems  to  have  fully  regained  consciousness, 
there  remains  a  sense  of  inability  to  move  the  lids.  This  may  be  com- 
pared with  the  feeling  experienced  in  a  nightmare,  of  inability  to  per- 
form some  act  in  spite  of  the  strongest  desire  to  do  it.  The  specially 
intimate  connection  of  closure  of  the  eyes  with  the  phenomena  of  sleep 
is  probably  the  basis  of  this  kind  of  ptosis. 

Atonic  Ptosis. — Without  loss  of  power  in  the  elevator  of  the  upper 
lid,  and  with  normal  movement  of  the  tarsal  cartilage,  the  skin  of  the 
lid  may  form  a  fold  hanging  down  over  the  lid  margin  and  roots  of 
the  lashes,  so  as  to  limit  materially  the  field  of  vision.     This  condition 


Fig.  H. — Swelling  of  lida  following  traumatism.    (Case  of  Dr.  Jackson.) 

may  be  simulated  by  a  redundancy  of  tissue.  In  the  older  works  it 
was  called  ptosis  from  hypertrophy,  while  atonic  ptosis  (Mackenzie) 
was  used  to  designate  what  would  now  be  known  as  hysterical  ptosis. 
Anatomical  examination  of  the  most  typical  cases  of  this  class  has 
shown  atro'phy  rather  than  hypertrophy  of  the  tissue.  Hotz  pointed 
out  that  the  condition  was  due  to  impairment  or  destruction  of  the 
normal  relation  between  the  skin  of  the  upper  lid  and  the  tarsus.  So 
that  when  the  tarsus  was  retracted  the  relaxed  skin  formed  a  fold  which 
fell  over  its  margin.  The  connection  which  Dwight  has  pointed  out, 
between  a  part  of  the  skin  and  a  slip  of  Mueller's  muscle  connecting 
it  with  the  tendon  of  the  elevator,  suggests  that  this  form  of  ptosis 
may  be  due  to  relaxation  of  this  muscle,  and  properly  rank  as  a  form 


356  THE    EYE    AXD    :N"ERV0US    SYSTEM 

of  ptosis  from  disease  of  the  sympathetic.  Its  treatment  will  be  men- 
tioned under  the  operative  treatment  of  ptosis. 

Traumatic  Ptosis. — Scars  involving  the  lids  may  cause  ptosis,  but 
this  form  need  not  be  described  here.  Attention  is  called  to  it  that 
inability  to  raise  the  lids  from  this  cause  shall  not  be  confused  with 
that  due  to  impaired  nerve  action.  Penetrating  wounds  of  the  orbit 
may  involve  the  branch  of  tlie  oculomotor  nerve  which  supplies  the 
elevator  muscle.  But  they  are  more  likely  to  do  direct  injury  to  the 
muscle.  If  the  wound  be  extensive  the  involvement  of  the  muscle  in 
the  resulting  scar  is  likely  to  produce  permanent  disability.  Ptosis 
from  this  cause  is  illustrated  in  Fig.  14.  Some  power  of  movement 
may  be  regained  with  the  subsidence  of  the  inflammatory  swelling,  but 
some  permanent  impairment  is  the  rule  after  such  injuries. 

Operative  Treatment  of  Ptosis. — Without  discussing  the  numerous 
ptosis  operations  in  detail,  they  may  be  mentioned  with  the  principle 
involved  in  each.  For  traumatic  ptosis  a  plastic  operation,  planned 
to  meet  the  indications  of  the  particular  injury,  may  be  required.  The 
object  aimed  at,  in  such  an  operation,  will  be  the  freeing  of  the  lid  from 
abnormal  restriction  of  its  upward  movement. 

For  ptosis  due  to  hypertrophy  eitlier  of  the  skin  of  the  lid  or  sub- 
cutaneous tissue,  the  removal  of  the  proper  quantity  of  hypertrophied 
tissue  is  the  rational  procedure.  But  for  ptosis  not  due  to  actual  hyper- 
trophy, even  for  that  which  is  evidently  associated  with  great  relaxa- 
tion, the  mere  removal  of  tissue  is  likely  in  the  end  to  prove  unsatis- 
factory. Excision  of  an  elliptical  piece  of  skin  may  be  of  some  benefit 
in  the  slighter  degrees  of  paretic  ptosis.  But  contraction  thus  brought 
about  tends  to  obliterate  the  fold  between  the  lid  and  upper  margin 
of  the  orbit,  causing  a  deformity  as  noticeable  as  that  which  it  removes. 
Excision  of  an  excessively  large  flap,  in  addition  to  causing  this  new 
deformity,  may  produce  a  dangerous  lagophthalmos,  while  it  still  fails 
to  remove  the  ptosis  on  turning  the  eye  upward. 

A  more  reliable  operation  and  one  that  does  not  cause  the  same 
obliteration  of  the  oculomotor  fold  is  that  of  Wilder.  lie  exposes  the 
tarso-orbital  fascia  by  making  an  incision  just  below  the  brow  and  dis- 
secting a  flap  down  to  the  tarsus.  Sutures  introduced  through  the  mar- 
gin of  the  tarsus  are  carried  in  and  out  through  the  fascia,  as  gathering 
stitches,  up  to  the  line  of  incision,  drawn  upon  sufficiently  to  give  the 
proper  elevation  of  the  lid,  tied  and  cut  short.  The  incision  is  closed  by 
skin  sutures.  This  gathers  or  folds  the  fascia  upon  itself,  and  leaves  an 
almost  imperceptible  scar. 

Atonic  ptosis,  which  has  often  been  taken  for  hypertrophic  and 


THE  LIDS  AND  THEIR  MOVEMENTS  357 

subjected  to  excision,  should  be  treated  as  suggested  by  Hotz.  An 
incision  in  the  general  direction  of  the  lid  margin,  two  or  three  milli- 
metres from  the  margins  at  its  ends,  and  somewhat  farther  from  the 
margin  in  the  centre,  is  made  through  the  skin  and  down  to  the  cartilage. 
This  incision  is  closed  by  sutures  which  pass  first  through  the  lower  lip 
of  the  incision  and  then  through  the  upper  margin  of  the  tarsus.  These 
sutures  when  tightened  stretch  the  lower  flap  of  skin  and  unite  it  firmly 
to  the  tarsus,  so  that  the  subsequent  folding  or  falling  below  the  lid 
margin  would  be  impossible. 

For  ptosis  due  to  paresis  of  the  elevator,  operations  for  the  advance- 
ment, resection,  or  folding  (tucking)  of  the  tendon  of  the  elevator  are 
appropriate.  In  Eversbusch's  folding  operation  the  tendon  is  exposed 
four  millimetres  above  its  insertion  into  the  tarsus.  A  suture  with  a 
needle  on  each  end  is  applied  to  the  tendon,  and  the  needles  carried 


Fig.  15. — Complete  bilateral  ptosis.    Maximum  elevation  of  the  lids  by  contraction 
of  the  frontalis.     (Case  of  Dr.  Jackson.) 

between  the  skin  and  tarsus  to  the  lid  margin,  where  they  are  brought 
out  and  the  ends  of  the  suture  tied  over  a  bit  of  rubber  tubing.  Three 
of  these  sutures  are  placed :  one  at  the  centre  and  one  at  each  end  of 
the  lid.  The  tightening  of  these  sutures  folds  the  tendon  down  over 
the  tarsus. 

Snellen's  operation  is  a  resection.  The  tendon  is  exposed  and  three 
or  four  needles  thrust  through  it  the  necessary  distance  back  from  the 
tarsal  margin.  The  end  of  the  tendon  is  then  excised  by  parallel  in- 
cisions along  the  tarsal  margin  and  in  front  of  the  needles.  The  nee- 
dles being  brought  through  the  upper  margin  of  the  tarsus,  and  the 
threads  tightened,  the  desired  shortening  of  the  tendon  is  secured. 

Wolff  does  resection  by  exposing  the  tendon  and  raising  it  on  two 
strabismus  hooks.     The  sutures  are  introduced  through  the  tendon  just 


.'^58  THE    EYP:    and    NERVOUS    SYSTEM 

below  the  upjier  hook.  The  end  of  the  tendon  is  then  cut  away  from 
the  tarsus,  and  the  sutures  passed  through  the  insertion  of  the  tendon 
into  the  tarsus. 

De  Lapersonne  practises  advancement  of  the  tendon  of  the  levator, 
with  or  without  excision,  by  suturing  the  tendon  upon  the  outer  surface 
of  the  tarsus  instead  of  to  its  upper  margin. 

For  paralysis  of  the  elevator  which  is  complete  and  permanent,  the 
only  operations  worthy  of  much  attention  are  those  which  give  power 
to  raise  the  lid  by  attaching  it  to  some  other  muscle  which  may  thus 
be  made  to  perform  a  vicarious  function.  The  effort  of  nature  in  these 
cases  is  to  drag  the  lid  upward  through  its  skin  connection  with  the 
frontalis.     The  result  achieved  is  shown  in  Fig.  15. 

To  give  a  more  firm  and  efficient  connection  between  the  lid  and 
frontalis,  Dransart  sought  to  produce  a  scar  by  introducing  cat-gut 
sutures  subcutaneously  from  the  lid  to  the  brow.  To  increase  the 
cicatricial  change,  Pagenstecher  substituted  silk  for  the  catgut.  This 
was  tied  over  a  bit  of  rubber  tubing  above  the  brow,  allowing  it  to  be 
shortened  from  time  to  time  as  seemed  desirable.  Mules,  finding  the 
scars  secured  by  such  measures  left  much  to  be  desired  in  the  way  of 
permanence  and  rigidity,  introduced  a  silver  wire  by  which  the  desired 
elevation  of  the  lid  was  secured.  The  wire  was  then  knotted  and  the 
knot  buried  above  the  brow.  Worth  has  used  kangaroo  tendon  for  this 
purpose.  Harman,  instead  of  silver  wire,  has  employed  the  light, 
woven  gold  chain  sometimes  used  for  an  eyeglass-cord.  This  being 
thoroughly  sterilized  and  buried  becomes  intimately  incorporated  with 
scar-tissue,  and,  being  entirely  flexible,  is  less  likely  to  cause  subsequent 
disturbance  than  is  the  silver  wire. 

The  connection  of  the  lid  and  frontalis  tendon  by  transplanted  tissue 
was  effected  by  Panas  in  the  operation  which  bears  his  name.  In  this 
operation  an  incision  is  made  parallel  to  the  brow  above  it  and  another 
below.  The  tissue  between  them  is  undermined.  A  broad,  tongue- 
shaped  flap  of  skin  and  subcutaneous  tissue  is  dissected  up  from  the 
lid  and  oculomotor  fold,  carried  up  under  the  bridge  of  tissue,  and  made 
fast  at  the  insertion  of  the  frontalis  muscle  into  the  skin  of  the  brow, 

Panas's  operation  has  been  the  subject  of  slight  modifications  by 
individual  operators;  but  is  essentially  the  operation  most  commonly 
done  to-day  for  this  form  of  ptosis.  All  operations  that  aim  to  connect 
the  lid  witli  the  frontalis  tendon,  whether  by  thread,  wire,  scar-tissue, 
or  flap,  have  these  defects :  the  oculo-orbital  fold  is  more  or  less  obliter- 
ated and  the  movement  of  the  lid  is  at  best  accomplished  by  an  abnor- 
mal contraction  of  the  frontalis. 


THE  LIDS  AND  THEIR  MOVEMENTS  359 

The  muscle  which  normally  contracts  in  closest  association  with  the 
elevator  of  the  upper  lid  is  the  superior  rectus.  In  a  normal  state  every 
contraction  or  relaxation  of  the  one  muscle  is  accompanied  by  a  similar 
contraction  or  relaxation  of  the  other;  except  that  when  the  eye  is 
strongly  closed  the  elevator  of  the  lid  is  relaxed,  but  the  eyeball  is  rolled 
up  by  contraction  of  the  rectus. 

Motais  devised  the  operation  of  connecting  a  portion  of  the  tendon 
of  insertion  of  the  rectus  with  the  tarsus.  An  incision  being  made 
through  the  conjunctiva  and  subconjunctival  tissue  down  to  the  tendon 
of  the  superior  rectus  on  the  eyeball,  and  in  the  direction  of  the  tendon, 
is  carried  back  to  the  retrotarsal  fold  and  through  the  palpebral  con- 
junctiva to  the  tendon  of  the  elevator  of  the  lid.  The  rectus  tendon  is 
isolated,  split  into  three  parts,  and  the  middle  part  separated  from 
the  insertion  into  the  sclera  and  by  two  sutures  carried  up  and  inserted 
in  the  upper  margin  and  external  surface  of  the  tarsus. 

Thus  treated,  the  superior  rectus  muscle  quickly  gains  power  to 
move  both  the  lid  and  eyeball.  So  that  henceforth  the  lid  moves  nor- 
mally with  the  globe  of  the  eye,  except  that  on  forcible  closure  of  the 
lids  the  upper  lid  is  pulled  up  with  the  effort  of  the  superior  rectus  to 
roll  the  eye  upward.  Motais's  operation  causes  no  visible  scar  and 
tends  to  restore  to  normal  the  oculo-orbital  fold.  It  gives  to  the  lid 
its  most  important  and  useful  movements.  But  it  does  not  confer 
the  power  of  retraction  of  the  lid  independently  of  movements  of  the 
eyeball. 


CHAPTER   VIII. 
DISEASES  OF  THE  RETINA  AND  OPTIC  NERVE. 
By  H.  V.  WlJRDEMANN,  M.  D. 

Ophthalmoscopy. 
Since  the  possibility  of  examining  the  interior  of  the  eye  by  means 
of  the  oi)hthahiioscope  was  demonstrated  by  von  Hehiiholtz  in  1851, 
much  light  has  been  thrown,  by  the  use  of  this  instrument,  upon  various 


Fig.  1.— The  Loriug  opbtb&lmoecope. 

general  as  well  as  ocular  diseases,  and  the  diagnosis  of  many  general 
disorders  and  diseases  of  the  ner\'ous  system  has  been  greatly  simplified. 
Though  the  instrument,  as  originally  devised  by  von  Helmholtz,  pos- 
sessed all  the  properties  essential  to  a  thorough  examination  of  the 
fundus,  its  elaboration  and  improvement  by  Rente,  Liebreich,  Loring, 

and  Morton,  have  added  greatly  to  the  facility  with  which  it  may  be 
360 


•=  a 

H  5 


"35 
S 


OPHTHALMOSCOPY  361 

used  and  have  rendered  it  possible  for  the  expert  to  estimate  very  accu- 
rately the  refraction  of  the  eye. 

The  accompanying  cuts  illustrate  the  Loring  ophthalmoscope  (Fig. 
1),  the  most  popular  instrument  in  this  country,  and  a  model  of  an  elec- 
tric-lighted ophthalmoscope  (Fig.  2),  which  is  especially  serviceable  for 
bedside  work.  Fig.  3  illustrates  a  model  of  a  stationary  oi)hthalmoscope. 
This  instrument  is  a  valuable  adjunct  to  the  hand  ophthalmoscope,  as  it 
gives  an  extended  view  of  the  magnified  and  inverted  image,  is  most 
useful  for  demonstration  to  students,  and  for  the  purpose  of  reproduc- 
ing fundus  pictures  by  drawiiig  or  photography. 

In  ophthalmoscopy  the  illumination  should  be  steady  and  uniform, 
and  while  the  fundus  may  be  seen  on  bright  days  with  ordinary  day- 
light passing  through  a  small  aperture  into  a  darkened  room,  this  source 
of  illumination  can  be  used  only  occasionally,  and  thus  most  of  our  con- 
ceptions of  the  fundus  are  based  upon  observation  by  yellow  artificial 
light;  a  candle  or  lamp  flame,  a  gas  jet  or  an  electric  light  covered  by 
frosted  glass,  being  utilized  for  the  purpose.  In  the  performance  of 
all  ordinary  tests  with  the  ophthalmoscope,  both  the  observer  and  the 
patient  should  be  comfortably  seated.  If  the  patient  be  bedridden 
this  is  impossible  and  the  examination  must  often  be  conducted  from 
a  constrained  posture.  Although  this  is  often  fatiguing,  the  surgeon 
should  not  desist  until  he  has  satisfied  himself  that  he  has  accomplished 
all  that  was  possible  under  the  circumstances. 

The  interior  of  the  eye  may  be  viewed  with  the  ophthalmoscope  by 
either  the  direct  or  indirect  method.  The  former  is  to  be  preferred,  for 
although  somewhat  more  difficult  of  performance,  it  permits  of  a  study 
of  the  fundus  under  a  much  greater  magnification,  the  back  part  of 
the  eye  being  magnified  14  diameters  by  the  direct  method,  and  but 
4  by  the  indirect,  and  at  the  same  time  it  enables  the  observer  to  obtain 
a  very  accurate  idea  of  the  refraction  of  the  eye.  The  indirect  method 
is  only  to  be  preferred  in  high  myopia  and  in  cases  where  the  media  of 
the  eye  are  hazy.  Before  resorting  to  the  study  of  the  interior  of 
the  eye,  the  observer  should  always  obtain  as  much  information  as  is 
possible  of  the  media  by  means  of  transmitted  light.  For  this  purpose, 
the  observer,  at  a  distance  of  about  30  centimetres,  flashes  the  light 
which  is  placed  behind  and  slightly  to  the  side  of  the  patient's  head 
into  the  eye,  by  means  of  the  mirror  of  the  ophthalmoscope.  A  faint 
pinkish-red  glow  will  be  seen  to  replace  the  blackness  of  the  pupil.  This 
is  known  as  the  fundus  reflex,  and  if  the  glare  be  unbroken  and  uni- 
formly bright,  even  when  the  eye  is  moved  about  through  the  different 
meridians,  the  media  are  clear  and  no  opacities  obscure  vision. 


362  THE    EYE    AND    NERVOUS    SYSTEM 

The  Direct  Method. — Having  satisfied  himself  of  the  condition 
of  the  media,  the  observer  now  brings  his  chair  alongside  the  patient's, 
and  concentrating  the  light  upon  the  cornea,  by  means  of  the  ophthal- 
moscope which  he  holds  in  close  apposition  with  his  own  eye,  he  en- 
deavors to  approach  as  closely  as  possible  to  the  patient's  eye.  If  he 
will  now  relax  his  accommodation,  by  fancying  that  what  he  is  now 
regarding  is  situated  far  in  the  distance,  and  request  the  patient  to 
look  straight  forward  into  the  darkness,  for  ophthalmoscopy  should 
preferably  be  performed  in  a  darkened  room,  the  observer  will  now 
see  the  vessels  of  the  retina  and  the  head  of  the  optic  nerve. 

Attention  should  be  paid  next  to  the  macular  region,  which  may 
be  brought  into  view  by  slight  movements  of  the  patient's  eye  to  the 
right  or  left,  and  finally  the  patient  should  look  up,  down,  in  and  out, 
as  requested  by  the  observer,  bringing  the  various  parts  of  the  fundus 
into  view.  Observations  are  made  of  the  color  of  the  fundus  and  of 
the  optic  disk;  of  changes  in  the  elevation  of  the  disk  by  focussing  it 
and  the  surrounding  areas;  of  changes  in  the  blood-vessels,  hemor- 
rhages, pigment  deposits,  and  degenerative  areas. 

The  Indirect  Method. — In  the  application  of  this  method,  the  ob- 
ser^•er  stations  himself  in  a  position  corresponding  to  that  assumed 
in  the  test  by  transmitted  light  and  throws  the  light  into  the  eye 
under  examination  by  the  concave  mirror  of  the  ophthalmoscope.  A 
convex  lens  of -{-14.00  D.  strength  is  then  held  before  the  eye  to  be 
examined,  and  an  aerial  image  obtained  of  that  portion  of  the  fundus 
illuminated  by  the  reflected  light.  In  case  the  details  of  the  fundus 
are  not  plainly  seen  at  first,  the  object  lens  should  be  slightly  moved 
to  or  from  the  eye,  as  a  compensation  for  the  patient's  error  of  refrac- 
tion. The  image  obtained  by  the  indirect  method  is  inverted,  and  it 
must  be  remembered  that  the  upper  part  of  the  image  corresponds  to 
the  lower  part  of  the  eye-ground  and  the  right  side  of  the  image  to  the 
left  of  the  eye-ground. 

The  Normal  Fundus.- — The  normal  eye  presents  many  variations, 
and  an  extended  experience  is  frequently  necessary  to  distinguish  con- 
genital variations  from  changes  wrought  by  disease. 

The  Fundus  Reflex. — The  color  in  normal  eyes  is  bright  pink  or 
red,  due  to  the  reflection  from  the  pigment  of  the  retina,  choroid,  and 
the  choroidal  vessels.  In  the  albino  there  is  no  fundus  pigment,  and 
the  reflex  is  that  of  the  choroidal  and  retinal  vessels  with  the  sclera 
as  the  background,  and  is  a  light  pink.  In  tlie  negro  the  reflex  is  gray, 
on  account  of  absorption  of  the  light  rays  by  the  abundant  pigment 
(See  Plate  III.) 


Fig.  6.— Region  of  optic  disk  showing  opEique  nerve-fibres  and  two  cilioretinal 

vessels  (from  photograph). 


OPHTHALMOSCOPY  363 

The  Papilla. — The  optic  disk  or  nerve-head  is  about  1.75  millimetres 
in  diameter,  appearing  under  the  ophthalmoscope  enlarged  about  fifteen 
times,  and  hence  from  10  to  15  millimetres  (one-half  to  six-«ighths 
inches)  in  width.  It  is  round  or  oval,  with  clear-cut  margins;  its 
•centre  is  whitish,  though  the  connective  tissue  lace-work  supporting 
the  nerve-fibres  and  the  lamina  cribosa  are  a  mottled  gray.  The 
periphery  of  the  disk  is  pink,  owing  to  fine  blood-vessels  in  its  structure 
and  on  its  surface.  (Plate  III.)  There  is  usually  an  excavation  in 
the  nerve-head,  due  to  the  entrance  of  the  retinal  vessels ;  in  some  cases 
this  is  quite  small,  while  in  others  it  is  so  large  that  it  embraces  nearly 
the  entire  papilla.  This  excavation  is  physiological  and  should  not 
be  confused  Avith  that  of  glaucoma,  which  occupies  the  whole  of  the 
disk,  or  that  which  is  consecutive  to  optic  nerve  atrophy. 

The  Blood-Vessels. — The  retinal  vessels  leave  the  disk  slightly  to 
the  nasal  side  of  the  centre  and  divide  into  two  or  more  main  stems, — 


Fig.  7.— Diagram  of  the  blood-vessels  of  the  human  retina  (Leber,  after  Jaeger), — ana,  vns,  superior 
-nasal  artery  and  vein ;  ats,  vts,  superior  temporal  artery  and  vein  ;  ani,  mi,  inferior  nasal  vessels;  ati, 
vti,  inferior  temporal  vessels  ;  ame,  vine,  median  vesseis ;  am,  vm,  macular  vessels. 

the  superior  and  inferior  retinal,  sending  numerous  fine  branches  to 
the  adjacent  retina.  Just  above  the  disk  each  trunk  bifurcates  into 
Taranches  which  pursue  a  semicircular  course,  curving  round  towards 
the  temporal  and  nasal  parts  of  the  eye.  The  veins  follow  the  arteries 
in  their  course  more  or  less  closely,  but  may  be  differentiated  from 
them  by  being  darker  in  color,  and  being  about  one-third  larger.     Both 


364  THE    EYE    AND    NERVOUS    SYSTEM 

arteries  and  veins  have  a  light  streak  along  the  centre,  which  is  fainter 
upon  the  veins,  the  refies.  streak.  A  distinct  pulse  occurs  physiologi- 
cally in  the  veins  upon  the  head  of  the  nerve,  but  pulsation  in  the 
arteries  is  always  pathological.  The  clinician  should  acquire  the 
habit  of  observing  the  retinal  blood-vessels  with  great  care,  for  their 
condition  is  frequently  indicative  of  the  state  of  the  blood-vessels  else- 
where throughout  the  system;  and  on  account  of  the  possibility  of 
viewing  the  actual  blood-column  itself  an  exceptional  opportunity  is 
ofiFered  of  diagnosing  pathological  conditions  of  the  blood. 

The  Macula. — The  yellow  spot,  which  is  situated  about  2  disk  diam- 
eters (3  mm.)  towards  the  temporal  side  of  the  nerve  in  the  horizontal 
meridian  of  the  fundus,  is  the  most  sensitive  part  of  the  retina.  It 
consists  of  an  apparently  yellow  spot,  surrounded  by  a  circular  or  oval 
area,  the  foveal  region,  which  is  darker  in  color  than  the  rest  of  the 
fundus  and  about  the  size  of  the  disk.  The  centre  of  the  macula  is 
known  as  the  fovea  centralis,  and  appears  as  a  glistening  reflex.  The 
blood-vessels  do  not  cross  the  fovea  but  converge  towards  it.  In  order 
to  obtain  the  best  view  of  the  macula,  the  ophthalmoscope  should  be 
slowly  moved  upward  and  inward,  while  the  observer  brings  his  line 
of  vision  to  a  point  about  two  to  two  and  a  half  disk  diameters  out- 
ward from  the  disk,  ^^^len  the  pupil  is  dilated  the  macula  may  be  seen 
by  having  the  patient  gaze  directly  into  the  sight-hole  of  the  mirror. 
The  periphery  of  the  eye-ground  should  be  carefully  studied;  and  in 
order  that  no  part  of  it  may  escape,  it  is  well  for  the  student  to  follow 
each  branch  of  the  central  artery  of  the  retina  as  far  forwards  as 
possible. 

Ophthalmoscopic  Signs  of  General,  and  Nervous  Diseases. 

Certain  conditions  of  the  fundus  oculi  are  of  preeminent  interest 
and  value  in  the  diagnosis  of  various  general  and  nervous  diseases: 

(1)  Sensory  affections :  hyperesthesia,  anaesthesia. 

(2)  Vascular  lesions : 

(a)    Changes   in    the   blood-vessels:    anaemia,   hyperemia, 

pulsation,  opdema. 
(6)   Inflammation  of  the  vessel-walls, 
(c)   Hemorrhages  from  the  vessels. 
{d)   Stoppage  of  the  vessels :   embolism,  thrombosis. 

(3)  Inflammation  of  the  retina. 

(4)  Inflammation  and  oedema  of  the  optic  nerve:    optic  neuritis, 

choked  disk. 

(5)  Atrophy  of  the  optic  nerve. 


PLATE  III. 


.M 

9 

^ 

^^^Sa^^li^EC: 

■r'v^"?^ 

-•\' 

:^^^H 

S 

■^.1^ 

Wk 

■  :<'^ 

M 

^^^ 

W 

C  JJ 

Varieties  of  the  Normal  Fundus.—^.  The  albinotic  fundus;  albino  and  light  blonde  (after 
Greeff,  modified  by  H.  V.  W.) ;  B.  The  tessellated  fundus ;  brunette  (after  Greeflf,  modified  by  H.  V.  W.) ; 
C.  The  negroid  fundus;  Negro  (H.  V.  W.) ;  D.  The  yellow  fundus;  Chinese  (after  Oeller,  modified  by 
H.  V.  W.) 


DISEASES  OF  THE  RETINA 


365 


These  lesions  are  also  described  under  headings  descriptive  of  various 
diseases, — i.e.,  as  syphilitic,  albuminuric,  diabetic,  and  anaemic  retinitis. 

(i)  SENSORY  AFFECTIONS. 

Hyperaesthesia  is  due  to  retinal  irritation  and  hypersemia  giving  rise 
to  photophobia,  asthenopia,  and  blepharospasm.  It  is  usually  origi- 
nated by  local  affections,  such  as  uncorrected  ametropia,  presbyopia, 
and  muscular  imbalance,  but  it  may  likewise  occur  in  neurasthenia, 
gout  and  oxaluria.  Anaesthesia  of  the  retina  is  characterized  by  diminu- 
tion of  the  central  visual  acuity  and  contraction  of  the  visual  field,  or 
misplacement  of  the  boundaries  of  the  field  or  by  changes  in  the  bound- 


!Right 


870° 


YiG.  8.— Hysteric  Field,  simulating  Tempo-  Fig.  9.— Hysteric  Field,  showing  Progres- 

RAL  HEMIANOPIA  WITH  GREAT  REDUCTION  OF  VIS-  SIVE  CONTRACTION  (FATIGUE  TYPE)  AND  OVER- 
UAL  ACUITY.  LAPPING   OF  COLOR  FIELD. 

1.  Form  limits ;  2.  Red,  taken  immediately  afterwards ;  3.  Red,  taken  5  minutes  later ;  4,  Form,  taken 
5  minutes  still  later. 

aries  for  color  vision  (Figs.  8-9).  It  is  a  prominent  symptom  of 
hysteria  and  neurasthenia,  is  present  in  exhausting  fevers  and  in  con- 
valescence, and  in  women  with  pelvic  diseases.  It  may  be  induced 
by  traumatism  and  by  suggestion.  The  ophthalmoscope  usually  revfeals 
hypersemia  or  anaemia  of  the  nerve-head  and  retina. 


(2)  VASCULAR    LESIONS. 

Anaemia  of  the  disk,  as  well  as  the  retina,  expresses  impaired  nutri- 
tion. It  is  found  in  ischsemia  and  atrophy  due  to  functional  disturb- 
ances and  blood  diseases,  and  after  severe  hemorrhages.  With  the  oph- 
thalmoscope the  arteries  are  seen  to  be  apparently  contracted,  carry 
less  blood,  and  are  of  a  light  color ;  the  veins  are  full  and  even  tortuous. 


36G  THE    EYE    AND    NERVOUS    SYSTEM 

The  papilla  is  pale,  and  there  are,  as  a  nile,  no  small  vessels  on  its  sur- 
face.     (Plate  TV.,  A.) 

Hyperaemia  evidences  itself  most  prominently  in  the  papilla,  and  i» 
characterized  hy  increase  in  the  number  of  visible  blood-vessels  and 
by  dilatation  and  tortuosity  of  the  veins.  True  pathological  increase 
of  the  vessels  is  rare,  except  in  the  beginning  of  optic  neuritis  or  accom- 
panying retinitis.  The  retinal  vessels  become  abnormally  vascular  if 
the  cause  be  mechanical  obstruction  to  the  blood,  either  going  from  or 
coming  to  the  eye,  the  chorioid  likewise  shows  congestion,  and  the  pink 
color  of  the  healthy  fundus  assumes  a  dark  uniform  red.  Physiologic 
variations  in  the  color  of  the  nerve-head,  on  account  of  the  contrast 
afforded  by  the  varying  amount  of  pigment  in  the  retinal  layer  of  the 
chorioid,  should  always  be  considered  in  making  a  diagnosis. 

Causes. — Ana?mia  and  hypenemia  are  symptoms  of  both  constitu- 
tional and  local  diseases.  Among  the  constitutional  causes  may  be 
mentioned  retention  in  the  blood  of  excretory  products,  degenerative 
diseases  of  the  cerebral  cortex,  as  epilepsy  and  general  paralysis,  hyper- 
trophied  and  overacting  heart  from  obstruction  of  the  circulation,  or 
thrombosis,  organic  disease  of  the  abdominal  organs,  uterine  disease, 
the  beginning  and  cessation  of  menstruation,  and  finally  the  circulation 
of  malarial  or  other  toxic  materials  in  the  blood.  Locally,  the  usual 
causes  are  chronic  inflammation  of  the  uveal  tract  and  errors  of  refrac- 
tion, especially  hyperopia  and  astigmatism,  with  accommodative  strain. 

Treatment. — The  treatment  consists  in  the  removal  of  the  cause,  with 
the  use  of  atropine  and  the  correction  of  errors  of  refraction,  conjoined 
with  the  administration  of  alteratives  and  the  stimulation  of  the  excre- 
tory organs. 

Abnormalities  in  the  size  of  the  retinal  arteries  and  veins  may 
occur  as  a  result  of  some  intra-ocular  inflammation,  or  may  portend 
some  serious  affection  behind  the  eyeball. 

Atrophy  of  the  vessels  occurs  in  association  with  atrophy  of  the  retina 
and  nerve.     Varicosity  of  the  veins  may  be  congenital. 

Pulsation  of  the  retinal  veins  is  frequently  physiological,  whereas 
pulsation  of  the  arteries  is  always  pathological,  indicating  an  enlarged 
heart,  some  vascular  affection  of  the  head  or  orbit,  or  glaucoma. 

CEdema  of  the  retina  may  be  general  or  limited  to  a  certain  por- 
tion. It  manifests  itself  as  an  opaque,  slightly  elevated  area,  with  the 
blood-vessels  running  over  and  through  it.  The  vessels  are  decidedly 
dark,  but  lose  their  central  light  streak,  and  distinction  between 
veins  and  arteries  is  difficult.  In  general  oedema  the  retina  is  infil- 
trated in   all   its  layers.      (Plate  IV.,  A  and  E,  and  V.,  A  and  B.) 


PLATE   IV 


E  F 

Inflammations  of  the  Retina.— yl.  CEdema  in  Pernicious  Ansemia  (Oliver) ;  B.  Leuksemic  Retinitis 
(Oliver) ;  C.  Albuminuric  Retinitis  and  Neuritis  occurring  in  Pregnancy  (H.  V.  W.)  ;  D.  Albuminuric 
Retinitis  in  the  Xegro  (H.  V.  W.)  ;  E.  Syphilitic  Retinitis  (Haab) ;  F.  Atrophy  of  Retina,  Nerve  and 
Chorioid  following  Disseminated  Chorioretinitis  Luetica  (Oeller). 


DISEASES   OF   THE   RETINA  367 

Hemorrhages  and  exudation  may  appear  in  any  part  of  the  retina. 
(Edema  is  met  with  in  syphilitic  retinitis;  it  may  be  a  forerunner  of 
retinal  detachment,  of  tumor  of  the  retina  or  chorioid ;  it  may  be  occa- 
sioned by  traumatism,  or  may  arise  from  exposure  to  bright  light  or 
electric  flashes.    Qildema  of  the  disk  occurs  in  the  first  stage  of  neuritis. 

Inflammation  of  the  Vessel  Walls. — Vasculitis  and  Perivasculitis 

These  conditions  are,  as  a  rule,  due  to  arteriosclerosis  or  syphilis,  or 
occur  in  degenerative  diseases  of  the  cerebral  cortex,  and  are  attended 
by  other  lesions  in  the  optic  nerve  and  retina  as  well  as  the  general 
system.      (Plate  V.)      (See  Chapter  XL) 

Perivasculitis  is  an  inflammation  of  the  external  layers  and  fibrous 
tissues  surrounding  the  blood-vessels.  It  is  most  commonly  met  with 
in  retinitis,  but  may  exist  separately.  The  blood-vessels  are  swollen  in 
places,  but  are  eventually  replaced  by  fibrous  cords.     (Plate  V.,  F.) 

Angeoid  streaks  are  cicatricial  changes  in  the  retina,  usually  the 
remains  of  hemorrhages,  though  they  may  mark  a  previous  detachment 
of  the  retina.  They  course  from  the  disk  to  the  periphery,  are  whitish 
in  the  centre,  and  are  bordered  by  black  lines. 

Hemorrhages  from  the  Vessels. — Apoplexy  of  the  retina  occurs  most 
commonly  in  elderly  people  with  degenerated  arteries.  (Plate  V.,  E.) 
It  is  met  with  in  cardiac  hypertrophy  from  valvular  disease,  ulcerative 
endocarditis,  chronic  neuritis,  gouty  and  uric  acid  diathesis,  fatty  and 
dilated  heart ;  in  scurvy,  haemophilia,  purpura,  and  grave  aniemias, 
malarial  fever,  pyaemia,  leukaemia,  suddenly  suppressed  menstruation, 
and  the  menopause. 

Retinal  hemorrhages  may  occur  with  or  without  true  inflammation  of  * 
the  membrane.  The  extravasation  may  be  small  and  confined  to  the 
retina,  or  may  be  large  enough  to  fill  the  vitreous.  Rupture  of  the 
vessels  near  the  macula  or  extensive  bleeding  into  the  structure  of  the 
retina  seriously  interferes  with  vision,  but  many  small  extravasations, 
especially  those  upon  the  nasal  side  or  in  the  periphery,  may  not  be 
noticed  by  the  patient.  Hemorrhages  which  occur  in  the  nerve-fibre 
layer  of  the  retina  are  flame-shaped,  while  those  into  the  deeper  layers 
are  round  or  oval,  fresh  ones  appearing  as  dark  red  blotches ;  small 
hemorrhages  are  rapidly  absorbed,  leaving  whitish  patches  surrounded 
by  pigment,  or  a  simple  mass  of  pigment.  If  bleeding  occurs  into  the 
vitreous,  whitish  streaks  may  remain,  which  have  been  given  the  name 
of  retinitis  proliferans.  The  distinction  between  simple  hemorrhage 
into  the  retina  and  retinitis  with  hemorrhages  cannot  be  definitely 
drawn.  Most  forms  of  retinitis  are  attended  with  effusion  of  blood 
either  from  diapedesis  or  rupture  of  the  vessel  wall.     Under  such  con- 


368  THE    EYE    AND    NERVOUS    SYSTEM 

ditions  the  ophthalmoscope  shows  numerous  small  extravasations,  espe- 
cially marked  at  the  posterior  pole,  cedema  of  the  nerve-head,  venous 
dilatation  and  arterial  contraction,  patches  of  degeneration,  and  pig- 
ment changes.  In  apoplexy  of  the  retina,  extravasation  takes  place 
without  apparent  preceding  inflammation.  This  causes  destruction  of 
the  retinal  elements,  and  induces  inflammation  in  their  immediate 
neighborhood. 

Hemorrhages  into  the  optic  nerve  unaccompanied  by  retinal  extrava- 
sations are  rare.  They  appear  as  irregularly  outlined  dark  red  clots, 
and  when  unaccompanied  by  neuritis  are  indicative  of  arteriosclerosis. 

Prognosis. — Hemorrhage  into  the  retina  is  a  grave  affection,  the 
restoration  of  vision  depending  upon  the  situation  of  the  apoplexy  and 
degree  of  destruction  of  the  retinal  elements.  Complete  restoration  of 
the  function  of  rods  and  cones  does  not  occur.  Hemorrhage  into  the 
retina  is  a  forerunner  of  cerebral  apoplexy,  a  symptom  of  brain  tumor, 
heart  or  kidney  disease,  diabetes,  and  blood  affections.  Where  found, 
a  thorough  investigation  of  the  vascular  system  and  abdominal  organs 
should  be  made. 

Treatment. — Although  the  resorption  of  the  blood  is  probably  as 
active  without  any  treatment  as  with,  yet  rest  of  the  body  and  eyes 
prevents  further  bleeding.  Iodide  of  potash  and  soda  or  mercurials 
should  be  administered  to  produce  more  rapid  resorption  of  the  blood 
elements.  Dionin  locally  in  5  to  10  per  cent,  solutions,  or  subconjunc- 
tivally,  or  in  powder,  is  an  active  lymphagogue  and  aids  resorption. 

Embolism  of  the  Central  Artery. — The  main  stem  of  the  artery 
may  be  occluded  in  the  optic  nerve,  or  the  embolus  may  lodge  in  one 
of  the  branches.  The  course  of  the  blood  is  thereby  interrupted  and 
the  retina  being  deprived  of  its  supply,  loses  its  function.  Should  the 
clot  occupy  the  entire  calibre  of  the  artery,  total  and  sudden  blindness 
follows;  should  it  be  small  enough  to  pass  into  a  retinal  branch,  the 
blindness  will  correspond  to  that  part  of  the  retina  supplied  by  the 
affected  vessel  (Plate  V.,  A  and  B) ;  should  the  clot  only  partially 
block  the  lumen  of  the  vessel,  loss  of  vision  is  at  first  partial,  but  gener- 
ally becomes  complete.  In  such  cases  recurrent  attacks  of  temporary 
blindness  are  liable  to  occur,  usually  followed  by  an  atack  in  which 
tliere  is  permanent  blindness.  A  portion  of  the  retina  may  receive  its 
blood-supply  from  a  cilioretinal  vessel  and  thus  retain  its  function  in 
a  small  area  of  the  field.    Embolism  is  usually  monocular. 

Causes. — This  condition  is  indicative  of  cardiac  or  vascular  disease, 
ulcerative  endocarditis,  valvular  disease,  particularly  atheroma  of  the 
arteries.     In  a  small  proportion  of  cases  the  sudden  loss  of  sight  in 


PLATE  V. 


A.  Partial  embolism  ;  superior  branch  retinal  artery  (Haab)  ;  B.  Total  embolism  central  artery  in 
nerve ;  a  cilio-retinal  vessel  supplies  a  small  area  of  the  retina  in  which  function  is  preserved  (H.  V.  W.) ; 
C.  Thrombosis  of  central  vessels  from  mumps  (H.  V.  W.) ;  D.  Results  of  thrombosis  of  central  vessels; 
same  case  6  months  later  (H.  V.  W.) ;  E.  Hemorrhages  from  retinal  vessels ;  apoplexy  of  retina  (Magnus) ; 
F.  Perivasculitis  luetics  (Magnus). 


DISEASES   OF   THE   KETIXA  369 

chronic  nephritis  is  due  to  this  cause.  Syphilis  and  degeneration  of 
the  arteries  may  be  suspected.  Embolism  has  been  said  to  occur  in 
chorea. 

Ophthalmoscopic  Appearances. — These  are  characteristic ;  in  complete 
occlusion  the  arteries  carry  no  blood,  and  -the  intima  only  is  visible ; 
the  veins  and  papilla  are  pale  and  the  retina  oedematous ;  sometimes 
small  hemorrhages  are  found.  A  blood-red  cherry  spot  appears  in  the 
fovea,  resembling  a  small  round  hemorrhage.  As  a  consequence  of  the 
ischaemia  atrophy  of  the  retinal  elements  and  of  the  optic  nerve  ensue. 

Sjmiptoms. — In  some  cases  the  ultimate  blindness  is  preceded  by 
occasional  transitory  attacks  of  loss  of  sight.  In  complete  obstruction 
of  the  central  artery  the  loss  of  vision  is  immediate  and  entire,  and 
in  most  cases  permanent.  In  a  few  instances  the  clot  has  been  dislodged 
and  broken  up  and  absorbed  by  treatment,  securing  restoration  of  func- 
tion. In  others  where  a  part  of  the  retina  regained  its  function  by 
an  anastomosis  through  the  choroidal  vessels,  or  where  cilioretinal  ves- 
sels existed,  a  certain  degree  of  restoration  may  occur. 

Diagnosis. — The  sudden  monocular  blindness,  the  cherry-red  spot 
at  the  fovea,  the  diffused  retinal  oedema,  absence  of  pain  and  inflamma- 
tion, render  the  diagnosis  positive. 

Prognosis. — If  the  patient  is  seen  within  a  few  hours  or  days  after 
the  attack,  treatment  may  restore  vision,  though  if  this  does  not  occur 
within  a  short  period,  the  retina  and  nerve  will  atrophy  and  the  blind- 
ness persist. 

Treatment. — If  the  case  be  seen  within  the  first  few  days,  deep  mas- 
sage through  the  closed  lids  by  the  thumb  or  by  a  mechanical  masseur, 
or  by  forcibly  pressing  the  eye  back  in  the  orbit  and  holding  it  there 
for  a  few  seconds,  may  break  up  or  dislodge  the  embolus;  this  con- 
joined with  subsequent  treatment  by  large  doses  of  iodide  of  potash, 
saline  cathartics  and  pilocarpine  sweats  has  succeeded  in  a  number  of 
reported  instances  in  securing  partial  or  complete  recovery. 

Thrombosis  of  the  Central  Vein. — Ophthalmoscopic  Appearances. — 
In  this  condition  there  is  an  unusual  dilatation  and  tortuosity  of  the 
veins,  the  latter  appearing  like  sausages  (Plate  V.,  C),  while  the 
arteries  are  contracted  to  narrow  white  lines.  There  are  numerous 
hemorrhages  and  intense  papillitis.  After  a  few  weeks  the  hypersemia 
lessens,  but  atrophy  of  the  retina  and  nerve  ensues.    (Plate  Y.,  D.) 

Symptoms  and  Causes. — Vision,  although  seriously  affected  from  the 
first,  may  not  be  entirely  absent,  though  eventually  it  is  lost    The  occlu- 
sion of  the  vein  is  due  to  inflammation  following  phlebitis  or  endarteritis 
usually  as  a  consequence  of  microbic  infection  (Fig.  10).    It  may  occur 
24 


370  THE    EYE    AND    NERVOUS    SYSTEM 

from  erysipelas,  abscess  of  the  orbit,  purulent  meningitis,  mumps,  or 
infectious  blood-diseases.     If  the  phlebitis  has  its  site  in  a  small  vein. 


Fiu.  10.  —Section  through  optic  nerve  entrance  in  a  case  of  tlirombosia  of  the  central  retinal  vela 

occorring  in  mumps. 

the  signs  are  confined  to  the  corresponding  portions  of  the  retina.  It 
is  usually  limited  to  one  eye,  but  cases  have  been  reported  as  occurring 
in  both. 

(3)  INFLAMMATION    OF    THE   RETINA. 

Retinitis  may  occur  alone,  but  is  usually  associated  with  some 
change  in  the  optic  nerve  producing  neuroretinitis,  with  ultimate  atro- 
phy. Inflammation  of  the  retina  is  announced  subjectively  by  disturb- 
ance of  vision,  particularly  of  the  central  acuity,  contraction  or  limita- 
tion of  the  field,  scotomata,  and  other  field  disturbances.  Ophthalmo- 
scopically  there  may  be  oedema,  opacity,  hemorrhage,  exudation,  pig- 
ment changes,  and  alterations  of  the  vessels  of  the  nerve-head  and 
retina.      (Plates  IV.  and  V.) 

Visual  Disturbances. — The  sensibility  of  the  retina  may  be  increased 
or  diminished ;  in  the  former  instance  there  is  hypenusthesia,  the  retinal 
elements  being  so  sensitive  that  subjects  see  best  in  subdued  light  (7iyc- 
talopia).  In  neurasthenia  the  retina  is  sometimes  acutely  sensitive, 
objects  being  perceived  during  both  day  and  night  that  are  not  ordinarily 
visible  to  healthy  eyes. 

Day-Blindness. — The  sensibility  of  the  retina  may  be  so  decreased 
by  long  exposure,  as  in  snow-blindness,  that  the  retina  cannot  perceive 
objects  illuminated  by  a  moderate  amount  of  light.  Such  disturbances 
may  occur  also  from  nutritional  changes,  as  is  evinced  in  retinitis  pig- 
mentosa and  in  hemcralopia  (night-blindness). 


DISEASES  OF  THE  RETINA  371 

Photopsia  is  due  to  irritation  of  the  retinal  cells  and  nerve-fibres, 
and  is  an  early  symptom  of  retinal  disease.  Sparks,  flashes  of  light  and 
color,  which  are  more  pronounced  when  the  eyes  are  closed  or  in  the 
dark,  portend  serious  disease  of  the  eye,  such  symptoms  being  commonly 
met  with  in  the  early  stages  of  detachment  of  the  retina.  They  may 
be  occasioned  by  transient  disturbances  of  the  circulation  from  con- 
stitutional affections  and  from  the  exhibition  of  certain  drugs,  such 
as  santonin,  etc. 

Metamorphopsia  is  the  term  applied  to  distortion  of  an  object  as  a 
result  of  partial  destruction  of  the  rods  and  cones  or  obstruction  by 
retinal  pigment  or  hemorrhage.  Patients  with  this  symptom  complain 
of  overlapping  of  words  and  letters  and  of  straight  lines  appearing 
curved  or  broken. 

Vakieties  of  Retinitis  Associated  With  Genee4L 
AND  Nekvous  Affections. 

Retinitis  pigmentosa  is  a  gradual  degeneration  of  the  nerve-cells 
and  fibres  of  both  the  retina  and  optic  nerve  with  pigment  infiltration 
into  the  structure  of  the  retina.  It  is  primarily  a  disease  of  the  retina, 
and  in  some  rare  cases  the  pigment  infiltration  is  absent.  The  disease 
begins  in  early  life,  and  is  inherited  in  33  per  cent,  of  the  cases,  70 
per  cent  of  which  occur  in  males.  It  is  sometimes  complicated  by  other 
congenital  defects  of  development.  About  25  per  cent,  of  the  patients 
are  offspring  of  consanguineous  marriages.  In  these  families  syphilis 
has  been  found  in  piost  instances.  The  central  visual  acuity  is  preserved 
to  a  marked  extent,  though  peripheral  vision  is  usually  much  restricted 
or  lost.  The  ophthalmoscopic  appearances  are  characteristic.  The 
pigment  cells  of  the  retina  are  collected  into  masses,  resembling  bone 
corpuscles,  and  are  distributed  mainly  along  the  blood-vessels,  appear- 
ing first  near  the  equator,  and  are  usually  more  thickly  distributed 
at  this  point.  The  arteries  are  diminished  in  calibre  and  appear 
as  whitish  lines;  the  veins  later  undergo  similar  changes.  The 
retina  finally  atrophies  and  the  optic  nerve  becomes  white  and  very 
much  shrunken. 

Symptoms. — The  earliest  symptom  is  night-blindness  (hemeralopia) , 
the  visual  acuity  being  greatly  lowered  by  diminished  illumination,  due 
to  anaesthesia  of  the  retina.  Ring  scotoma  are  sometimes  present,  the 
visual  field  is  greatly  concentrically  contracted,  the  central  vision  re- 
maining moderately  acute.  Xystagmus  is  generally  present.  The 
progress  of  the  affection  is  slow,  and  the  patients  often  die  of  inter- 
current disease  before  vision  is  totally  lost. 


372  THE    EYE    A:N"D    :N"ERV0US    SYSTEM 

Treatment. — Xo  restoration  of  vision  can  be  secured.  Strychnine 
injections  and  galvanism,  and  lately  the  high  frequency  faradic  cur- 
rent, are  advocated.  Nerve  tonics,  good  diet,  open-air  life,  and  iodide 
of  potash  are  advised. 

Syphilitic  retinitis  is  usually  secondary  to  chorioiditis,  but  the  acute 
manifestation  may  first  appear  in  the  retina. 

Ophthalmoscopic  Appearances. — (Edema  of  the  retina,  especially  near 
the  macula,  is  first  observed.  (Plate  IV.,  E.)  This  is  followed  by 
serous  infiltration,  the  vessels  become  tortuous,  and  are  partially  con- 
cealed by  the  exudation.  The  retina  usually  retains  its  normal  appear- 
ance towards  the  periphery,  although  isolated  patches  of  retinitis  may 
occur  some  distance  from  the  papilla.  The  swelling  may  reach  a  milli- 
metre or  more,  is  whitish  in  color,  and  free  from  blood-vessels.  The 
arteries  are  accompanied  by  white  lines,  indicating  sclerosis  of  the 
walls  and  diminished  calibre.  The  posterior  part  of  the  vitreous  is 
infiltrated  with  a  fine  dust-like  opacity.  These  particles  of  opacity  are 
separate  and  distinct  from  one  another,  but  may  later  form  a  membrane, 
due  to  their  coalescence  and  incomplete  absorption.  Hemorrhages  and 
pigment  degeneration  only  occur  after  absorption  of  the  oedema  and 
the  retina  has  been  partially  destroyed.  A  concomitant  disseminated 
chorioiditis  is  likewise  to  be  observed  at  this  time.     (Plate  IV.,  F.) 

Pathology. — As  a  rule,  the  retinitis  is  secondary  to  primary  inflam- 
mation of  the  chorioid,  although  ophthalmoscopic  examination  may  at 
first  reveal  no  symptom  of  the  latter  disease.  The  first  changes  are  a 
vasculitis,  consisting  of  proliferation  of  the  endothelium,  infiltration 
of  the  walls  of  the  vessels  passing  later  into  sclerosis.  Subsequently 
infiltration  of  the  retina  from  pigmentary  migration  occurs  which 
extends  mainly  along  the  course  of  the  vessels.  Eventually  both  cho- 
rioid and  retina  become  atrophied  and  degenerated,  and  are  united  in 
places  by  cicatricial  changes. 

Prognosis. — As  syphilis  is  a  curable  disease  and  readily  amenable 
to  treatment,  the  destructive  processes  in  the  retinal  and  chorioidal  ele- 
ments are  favorably  influenced  by  prompt  antisyphilitic  treatment. 
Impairment  of  vision  is  to  be  expected,  however,  in  all  cases,  as  some 
degree  of  atrophy,  not  only  of  the  retina  and  chorioid,  but  of  the  optic 
elements,  always  results. 

Sjrmptoms. — Central  vision  is  diminished  by  the  vitreous  opacities 
and  retinal  oedema,  and  may  be  reduced  even  to  the  perception  of  light, 
though  ultimate  partial  recovery  is  attained.  The  field  may  be  con- 
tracted, and  there  are  usually  central  and  disseminated  scotomata.  Pho- 
topsia  and  metamorphopsia  are  also  present.     There  is  little  pain  even 


DISEASES   OF   THE  RETIXA  373 

when  iritis  is  present,  which  is  a  not  infrequent  complication,  as  the 
inflammation  in  the  iris  is  usually  mild  and  insidious  and  may  not  be 
recognized  until  after  the  formation  of  synechiae.  Though  the  disease 
is  usually  accompanied  by  a  true  uveitis,  there  is  but  little  photophobia 
and  but  few  signs  of  external  irritation. 

Treatment. — Energetic  antisyphilitic  treatment  by  mercurial  inunc- 
tions should  be  pushed  to  mild  ptyalism ;  pilocarpine  should  be  injected 
hypodermically  and  followed  by  hot  baths ;  iodide  of  potash  should  be 
administered  in  increasing  and  large  doses,  together  with  the  application 
of  hot  compresses  to  the  eyes.  By  these  measures  the  disease  may  be 
shortened,  relapses  prevented,  and  the  sight  preserved. 

The  recurrence  of  retinitis  in  syphilis  indicates  a  tertiary  form  of 
the  disease  and  is  an  evidence  of  severe  infection. 

Albuminuric  Retinitis. — Very  rarely  in  acute  inflammation  of  the 
kidney,  but  in  about  10  per  cent,  of  persons  with  chronic  nephritis, 
patliologic  degeneration  of  the  retina  and  optic  nerve,  and  with  this 
changes  in  the  pia  mater  and  other  brain  structures  arises.  These  in- 
flammations of  the  retina,  optic  nerve,  and  meninges  are  due  to  retention 
in  the  blood  of  toxic  elements  consequent  upon  the  disturbed  functions 
of  the  kidney  and  impairment  from  deficient  nutrition.  Transient 
cerebral  oedema,  with  increased  vascular  tension,  which  is  dependent 
upon  uraemia,  is  found  in  the  late  stages  of  nephritis  and  in  kidney 
disease  due  to  pregnancy.  The  latter  conditions  are  characterized  by 
complete  and  sudden  loss  of  vision,  but  are  unattended  by  retinal  or 
optic  nerve  changes,  whereas  in  the  former  characteristic  nerve  and 
retinal  lesions  are  developed. 

Ophthalmoscopic  Appearances. — These  are  so  characteristic  that  they 
should  not  be  confused  with  changes  other  than  those  due  to  extensive 
oedema  of  the  retina  and  optic  nerve.  In  the  early  stages  of  albuminuric 
retinitis  there  is  oedema  and  hyperemia  of  the  retina,  the  edges  of  the 
nerve  are  blurred  and  small  hemorrhages  occur  into  the  retina.  In 
the  macular  region  there  are  usually  small,  round,  or  oval  glistening 
white  dots  and  streaks,  which  slowly  increase  in  size  until  they  form  a 
characteristic  star-shaped  figure.  When  this  appears,  the  veins  become- 
tortuous,  the  optic  disk  is  more  swollen,  and  more  hemorrhages  occur; 
then  irregularly  shaped  isolated  patches  appear  in  places,  which  later 
coalesce,  showing  marked  degeneration  of  the  retinal  elements.  The 
hemorrhages,  depending  upon  their  location  in  the  retina,  are  either 
round,  when  they  are  situated  in  the  deeper  layers,  or  flame-shaped, 
when  they  occur  into  the  nerve-fibre  layer.  The  whitish  patches  and 
streaks  are  sho-v\Ti  by  the  microscope  to  be  due  to  fatty  degeneration  of 


374  THE    EYE    AND    NERVOUS    SYSTEM 

the  nerve-fibres,  which  later  become  hypertrophic  and  oedeniatous.  The 
inflannnation  of  the  retina  gradually  disappears  after  a  few  months 
or  a  year,  and  atrophy  of  the  retina  and  optic  nerve  ensues.  The  dis- 
ease is  almost  always  binocular,  but  the  ophthalmoscopic  appearances 
vary  in  the  two  eyes,  depending  upon  the  amount  of  hemorrhage  and 
fatty  degeneration  and  the  grade  of  the  optic  neuritis.  (Plato  IV., 
C  and  D. )     It  occurs  only  in  adults  and  is  most  common  in  women. 

Diagnosis. — The  radiating  macular  figure  is  almost  characteristic, 
although  a  very  similar  condition  is  seen  at  times  in  papillitis  occur- 
ring in  the  course  of  tumor  of  the  brain.  Examination  of  the  urine 
is  necessary  to  establish  the  diagnosis. 

Prognosis. — The  occurrence  of  retinitis  albuminurica  is  exceedingly 
grave;  the  duration  of  life  in  hospital  patients  averaging  eighteen 
months,  though  in  well  cared  for  private  patients  it  is  considerably 
longer.  The  retinitis  of  pregnancy  and  acute  nephritis,  especially 
in  young  persons,  is  not  so  unfavorable.  Induced  premature  delivery 
of  the  foetus  is  usually  followed  by  subsidence  of  the  nephritis  and 
a  certain  degree  of  recovery  of  vision,  although  some  atrophy  of  the 
nerve  with  loss  of  central  acuity  and  contraction  of  the  visual  field  takes 
place,  depending  upon  the  amount  of  actual  degeneration  in  the  retina. 
It  is  said  that  this  form  of  retinitis  is  usually  of  the  serous  rather  than 
of  the  exudative  type. 

Treatment. — Treatment  is  that  of  the  nephritis,  or  removal  of  the 
cause,  as  in  induced  abortion  for  the  retinitis  of  pregnancy.  Local 
remedies,  with  exception  of  smoked  glasses  and  non-use  of  the  eyes,  are 
of  no  effect. 

Diabetic  Retinitis. — The  fundus  changes  resemble  those  of  albu- 
minuric nephritis,  although  the  degeneration  is  not  so  marked  about 
the  macula  and  the  star-shaped  figure  is  not  usually  present.  Hemor- 
rhages are  more  common ;  the  optic  nerve  is  moderately  swollen ;  the 
arteries  contracted ;  the  veins  dilated  and  tortuous.  Small  white  patches 
of  degeneration  are  usually  found  about  the  fovea,  but  may  occur  any- 
where in  the  fundus.  Retinitis  in  diabetes  is  a  comparatively  rare 
complication,  being  much  less  common  tlian  cataract,  iritis,  other  forms 
of  uveitis  and  opacities  of  the  vitreous. 

Anaemic  Retinitis. — Changes  in  ansemia  are  usually  flmited  to  local 
anaemia,  but  in  leucocythsemia  and  pernicious  anaemia  there  are  pro- 
nounced alterations.  The  arteries  may  be  contracted,  the  veins  dilated 
and  tortuous,  and  hemorrhages  and  oedema  of  the  retina,  with  a  few 
white  patches  of  exudation  with  edges  colored  from  extravasations  of 
blood,  and  slight  optic  neuritis,  may  be  present.     (Plate  IV.,  B.) 


DISEASES  OF  THE  OPTIC  NERVE  3T5 

(4)  INFLAMMATION    OF    THE    OPTIC    NERVE. 

Optic  Neuritis  (Papillitis,  Choked  Disk). — The  most  pronounced 
and  important  eye  symptoms  of  certain  general  disorders  and  many 
forms  of  disease  of  the  nervous  system  manifest  themselves  in  an  in- 
flammation of  the  optic  nerve  and  an  associated  retinitis  in  which  fluid 
and  solid  exudation  fills  up  the  interstices  of  the  fibres  of  the  nerve, 
and  even  the  physiologic  excavation.  The  optic  nerve  elements  are 
swollen,  crowded  together  and  distorted,  thus  elevating  the  papilla  above 
the  level  of  the  surrounding  retina,  and,  by  pressing  upon  the  ves- 
sels, partly  obliterate  and  conceal  them.  The  veins  become  occluded, 
giving  rise  to  numerous  hemorrhages.  If  this  condition  prevails  for 
any  length  of  time,  atrophy  of  the  nerve  elements  and  the  connective 
tissue  takes  place. 

Optic  neuritis  may  be  differentiated  into  three  classes:  (IJ  medul- 
lary or  ascending  neuritis,  or  true  inflammation  of  the  nerve-tissue; 
(2)  papillitis,  or  simple  swelling  of  the  intra-ocular  portion  of  the 
nerve  from  irritation  or  pressure;  (3)  descending  neuritis,  originating 
from  intracranial  diseases,  affecting  the  nerve  or  its  sheaths. 

Ascending  neuritis  commences  in  the  papilla  and  usually  terminates 
in  the  brain.  Descending  neuritis  commences  in  the  brain,  later  finding 
its  way  down  the  nerve-sheath  or  nerve  elements  into  the  eyeball.  Choked 
disk  is  an  arbitrary  expression,  denoting  swelling  of  the  papilla  from 
2  D  to  6  D,  or  even  more.  (The  difference  between  the  highest  point 
on  the  disk  and  that  of  the  surrounding  retina  in  diopters  may  likewise 
be  expressed  in  millimetres,  2^  D  being  equivalent  to  1  millimetre  of 
SW' elling) . 

Causes. — The  causes  of  optic  neuritis  are  ocular,  orbital,  intracranial, 
and  constitutional. 

Ocular  causes  include  chorioiditis  and  retinitis,  which  are,  however, 
usually  dependent  upon  some  constitutional  cause. 

Orbital. — Fracture  of  the  orbit,  cellulitis  of  the  orbit,  hemorrhage 
into  the  cellular  tissue  of  the  orbit  or  nerve-sheaths,  deformities  of  the 
skull,  affections  of  the  neighboring  sinuses,  etc.,  may  be  enumerated 
under  this  heading. 

Intracranial. — Optic  neuritis  may  occur  in  the  course  of  diseases 
of  the  brain;  these  may  be  enumerated  in  order  of  frequency,  as  fol- 
lows :  meningitis,  tumors,  gummata,  sclerosis,  tubercle,  cysts,  and  aneu- 
rism. A  low  grade  of  retinitis  and  optic  neuritis  is  seen  in  some  cases 
of  epilepsy  and  general  paralysis.  In  children  probably  the  most  fre- 
quent cause  of  neuritis  is  tiibercular  meningitis.    Intracranial  affections 


376  THE    EYE    AND    NERVOUS    SYSTEM 

give  rise  to  optic  neuritis  by  direct  pressure  upon  the  chiasm,  the  optic 
nerves,  and  the  tracts;  or  by  occasioning  increase  of  the  ventricular 
and  subarachnoid  fluids  and  disturbing  the  equilibrium  of  the  different 
portions  of  the  brain ;  or  by  disturbances  of  nutrition  through  inter- 
ference with  the  circulation. 

The  precise  manner  in  which  intracranial  growths  occasion  optic 
neuritis  is  still  disputed.  Von  Graefe  was  of  the  opinion  that  the  neu- 
ritis was  due  to  a  strangulation  of  the  lymphatics  of  the  head  of  the 
nerve,  as  a  consequence  of  the  increase  in  intracranial  pressure,  occa- 
sioning a  blocking  of  the  cavernous  sinus  and  of  the  tributary  oph- 
thalmic vein.  This  theory  has  been  supported  by  Manz  and  Schmidt- 
Rimpler,  who  demonstrated  that  the  fluid  displaced  by  a  cerebral  tumor 
found  its  way  into  the  lymph-spaces  of  the  optic  nerve,  distending  the 
intersheath  spaces  with  fluid.  Leber  and  Deutschmann,  on  the  other 
hand,  believe  that  the  choked  disk  is  not  merely  a  stasis  but  claim  that 
the  presence  of  the  fluid  sets  up  a  toxic  condition  which  gives  rise  to  a 
true  inflammation  of  the  nerve.  Others  incline  to  the  view  that  the 
neuritis  is  a  true  descending  one,  as  they  have  shown  that  there  is  an 
increased  cellular  exudation  in  the  tissues  surrounding  the  cerebral 
tumor,  which  extends  the  whole  way  from  the  tumor  to  the  nerve  and 
along  the  latter  to  the  eye.  In  view  of  these  facts,  it  would  appear 
that  the  element  of  intracranial  and  intervaginal  pressure  plays  an 
important  part  in  the  production  of  choked  disk,  but  that  the  latter,  in 
the  majority  of  cases  at  least,  depends  upon  an  active  inflammation. 

Choked  disk  and  optic  neuritis  occur  in  about  90  per  cent,  of  cases 
of  brain  tumors,  irrespective  of  their  size  or  location.  The  absence  of 
optic  neuritis  does  not  exclude  the  diagnosis  of  brain  tumor,  but  its 
presence  is  a  strong  confirmatory  sign.  Tumors  of  the  occipital  region 
and  the  base  of  the  brain  are  apparently  more  often  accompanied  by 
choked  disk  than  those  in  other  portions  (Compare  Chapter  IX). 

Constitutional. — Optic  neuritis  sometimes  occurs  in  eruptive  fevers, 
diphtheria,  epidemic  influenza,  and  other  contagious  diseases;  it  may 
also  be  occasioned  by  albuminuria,  especially  of  the  puerperal  type, 
diabetes,  leucocythemia,  toxemia,  pernicious  anaemia,  acute  rheumatism 
after  exposure  to  cold,  and  menstrual  affections. 

It  is  believed  by  some  that  in  all  cases  of  optic  nerve  atrophy  there 
is  first  an  active,  although  evanescent,  primary  inflammation  of  the  optic 
nerve,  which  is  manifest  though  for  a  short  time  upon  the  optic  disk. 
In  spinal  cord  affections  a  slight  degree  of  neuritis  or  neuroretinitis  is 
a  forenmner  of  optic  atrophy.  Optic  neuritis  occurring  in  the  course 
of  syphilis  is  an  evidence  of  the  tertiary  stage  of  the  disease. 


PLATE   VI. 


Optic  Nerve  Changes  in  Brain  and  Spine  Diseases.—^.  Atrophy  following  infantile  cerebral 
disease  (Yellowish  Gray  Neuritic  Atrophy)  ;  B.  Atrophy  in  disease  of  the  optic  nerve  between  bulb  and 
chiasm  (Atrophy  of  Macuho  Fibres) ;  C.  Atrophy  in  disease  of  the  chiasm;  D.  Atrophy  in  tabes  (White 
Spinal  Atrojihy) :  E.  Optic  neuritis  in  basilar  meningitis:  F.  Choked  disk  in  tumor  of  the  cerebrum. 


DISEASES  OF  THE   OPTIC  XEEVE  377 

Ophthalmoscopic  Signs.- — The  inflammatory  process,  as  observed  by 
the  ophthalmoscope,  may  be  arbitrarily  divided  into  three  stages,  which 
pass  impercef)tibly  into  each  other. 

First  Stage. — The  border  of  the  papilla  is  blurred  by  infiltration 
or  swelling;  the  physiologic  excavation  is  filled  in  and  the  disk  red- 
dened, as  a  consequence  of  relative  increase  in  the  size  of  the  blood- 
vessels, whereby  fine  twigs  invisible  in  health  are  brought  into  view, 
and  the  veins  increased  in  size  and  tortuosity.  The  main  arteries  of 
the  disk  are  contracted,  the  connective  tissue  becomes  hypertrophied 
and  opaque  and  conceals  the  disk  outlines.  The  papilla  projects  for- 
ward, as  may  be  determined  by  focussing  the  blood-vessels  and  its 
surface.  (Plate  VI.,  E.)  During  this  stage  there  are  no  marked 
changes  in  the  retina  excepting  dilatation,  tortuosity  and  dark  color 
of  the  veins,  and  the  disease  may  not  advance  ophthalmoscopically 
beyond  this  condition. 

Second  Stage. — If  the  inflammatory  process  continues  in  intensity, 
these  ophthalmoscopic  signs  increase.  Then  the  swelling  of  the  nerve 
may  become  more  marked  and  the  disk  may  project  as  much  as  three 
millimetres  (7^  D)  into  the  vitreous.  (Plate  VI.,  F.)  Its  outlines 
are  hidden  by  exudation  and  hemorrhage,  and  jthe  swelling  over- 
lapping in  all  directions  apparently  doubles  the  size  of  the  papilla. 
Its  position  is  thus  only  determined  by  the  entrance  of  the  blood-vessels 
which  are  obscured  and  interrupted  in  places  and  difficult  to  follow. 
The  veins  are  enormously  dilated,  tortuous  and  dark  in  color,  appar- 
ently outnumbering  the  arteries,  which  are  proportionately  reduced  in 
number  and  calibre  and  carry  little  blood,  showing  in  places  as  simple 
white  lines.  Small  flame-shaped  hemorrhages  appear  near  the  disk. 
The  retina  in  the  neighborhood  is  (edematous,  and  its  fibre  layer  hyper- 
trophied, showing  radiating  white  lines.  This  stage  varies  in  duration 
from  a  few  weeks  to  several  months,  gradually  passing  into  the  stage 
of  degeneration. 

Third  Stage. — Absorption  of  the  blood  and  exudation  now  takes 
place,  the  swelling  of  the  nerve-head  declines,  the  periphery  contracts, 
the  exudative  material  is  absorbed  or  transformed  into  connective  tissue, 
the  veins  are  less  tortuous,  and  the  arteries  smaller.  The  papilla  be- 
comes a  dirty  grayish  white,  and  a  firm,  organized  exudation  fills  in 
the  physiologic  excavation,  completely  hiding  the  lamina.  The  nerve 
passes  ultimately  into  complete  atrophy.     (Plate  VL,  A.) 

Symptoms. —  Visual  Acuity.- — The  vision  is  usually  but  little  affected 
at  first,  and  thus  few  patients  are  seen  until  the  second  stage  ensues, 
when  the  vision  is  usually  somewhat  reduced,  the  reduction  becoming 


378 


THE    EYE    AND    NERVOUS    SYSTEM 


gradually  worse  during  the  continuance  of  the  disease.  Large  hemor- 
rhages in  the  region  of  the  macula  may,  however,  cause  more  or  less 
sudden  blindness.  Exceptionally  there  is  no  great  deterioration  of  vision 
until  the  third  stage,  and  thus  a  considerable  degree  of  papillitis  may 
exist  witli  normal  visual  acuity.  Hemorrhage  or  exudation  into  the 
macular  region  and  other  parts  of  the  retina,  permanently  diminish 
tlic  function  of  the  retinal  cells  and  fibres  by  pressure,  and  later  by 
cicatricial  tissue  formation  and  contraction.  As  a  rule,  vision  dimin- 
ishes to  light  perception  or  ultimate  blindness  in  the  cases  which  outlive 
the  ocular  affection. 

Visual  Field. — The  changes  in  the  visual  field  are  important  in 
localization   of  the  cerebral   lesion.      The   most   common   defects   are 


Left  105: 

120 


Fig.  11.— Enlarged  blind  spot  with  paracentral 


Fig.  12.— Pericentral  absolute  and  Ring  soo- 


absolute  and  relative  Bcotomata  in  optic  neuritis       toma  for  colors,  in  syphilis,  due  to  probable  gumma 
from  brain  tumor.  of  optic  nenre. 


peripheral  contraction,  enlarged  blind  spot,  or  central  scotomata.  The 
former  is  characteristic  of  uniform  pressure  upon  the  nerve  by  fluids 
or  solids  in  or  external  to  its  sheath,  while  the  latter  indicates  axial 
neuritis  with  loss  of  function  of  the  central  nerve-fibres.  Sector-like 
defects  involving  definite  portion  of  tlie  visual  field,  such  as  hemian- 
opsia, which  may  be, — i.e.,  homonymous  (nasal  or  temporal),  heteron- 
ymous (or  lateral)  tetranopsia  (loss  of  a  quarter  of  tlie  field),  and 
smaller  defects  occur,  which  are  symptoms  of  descending  optic  neuritis, 
due  to  lesions  of  the  chiasm,  tract  or  deep  centres.  These  are  usually 
binocular  and  symmetrical.  Monocular  defects  arise  from  pressure 
upon  the  nerve  in  the  orbital  canal  or  orbit,  or  upon  a  portion  of  the 
■chiasm  or  upon  small  areas  of  the  occipital  lobes. 

The  color  fields  diminish  more  rapidly  than  the  perception  for  white 


DISEASES   OF  THE   OPTIC  NERVE 


379 


and  form,  though  there  is  usually  peripheral  contraction  for  all  colors. 
As  the  disease  progresses,  green  is  first  lost,  then  red,  and  finally  blue. 
The  loss  of  the  blue  field  is  contemporaneous  with  marked  contraction 
of  the  form  field  and  indicates  advancing  atrophy.  As  the  scotomata 
and  sector-like  defects  in  the  visual  field,  which  occur  from  intra-ocular 
disease  secondary  to  changes  in  the  retina,  frequently  resemble  those 


L.E. 


Fig.  13.— Temporal  hemianopia  and  peripheral  contraction  from  tumor  of  optic  chiasm,  causing 

bilateral  choked  disk. 


from  changes  in  the  cerebral  centres,  it  is  necessary  to  rely  upon  oph- 
thalmoscopic examination  to  establish  the  diagnosis.  (See  Chapter  IV.) 
Prognosis. — Optic  neuritis  of  all  grades  is  grave,  because  the  intra- 
cranial and  constitutional  diseases  of  which  it  is  a  lesion  involve  essen- 
tial organs  of  the  body.  An  unfavorable  prognosis  as  to  sight  may 
usually  be  given,  with  the  exception  of  cases  of  toxic  amblyopia,  in 
which  full  recovery  often  occurs  after  removal  of  the  cause.  Atrophy 
invariably  follows  the  inflammation,  and  as  the  degeneration  of  the 
nerve  progresses,  vision  diminishes.  The  atrophy  generally  advances 
more  rapidly  in  one  nerve  than  the  other,  so  that  some  useful  vision 
may  be  preserved  in  one  eye  even  after  the  other  has  become  blind. 
As  regards  life,  the  occurrence  of  an  optic  neuritis  often  indicates 
grave  cerebral  involvement,  and  when  it  is  concomitant  with  retinitis 
or  advanced  renal  or  vascular  lesions,  its  occurrence  is  usually  prog- 
nostic of  a  lethal  ending  within  a  few  weeks  to  two  or  three  years,  at 
most,  depending  upon  the  gravity  of  the  central  or  constitutional  lesion. 


380  THE    EYE    AISTD    NERVOUS    SYSTEM 

Treatment. — The  treatment  depends  upon  the  cause,  for  which  a  com- 
plete physical  examination  is  essential.  Discovery  of  albumin,  casts, 
or  sugar  in  the  urine,  determines  the  line  of  treatment,  which,  as  a 
rule,  is  to  be  given  by  the  family  physician.  If  the  case  is  found  to 
be  of  cerebral  origin,  the  oculist  may  be  seen  only  in  consultation. 
Syphilitic  cases  should  be  combated  by  energetic  mercurial  inunctions, 
large  doses  of  iodide  of  potash,  pilocarpine  sweats.  The  rheumatic, 
albuminuric,  and  toxic  types  are  likewise  benefited  by  pilocarpine. 
Acute  forms,  where  exposure  to  cold  has  been  followed  by  neuritis  and 
loss  of  vision,  are  helped  by  blood-letting  and  diaphoresis.  Salicylate 
of  soda  in  doses  corresponding  to  the  weight  of  the  individual, — i.e., 
1  grain  for  each  pound  of  weight  a  day, — are  of  service.  Regulation  of 
the  menses  is  an  important  factor  in  women.  Ethmoid  and  sphenoid 
disease  should  be  searched  for  and  properly  treated. 

Acute  Retrobulbar  Neuritis. — The  optic  nerve-fibres  may  be  affected 
primarily  by  disease  posterior  to  the  eyeball  from  injury  or  disease 
of  the  orbit  and  of  the  accessory  cavities,  or  by  rheumatism,  inhalation 
or  absorption  of  toxic  substances,  such  as  methyl  alcohol  or  quinine, 
or  by  influenza,  acute  alcoholism,  and  autotoxaemia. 

Pathology. — The  function  of  the  nerve  is  rapidly  diminished  by 
interstitial  inflammation  of  the  nerve-fibres,  or  by  pressure  upon  the 
nerve  or  vessels.  In  some  cases  the  inflammation  subsides,  as  the  exuda- 
tion is  absorbed  or  the  pressure  removed.  In  others,  atrophy  ensues 
with  more  or  less  complete  loss  of  vision. 

Symptoms. — There  is  rapid  loss  of  visual  acuity,  perception  of  light 
in  some  cases  being  lost  in  a  few  hours  or  days.  The  visual  field  may 
be  peripherally  contracted,  but,  as  a  rule,  there  is  a  central  scotoma 
which  increases  in  size  until  only  a  small  rim  of  peripheral  field  re- 
mains. The  central  scotoma  is  at  first  only  for  color, — ^particularly  for 
red  and  green.  Partial  recovery  occurs,  followed  later  by  further  loss 
of  vision.  Weakness  or  paralysis  of  the  facial  or  other  nerves  may 
accompany  or  precede  the  optic  nerve  affection. 

Ophthalmoscopic  Appearances. — The  ophthalmoscope  shows  the  nerve- 
head  to  be  hyperamic  with  discolored  surface  and  obscured  edges;  the 
arteries  are  contracted,  and  the  veins  dilated,  and  in  rare  cases  there 
are  a  few  small  hemorrhages. 

Prognosis. — Recovery  of  useful  vision  follows  early  energetic  treat- 
ment, though  cases  usually  go  on  to  more  or  less  complete  blindness. 

Treatment. — Removal  of  the  cause  with  local  blood-letting,  large 
doses  of  salicylates  in  rheumatic  cases,  strychnine  hypodermically  in 
increasing  doses,  iodide  of  potassium,  and  mercurial  inunctions  are  indi- 


DISEASES  OF  THE   OPTIC  Is^ERVE  381 

cated.  PilocaqDine  sweats  eliminate  poison  and  assist  the  action  of  the 
internal  remedies. 

Chronic  Retrobulbar  Neuritis  (Toxic  Amblyopia). — The  optic 
nerve  may  degenerate  from  the  constant  absorption  of  poisons,  notably 
large  quantities  of  tobacco  or  alcohol,  or,  as  is  usually  the  case,  their 
combined  action.  ISTumerous  other  poisons, — i.e.,  lead,  quinine,  the 
methyl  compounds,  salicylic  acid,  napthalin,  nitrobenzol, — will  pro- 
duce a  similar  inflammation.  Toxic  amblyopia  may  also  arise  in  the 
course  of  some  constitutional  diseases,  such  as  diabetes,  and  one  of  the 
theories  of  choked  disk  is  that  it  is  developed  by  a  local  toxemia  arising 
in  the  brain. 

Pathology. — The  disease  consists  in  a  true  axial  medullary  neuritis, 
possibly  secondary  to  degeneration  of  the  macular  ganglion  cells,  al- 
though it  is  more  probable  that  it  arises  in  the  bundle  of  the  nerve-fibres 


Right 


Fig.  14.— Toxic  amblyopia ;  small  absolute  central  scotoma ;  larger  central  scotoma  for  red 
and  green ;  contracted  field. 


supplying  the  macular  region.  It  is  a  gradual  degenerative  process  and 
which,  unless  checked,  encroaches  uj)on  the  other  fibres  adjoining  the 
papillomacular  bundle  until  the  entire  nerve  becomes  atrophic. 

Symptoms. — The  impairment  of  vision  is  gradual,  and  consists  in 
blurring  of  sight,  which  is  not  relieved  by  glasses.  The  pupillary  reflex 
may  be  disorganized  so  that  the  pupil  dilates  with  the  accommodation. 
The  visual  acuity  varies  from  slight  derangement  to  entire  loss  of  cen- 
tral acuity,  depending  upon  the  amount  of  central  scotoma.  As  a  rule, 
there  is  more  disturbance  for  color  than  for  form,  the  characteristic 
signs  of  the  disease  being  central  scotoma  for  colors,  more  particularly 
for  green  and  red.     Later  in  the  disease  the  scotoma,  which  was  at  first 


382  THE    EYE    AND    NERVOUS    SYSTEM 

relative,  liecoiues  absolute,  increases  iu  size,  and  finally  the  peripheral 
field  becomes  concentrically  contracted. 

Ophthalmoscopic  Appearances. — As  a  rule  nothing  distinctive  can  be 
seen  in  couimeneing  cases,  with  the  exception  that  the  outline  of  the 
nerve  is  not  so  sharp  and  clear  as  in  the  normal  subject  (Plate  V.,  B.), 
but  in  advanced  cases  the  nerve-head  is  usually  pale  in  the  lower  tem- 
poral quadrant, — i.e.,  in  the  region  occupied  by  the  macular  fibres. 

Prognosis. — Prompt  treatment  preserves  the  visual  acuity,  and  in 
favorable  cases  vision  may  be  entirely  restored,  the  amount  depending 
upon  the  degree  of  destruction  of  the  nerve-fibres. 

Treatment. — Removal  of  the  cause.  If  this  be  alcohol  or  tobacco  or 
both,  the  patient  may  have  to  receive  special  general  treatment,  which 
can  usually  only  be  properly  carried  out  away  from  his  usual  surround- 
ings, in  a  sanitarium  or  hospital.  Increasing  doses  of  strychnine,  hypo- 
dermically,  for  courses  of  several  weeks,  with  intermissions  of  a  week 
or  two,  may  restore  much  of  the  lost  vision.  Nitroglycerin  and  nitrite 
of  amyl  may  be  used.  If  there  be  exudation  into  the  nerve,  the  iodides 
are  of  value.  In  anaemia,  the  employment  of  iron  and  other  tonics, 
nourishing  diet,  exercise  out  of  doors,  early  hours,  and  plenty  of  sleep, 
are  necessary.  Galvanic  electricity  and  the  high  frequency  faradic  cur- 
rent seem  to  stimulate  the  retinal  elements  to  renewed  activity. 

(5)  OPTIC   NERVE    ATROPHY. 

Atrophy  of  the  optic  nerve  may  be  primary  or  secondary,  the  typea 
being  readily  distinguished  by  the  appearance  of  the  optic  disk. 

Primary  Atrophy. — Atrophy  not  preceded  by  pronounced  papillitis 
or  neuroretinitis  is,  in  the  majority  of  cases,  a  symptom  of  spinal  cord 
disease,  usually  of  sclerosis  of  the  posterior  columns  (locomotor  ataxia). 
It  occurs  less  frequently  in  disseminated  sclerosis,  lateral  sclerosis,  epi- 
lepsy, and  general  paralysis.  Toxic  amblyopia,  both  acute  and  chronic, 
the  specific  fevers,  diabetes,  and  arteriosclerosis  also  lead  to  atrophy  of 
the  medullary  fibres.  There  is  likewise  an  hereditary  form  following- 
retrobulbar  neuritis.  It  is  a  mooted  question  whether  or  not  oedema 
of  the  nerve  is  not  always  present  and  capable  of  being  observed  oph- 
thalmoscopically  in  the  early  stages.  Where  it  is  concomitant  with 
spinal  disease  the  structural  changes  in  the  optic  nerve  may  apparently 
precede  the  spinal  symptoms  for  months  or  years,  and  thus  the  dimin- 
ishing of  the  visual  function  may  be  the  first  symptom  of  the  general 
affection.  (See  Chapter  XTI.) 

Anatomical  Changes  in  Primary  Nerve  Atrophy. — Ophthalmoscopic- 
Appearances. — The  papilla  gradually  assumes  a  chalky-white  appear- 


Fig.  15.— Sections  through  the  optic  nen-e.     A,  normal  nerve ;  B,  papillitis ; 
C,  optic  atrophy.    (Reproduced  from  Elschnig.) 


DISEASES  OF  THE  OPTIC  NERVE 


383 


ance  or  it  may  acquire  a  bluish-green  tinge.  As  the  atrophy  progresses, 
contraction  of  the  nerve-fibre  bundles  and  their  surrounding  elastic 
tissue  takes  place,  causing  cupping  of  the  nerve.  The  cupping  is  grad- 
ual and  occupies  the  surface  of  the  nerve.  All  the  blood-vessels  become 
contracted  and  the  arteries  may  be  entirely  obliterated,  being  replaced 
by  white  streaks,  especially  when  the  disease  is  due  to  arteriosclerosis. 
This  process  causes  first  a  flattening  of  the  central  nerve-fibres  with 
circumscribed  atrophy  of  the  marginal  or  central  nerve-fibre  bundles, 
and  the  changes  in  the  papilla  are  always  preceded  by  failure  of  vision 
and  defects  in  the  visual  field.  Atrophy  of  the  .retina  follows  that  of 
the  nerve. 

Microscopic  Appearances. — This  is  not  an  ascending  atrophic  pro- 
cess, and  the  nerve  does  not  become  as  thin  and  attenuated  as  in  the 
neuritic  inflammation.  On  section,  the  septa  are  found  to  be  well  pre- 
served, and  the  fibres  transformed  into  fine  fibrillae  which  stain  with 
carmine.  The  blood-vessel  walls  are  sclerosed,  but  the  septa  only  become 
thickened  quite  late  in  the  disease.  Some  of  the  nerve-fibres  remain 
healthy,  and  cases  may  escape  the  sclerosis  entirely.  The  surface  fibres 
are  the  first  to  degenerate  and  this  may  show  in  the  visual  field  as 
peripheral  alterations.      (Fig.  15,  A.  B.  C.) 

Subjective  Signs. — The  acuity  of  vision  depends  upon  the  degree  of 
degeneration  of  the  nerve-fibres.     The  first  symptoms  are  diminution 


Eight         105°.    ^° 


Fig.  16.— Optic  nerve  atrophy  in  tabes  dorsalis. 


in  the  light  sense  and  contraction  in  the  color  fields;  the  patient  de- 
mands strong  light  and  has  difficulty  in  getting  about  in  the  dusk  or 
dark.  The  light  sense  may  be  tested  by  determining  the  acuity  of  vision 
in   different  degrees   of  illumination,   or  by  the   Forster   photometer. 


384 


THE    EYE    AND    NERVOUS    SYSTEM 


Usually  the  contraction  of  the  color  fields  is  greater  than  that  for  white, 
and  a  large  white  field  may  remain  when  the  color  vision  is  entirely 
gone  (Eig.  16). 

Prognosis. — The  disease  terminates  in  partial  or  complete  blindness. 

Treatment. — Treatment  is  that  of  the  constitutional  or  cerebral  dis- 
ease. Electricity  is  much  used,  but  is  of  little  avail.  Nitroglycerin 
and  nitrite  of  amyl  and  hypodermic  injections  of  strychnine  temporarily 
improve  vision.  Administration  of  the  iodides  of  mercury,  potash,  soda, 
and  strontium,  and  the  bromides  and  iodides  of  gold,  mercury,  and 
arsenic,  and  sweat  bafhs  are  recommended. 


Ji.E. 


^70"  -0 

Fig.  17.— Nasal  bemianopia.    (After  Veasey.) 


Secondary,  Ascending,  Consecutive,  or  Neuritic  Atrophy. — This 
condition  is  the  result  of  inflammation  of  the  orbital  end  of  the  optic 
nerve  following  retinitis,  neuroretinitis,  choked  disk,  or  chorioretinitis. 
The  inflammation  may  furthermore  be  induced  by  intracranial  lesions, 
tumors,  syphilis,  encephalitis,  meningitis,  or  distention  of  the  third 
ventricle  pressing  upon  the  chiasm,  or  may  result  from  retrobulbar 
neuritis,  injury,  oedema,  or  pressure  on  the  optic  nerve  at  the  optic 
foramen,  from  alcoholism,  from  the  manifestations  of  syphilis,  tuber- 
culosis, nephritis,  etc. 

Ophthalmoscopic  Appearances. — The  changes  in  the  optic  nerve  are 
somewhat  different  from  those  of  primary  or  spinal  atrophy.  Thus  the 
nerve-head  is  usually  of  a  dirty-gray  color,  is  not  cupped,  and  the  edges 
are  not  so  well  defined  as  those  of  spinal  atrophy.     (Plate  VI.,  A.) 


DISEASES  OF  THE  OPTIC  NEEVE 


385 


Microscopic  examination  demonstrates  that  tliis  is  only  a  strictly 
ascending  process  when  following  purely  intra-oeular  inflammations, 
as  chorioretinitis,  for  in  most  cases  anatomic  examination  shows  that 


Fig.  18.— Homonymous  hemianopia  (lateral)  with  external  ophthalmoplegia  occurring  in  disseminated 

sclerosis  with  nuclear  paralysis. 


choked  disk  or  optic  neuritis  involves  the  nerve  behind  the  globe,  even 
as  far  back  as  the  optic  chiasm  and  intracranial  divisions. 

The  subjective  signs  are  much  the  same  as  in  spinal  atrophy.  There 
is  a  loss  of  vision  and  contraction  of  the  visual  field,  the  various  changes 
in  the  field  depending  upon  the  intracranial  lesion  or  the  amount  of 
retina  that  has  deteriorated  as  a  result  of  the  intra-ocular  disease. 

Treatment. — Therapeutics  may  check  the  advance  of  the  atrophy  and 
postpone  the  inevitable  blindness,  as  has  been  noted  in  discussing  pri- 
mary atrophy. 

Hereditary  optic-nerve  atrophy  is  a  sufficiently  peculiar  form  to 
demand  separate  description.  Subjects  of  this  form  of  atrophy  are 
usually  affected  at  puberty  or  early  manhood.  As  a  rule,  their  skulls 
are  tower-shaped,  and  belong  to  the  so-called  scapho-  and  dolicho-cephalic 
varieties.  The  sphenoid  bone  is  not  fully  developed  until  rather  late 
in  life ;  it  contains  the  optic  foramen,  and  any  irregularity  in  its  growth 
may  cause  in  this  type  of  skull  a  lateral  pressure  which  occasions  the 
peculiar  ingrowing  visual  field  defect  which  invades  the  field  of  fixation 
and  ultimates  in  the  loss  of  central  vision.  (See  Chapter  XIV.) 

In  some  cases  the  central  scotoma  is  not  accompanied  by  marked 
25 


386 


THE    EYE    AND    NERVOUS    SYSTEM 


narrowing.  This  may  be  explained  by  the  fact  that  the  axial  fibres 
immediately  liehind  the  bulb  are  quite  lateral,  and  may  continue  so  to 
the  foramen. 

L.  E.  B.  E. 


Vm.  19.— Hereditary  optic-nerve  atrophy,  showing  development  of  central  absolute  and  relative 

scotoma  ta. 


Cases  of  this  disease  usually  occur  in  young  males  and  are  trans- 
mitted from  one  generation  to  another  through  the  females,  who  escape. 

Treatment  is  the  same  as  for  other  forms  of  atrophy,  but  is  practi- 
cally unavailing. 


CHAPTEE    IX. 

TUMORS  AND  OTHER  LESIONS  OF  THE  BRAIN,  ESPECIALLY 
THOSE  IN  RELATION  TO  THE  VISUAL  APPARATUS  AND 
OCULAR  NERVES. 

By  WILLIAM  G.  SPILLER,  M.  D. 

GENERAL  SYMPTOMS  OF  TUMOR. 

So  much  has  been  written  on  encephalic  tumors,  both  by  American 
and  foreign  writers,  that  it  has  seemed  well  in  this  chapter  to  pay  more 
attention  to  new  growths  that  are  of  special  interest  to  ophthalmologists. 
American  writers  have  made  notable  contributions  to  this  subject. 
Tumors  not  connected  with  the  visual  system  will  therefore  be  treated 
rather  briefly  in  this  chapter. 

Tumors  of  the  brain  may  cause  symptoms  that  are  not  indicative  of 
the  position  of  the  growth  but  merely  show  that  something  abnormal  is 
developing  within  the  cranial  cavity  producing  an  increase  of  intra- 
cranial pressure.  The  most  important  of  these  symptoms  are  headache, 
often  persistent  and  with  exacerbations,  nausea  and  vomiting,  general 
convulsions,  vertigo,  and  choked  disks.  They  do  not  imply  necessarily 
that  a  tumor  is  growing  within  the  brain,  and  they  may  be  caused  by 
meningitis,  abscess,  or  other  lesions.  There  are  in  most  cases  of  tumor 
certain  signs  and  symptoms  that  give  some  clue  to  the  location  of  the 
cerebral  growth ;  and  the  determination  of  these  is  of  great  importance. 
Choked  disk  in  itself  does  not  show  whether  a  tumor  is  in  the  cerebrum 
or  cerebellum.  Marcus  Gunn's  opinion  that  unilateral,  or  prepon- 
deratingly  unilateral  optic  neuritis  is  in  favor  of  the  tumor  being  on 
the  same  side  as  that  on  which  the  neuritis  is  more  intense  can  not  be 
accepted  for  all  cases.  Purves  Stewart,  for  example,  says  he  has  seen 
a  case  of  large  frontal  tumor  in  which  the  optic  neuritis  was  both  earlier 
and  more  intense  on  the  opposite  side  from  the  lesion.  This  is  an  inter- 
esting observation,  because  we  might  expect  that  if  unilateral  optic 
neuritis  were  indicative  of  the  side  on  which  the  tumor  is  situated,  it 
would  be  so  especially  when  the  tumor  is  in  the  frontal  lobe. 

LESIONS    OF    THE    OCCIPITAL    LOBE. 

There  are  certain  signs  and  symptoms  pointing  to  tumor  of  the 
occipital  lobe,  and  yet  they  are  not  pathognomonic.     Early  developing 

387 


388  THE    EYE    AXD    NERVOUS    SYSTEM 

hemianopsia  or  hemiachromatopsia,  sensations  of  light,  visual  hallucina- 
tions in  the  blind  fields,  with  absence  of  Wernicke's  hemianopic  pu- 
pillary sign  and  with  the  general  symptoms  of  brain  tumor  are  indica- 
tive of  a  growth  in  the  occipital  lobe.  In  some  cases  the  symptoms  of 
cerebellar  tumor  are  added  and  are  caused  by  pressure  upon  the  cere- 
bellum.    (For  remarks  on  optic  neuritis  see  Chapter  VIIT,  p.  375.) 

Hallucinations  of  Sight. — Ferrier  and  Jackson  are  said  to  have  been 
the  first  to  observe  photopsia  in  disease  of  the  occipital  lobe.  The  visual 
hallucinations  usually  appear  in  the  blind  fields.  Wilbrandt  and 
Ilcnsehen  believe  that  these  visual  hallucinations  occur  only  with  sub- 
cortical hemianopsia,  and  not  when  the  cortical  visual  centre  alone  is 
disturbed.     (See  also  Chapter  IV,  p.  174.) 

Harris,  in  referring  to  Swanzy's  view  that  hallucinations  of  sight  in 
the  blind  fields  are  possible  in  lesions  of  the  optic  radiations  but  not  in 
hemianopsia  the  result  of  cortical  damage,  says  that  the  reverse  seems 
to  be  more  common.  The  only  case  with  necropsy  supporting  Swanzy's 
view  is  that  reported  by  de  Schweinitz.  Harris  refers  to  a  number  of 
cases  of  visual  hallucination  with  hemianopsia  but  almost  all  were 
without  necropsy. 

Oppenheim  mentions  that  visual  hallucinations  and  illusions  may 
be  caused  by  intraocular  lesions,  and  refers  to  Uhthoff.  This  phe- 
nomenon I  also  have  observed.  I  recall  a  patient  under  the  care  of  Dr. 
C.  K.  Mills  whom  I  saw  with  him.  The  man  had  hallucinations  of  sight, 
and  saw  various  objects,  especially  horses,  as  a  symptom  of  hemorrhage 
into  the  retina. 

L.  Bruns  believes  that  visual  irritation  symptoms  preceding  or  ac- 
companying hemianopsia  are  of  value  as  indicating  a  location  of  the 
lesion  in  the  occipital  lobe.  These  symptoms  may  be  spots  or  lines  of 
light  before  the  eyes,  or  hallucinations  of  sight. 

In  a  case  observed  by  Byrom  Bramwell  ^  in  which  numerous  tumors 
were  found  in  the  brain,  and  the  occipital  lobes  and  each  optic  thalamus 
were  implicated  by  the  tumors,  the  patient  had  frequent  paroxysmal 
attacks  lasting  about  five  minutes  at  a  time,  with  headache,  flushing,  and 
flashes  of  light  in  the  eyes.  The  light  was  especially  bright  in  the  right 
eye  and  had  the  brilliancy  of  electricity,  and  varied  in  size  from  a  button 
to  a  large  globe.  During  these  paroxysms  the  patient  was  completely 
blind.  Hemianopsia  did  not  appear  to  be  present  after  an  attack.  Bram- 
well attributes  these  flashes  of  light  in  the  right  eye  to  irritation  of  the 
left  half-vision  centre.    It  is  well  to  remember,  however,  that  each  optic 

'  Byrom  Bramwell,  Brain,  1899,  vol.  xxii.  p.  41. 


TUMOKS  AXD  OTHER  BRAIX  LESI0:N'S  389 

thalamus  was  the  seat  of  tumor,  and  it  is  possible  that  the  irritation 
symptoms  were  caused  by  the  implication  of  these  bodies ;  such  a  com- 
plication was  not  present  in  another  case  observed  by  him. 

In  this  second  case  the  cicatrix  of  an  old  syphilitic  gumma  was 
found  on  the  outer  surface  of  the  posterior  end  of  the  left  occipital  lobe. 
The  patient,  a  man,  complained  of  severe  headache  confined  to  the  back 
and  top  of  the  left  side  of  the  head.  He  had  peculiar  sensory  (visual) 
Jacksonian  epilepsy.  The  attacks  occurred  on  an  average  about  once 
a  year,  and  each  attack,  consisting  of  numerous  paroxysms,  usually 
lasted  three  or  four  weeks.  The  paroxysms  occurred  every  five  or  ten 
minutes,  and  lasted  from  half  a  minute  to  three  minutes.  They  con- 
sisted of  flashes  of  light  in  the  right  eye,  and  the  light  was  said  to  be 
red  and  white.  For  some  days  after  the  attack  the  patient  was  unable 
to  see  to  the  right  side  (right-sided  homonymous  hemianopsia). 

Jolly  has  shown  that  scotoma  scintillans  may  result  from  a  disturb- 
ance of  the  visual  tracts,  and  more  recently  A.  Pick  ^  has  demonstrated 
by  the  report  of  four  clinical  cases  that  hallucinations  of  sight  in  the 
hemianopic  fields  may  be  caused  in  the  same  way.  The  first  of  his  cases 
shows  that  a  focus  of  softening  probably  existed  in  the  left  angular 
g)'rus.  Important  symptoms  in  this  case  were  paraphasia  and  para- 
graphia and  persistent  right  lateral  hemianopsia.  At  least  on  one  occa- 
sion everything  in  the  right  side  of  the  fields  of  vision  was  of  a  reddish 
color  and  changed  later  to  green.  For  about  half  an  hour  a  dog  was 
seen  by  the  patient  walking  along  with  him  at  his  right  side.  This  was 
merely  a  visual  hallucination.  • 

It  seems  that  hallucinations  of  sight  may  be  caused  by  disturbance 
of  any  part  of  the  visual  system  from  the  eyeball  to  the  cortical  layer  of 
the  occipital  lobe. 

Pick  has  observed  bitemporal  scotoma  scintillans,  and  he  attributes 
this  phenomenon  to  a  lesion  of  the  chiasm. 

According  to  Uhthoff,^  disease  of  the  retrobulbar  and  intracranial 
optic  pathways  relatively  seldom  causes  visual  hallucinations,  but  it  did 
in  one  of  his  cases  and  he  refers  to  some  others  in  the  literature  (Chris- 
tian, de  Schweinitz). 

Harris  reports  a  case  of  partial  hemianopsia  in  which  a  peripheral 
zone  of  the  left  lower  quadrant  remained  intact.  The  lesion  was  be- 
lieved to  be  a  softening  of  the  right  cuneus.  The  patient  had  frequent 
attacks  in  which  he  saw  red  and  green  lights  always  to  the  left  in  the 


^  A.  Pick,  Amer.  Journ.  of  the  Med.  Sci.,  January,  1904,  p.  82. 
'  Uhthoff,  Monatsschrift  fiir  Psychiatrie  und  Neurologie,  vol. 


390  THE    EYE    AND    NERVOUS    SYSTEM 

bliud  field,  and  they  apjieared  to  be  moving  in  both  directions,  from  the 
periphery  towards  the  centre  and  in  the  reverse  direction. 

I  know  of  a  case  in  which  the  diagnosis  of  fracture  of  the  base  of 
the  skull  was  made.  The  patient  at  times  had  visual  and  olfactory 
hallucinations  and  saw  his  bed  and  other  objects  move  about  him  and 
turn  upside  down. 

In  the  case  rej^orted  by  de  Schweinitz  the  patient  had  visions  of 
chairs,  tables,  and  other  articles  of  furniture,  not  really  present,  in  the 
fields  which  afterward  were  obliterated.  Left  lateral  hemianopsia  de- 
veloj)ed,  the  dividing  line  passing  in  advance  of  the  fixation-point.  The 
patient  was  unable  to  say  whether  the  visual  hallucinations  disappeared 
immediately  after  the  hemianopsia  set  in,  or  whether  they  remained  for 
some  time  in  the  dark  half-fields.  At  the  necropsy  made  by  Dercum 
gummatous  infiltration  at  the  base  of  the  brain  pressing  upon  the  right 
optic  tract  was  found.  De  Schweinitz  *  says  he  was  not  aware  that  any 
lesion  of  the  occipital  lobe  was  present,  and  it  was  not  probable  from 
the  symptoms  that  one  would  be  found. 

Bregman  "'  has  described  green  and  violet  vision  occurring  in  tabes 
dorsalis  with  optic  atrophy,  and  probably  resulting  from  the  irritation 
of  the  optic  nerves.  Alter  observed  a  case  of  paretic  dementia  in  which 
attacks  of  green  vision,  lasting  one  or  two  hours,  occurred,  and  occasion- 
ally were  followed  by  achromatopsia.  Objects  were  seen  in  various 
shades  of  green.  It  seems  probable  that  these  peculiar  visual  disturb- 
ances in  which  objects  appear  red  or  green  may  be  caused  by  irritation 
of  any  part  of  the  visual  system,  and  it  is  not  mere  chance  that  dictates 
the  colors  seen.  The  fibres  conveying  sensation  of  red  and  green,  if  such 
fibres  exist,  seem  to  be  chiefly  affected  in  optic  atrophy,  and  therefore 
from  irritation  sensations  of  light  might  be  more  likely  to  appear  as 
red  and  green  vision.  The  violet  seen  by  Bregman's  patient  contained 
red.  We  should,  therefore,  from  a  study  of  the  cases  mentioned,  be 
very  careful  before  assuming  that  subjective  disturbances  of  color  vision 
indicate  a  lesion  of  the  occipital  lobe. 

I  am  inclined  to  think  that  inverted  vision,  or  mirror  vision,  may  be 
caused  by  lesions  of  the  occipital  lobes,  but  much  study  is  needed  in 
regard  to  this  phenomenon. 

Alexia  and  Optic  Aphasia. — ^lore  valuable  are  alexia  and  optic  aphasia 
as  signs  of  a  lesion  of  the  left  occipital  lobe.  These  signs  are  explained 
by  L.  Bruns  as  tlie  result  of  a  lesion  of  the  association  tracts  between 

*  De  Schweinitz.  New  York  Med.  Journ.,  May  2,  1891,  p.  514. 

•  Bregman,  Deutsche  Zeitschrift  fiir  Nervenheilkunde,  vol.  xxvi.,  Nos.  4  to  6, 
p.  625. 


TUMORS  AND  OTHER  BRAIN  LESIONS  391 

both  occipital  lobes  and  the  left  first  temporal  convolution  at  a  point 
where  these  tracts  are  near  together  in  the  white  matter  of  the  left 
occipital  lobe.  This  explanation  is  bj  no  means  generally  accepted. 
The  symptom-complex  is  not  always  typical,  but  Bruns  says  it  has  been 
fairly  typical  in  a  number  of  cases,  and  was  so  in  one  that  he  himself 
observed.  Mind-blindness  is  present  to  some  extent  in  association  with 
the  optic  aphasia,  but  lesions  of  both  occipital  lobes  seem  to  be  requisite 
for  this  sign  to  be  pronounced.  The  reader  is  referred  to  the  chapter 
by  Dr.  C.  K.  Mills  on  the  "  Psychology  of  the  Visual  Act  and  the  Focal 
Diseases  of  the  Visual  Cortex"  for  a  fuller  discussion  of  achromatopsia, 
mind-blindness,  optic  aphasia,  word-blindness,  alexia,  and  allied  sub- 
jects. 

Transitory  amaurosis  sometimes  occurs  with  tumors  of  the  occipital 
lobe  (Bruns),  but  according  to  Hirschberg  it  has  little  localizing  value, 
as  has  also  conjugate  deviation  of  the  eyes,  which  according  to  Oppen- 
heim  occurs  sometimes  with  occipital  lobe  tumors.  There  may  be  a 
centre  for  associated  ocular  movements  in  the  parieto-occipital  lobe  as 
vs^ell  as  in  the  frontal  lobe. 

Hemianopsia. — The  most  valuable  localizing  sign  of  tumor  of  the 
occipital  lobe  is  homonymous  lateral  hemianopsia.  This  by  itself  is  not 
sufficient,  inasmuch  as  it  may  be  caused  by  a  lesion  of  the  visual  path- 
ways anywhere  between  the  chiasm  and  the  visual  cortex  of  the  occipital 
lobe.  According  to  Oppenheim,  Bernhardt  in  fifteen  cases  of  tumor  of 
the  occipital  lobe  found  hemianopsia  in  only  two  cases.  An  examination 
of  the  more  recent  reports  has  shown  that  rarely  hemianopsia  is  absent 
in  cases  of  tumor  of  the  occipital  lobe. 

It  has  been  supposed  by  some  investigators  that  if  the  hemianopsia 
is  caused  by  a  lesion  of  the  visual  cortex  the  patient  will  be  ignorant  of 
his  half-blindness  until  it  is  pointed  out  ("vision  nulle"  of  Dufour), 
whereas  this  will  not  be  the  case  if  the  hemianopsia  is  caused  by  a  sub- 
cortical lesion.  This  distinction  is  unreliable.  Harris  has  examined 
for  this  sign  several  times  without  any  satisfactory  results,  and  in  one 
case  in  which  the  lesion  was  in  the  posterior  part  of  the  internal  capsule, 
the  patient  was  not  aware  of  any  defect  of  vision,  and  could  witli  diffi- 
culty be  persuaded  that  considerable  portions  of  his  visual  fields  were 
lost.     (See  Chapter  IV,  p.  167.) 

It  seems  strange  that  hemianopsia  could  be  overlooked,  and  yet  a 
case  has  been  mentioned  to  me  by  Frankl-Hochwart,  in  which  the 
sign  was  discovered  after  it  was  noticed  that  the  patient,  then  in  a  large 
hospital,  Habitually  ate  only  the  food  on  one  half  of  his  plate.  It  was 
found  on  examination  that  he  saw  only  one  half  of  his  plate. 


392  THE    EYE    AND    NERVOUS    SYSTEM 

Hemiachromatopsia. — Hemiachromatopsia  has  been  considered  by 
some  as  the  result  of  lesion  of  certain  special  centres  or  fibres  in  the 
visual  system.  Ward  A.  Holden  refers  to  a  considerable  number  of 
cases  in  which,  with  a  normal  field  for  large  white  test-objects,  there 
was  hemianopsia  for  colors.  Most  of  the  older  writers  agree  that  there 
must  be  special  separate  cortical  centres  for  the  perception  of  form,  light, 
and  color,  and  that  in  the  cases  under  consideration,  the  cortical  color 
centre  was  exclusively  involved.  This  supposition  was  defended  par- 
ticularly by  Samelsohn,  Landolt,  and  Wilbrand.  Of  late  there  has  been 
a  tendency  to  depart  from  this  teaching,  and  Ole  Bull  and  Otto  Dahms 
do  not  accept  it.  Some  observations  of  his  own  have  convinced  Holden 
that  the  supposition  of  there  being  a  lesion  of  some  special  color  centre 
in  these  cases  is  wrong.     His  views  are  as  follows : 

"  A  slight  interference  in  conduction  of  any  of  the  fibres  of  the 
visual  tract,  leads  to  an  inability  to  recognize  green  or  even  red,  or  to  dis- 
tinguish slight  differences  in  luminous  intensity.  A  more  marked  dis- 
turbance in  conduction  leads  to  the  inability  to  recognize  blue,  or  to 
distinguish  quite  marked  differences  in  luminous  intensity.  A  greater 
interference  with  conduction  prevents  the  distinguishing  of  white  from 
black,  and  with  complete  interference  with  conduction  even  perception 
of  light  is  lost. 

"  Thus  the  recognition  of  color  varies  with  the  light  sense,  and  the 
assumption  of  the  involvement  of  a  particular  cortical  color  centre  in 
cases  of  hemiachromatopsia  is  not  only  unnecessary,  but  is  palpably 
erroneous."  ® 

De  Schweinitz  '^  expresses  the  opinion  that  homonymous  hemiachro- 
matopsia probably  is  caused  by  a  cortical  lesion  of  less  intensity  than 
one  which  produces  absolute  hemianopsia.  In  a  case  studied  by  him 
at  the  onset  the  hemianopsia  was  absolute ;  later  light  sense  and  form 
sense  returned.  The  obliteration  of  the  color  sense  remained,  although 
in  all  other  respects  the  patient  had  recovered.  Non-cortical  lesions,  he 
holds,  may  also  produce  hemiachromatopsia. 

An  interesting  case  recorded  by  Harris  shows  that  a  lesion  in  the 
neighborhood  of  the  internal  capsule  may  cause  hemiachromatopsia,  and 
that  the  lesion  need  not  be  cortical  to  produce  this  sign.  In  his  case 
there  were  almost  complete  left  hemiplegia,  partial  left  hemianesthesia, 
and  complete  loss  of  all  color  perception,  even  of  large  area,  in  the  left 
lower  quadrant  for  each  eye;    small  objects,  as  the  head  of  a  pin  or  a 


•  Holden,  Archives  of  Ophthalmology,  vol.  xxiv.  p.  447. 
'  Diseases  of  the  Eye,  p.  559. 


TUMORS  AND  OTHER  BRAII^  LESIOXS  393 

disk  of  white  paper  1  centimetre  in  diameter,  were  also  invisible,  and 
the  patient  could  not  distinguish  between  one  and  two  fingers  held  up 
in  the  same  area,  though  he  could  readily  do  so  in  each  of  the  remaining 
quadrants.  The  cuneus  was  found  to  be  normal  on  each  side,  but  there 
was  an  area  of  softening  in  the  right  internal  capsule. 

Harris  quotes  from  Swanzy's  "  Handbook  of  the  Diseases  of  the  Eye 
and  their  Treatment :"  "  It  is  now  generally  believed  that  relative 
hemianopsia  {e.g.,  color  hemianopsia  alone)  is  the  result  of  a  lesion  of 
less  intensity  than  that  which  causes  absolute  hemianopsia  .  .  .  and 
that  the  color  sense  is  more  easily  affected  by  disease  than  the  form  or 
light  sense,  and  that,  too,  irrespective  of  the  position  of  the  lesion  in  the 
visual  path." 

Hemianopic  Pupillary  Reaction  Sign. — The  absence  of  the  hemianopic 
pupillary  rigidity  of  Wernicke  points  rather  to  a  tumor  of  the  occipital 
lobe  when  a  lesion  of  the  visual  pathways  is  probable,  as  shown  by 
lateral  homonymous  hemianopsia,  but  the  sign  is  difficult  to  obtain  and 
the  danger  of  error  is  great.  I  believe,  however,  that  I  have  seen  this 
sign  positively  demonstrated  in  a  few  cases.  It  consists  in  contraction 
of  the  iris  to  light  only  when  the  halves  of  the  retinae  still  preserving 
visual  function  are  illuminated.  As  there  is  no  light  reflex  from  illu- 
mination of  the  blind  half  of  each  retina  when  this  sign  is  present,  the 
lesion  must  be  somewhere  within  the  pupillary  reflex  arc,  therefore  in 
or  below  the  primary  visual  centres  (external  geniculate  body,  pulvinar 
of  the  optic  thalamus,  and  anterior  colliculi  of  the  corpora  quadri- 
gemina).  This  sign  cannot  be  caused  by  a  lesion  above  the  primary 
visual  centres,  in  the  optic  radiations  or  visual  cortex,  although  there 
may  be  hemianopsia  from  such  a  lesion.  (See  Chapter  IV,  p.  170,  and 
Chapter  VI,  p.  318.) 

Paralysis  of  Cranial  Nerves  and  Cerebellar  Ataxia  as  Signs  of  Occipital 
Tumor. — \Vlien  paralysis  of  cranial  nerves  occurs  in  association  with 
homonymous  lateral  hemianopsia  it  is  in  favor  of  a  growth  below  the 
occipital  lobe,  and  yet  it  is  not  absolute  proof  of  a  basal  lesion,  especially 
when  the  paralysis  implicates  the  sixth  nerve  of  either  side.  This  nerve 
has  so  long  a  course  within  the  cranium  that  it  may  be  injured  by  in- 
creased intracranial  pressure.  Oppenheim  believes  it  is  possible  for  the 
nerves  of  the  posterior  cranial  fossa  to  be  affected  by  the  pressure  of  an 
occipital  tumor. 

Jacksonian  motor  epilepsy  cannot  be  regarded  as  a  common  sign  of 
tumor  of  the  occipital  lobe.  Cerebellar  ataxia  has  been  observed,  and  has 
been  explained  as  the  result  of  pressure  upon  the  cerebellum.^     The 


'L.  Bruns,  Die  Geschwiilste  des  Xervensystems.  S.  Karger,  Berlin,  1897. 


394 


THE    EYE    AND    NERVOUS    SYSTEM 


tentorium  usually  is  sufficient  to  prevent  pressure  upon  tlie  cerebellum, 
but  in  a  case  of  internal  hydroceplialus  in  the  service  of  Dr.  Mills, 
caused  by  closure  of  the  aqueduct  of  Sylvius,  which  I  had  the  oppor- 
tunity to  study  and  have  reported,  pressure  on  the  cerebellum  from  the 
distended  posterior  horns  of  the  lateral  ventricles  seemed  to  afford  a 
satisfactory  explanation  of  the  ataxia.  This  case  is  as  follows :  A  boy, 
aged  nineteen  years  at  the  time  of  death,  had  had  soine  headache  once 
or  twice  a  week,  with  vomiting,  since  childhood;  but  about  six  months 
before  death  the  headache  became  severe,  and  cerebellar  gait  was  ob- 
served, and  he  staggered  like  a  drunken  man.     The  station  with  feet 


Fio.  1.— Almost  complete  closure  of  the  aqueduct  of  Sylvius,  only  the  extreme  lower  end  re- 
maining patulous,  causing  internal  hydrocephalus,  and  symptoms  of  cerebellar  tumor.  The  fourth 
ventricle  was  normal  in  size. 

apart  was  good,  but  with  feet  together  was  poor,  and  the  sway  was  not 
increased  b}-  closure  of  the  eyelids.  The  gait  was  so  unsteady  that  at 
times  he  had  to  catch  hold  of  the  bed  to  keep  from  falling.  He  was 
occasionally  dizzy  when  lying  on  his  back,  and  the  dizziness  w'as  re- 
lieved by  turning  on  his  side.  Slight  exophthalmos  was  observed.  Dis- 
tinct optic  neuritis  was  present  in  each  eye.  The  patellar  reflexes  were 
exaggerated.  The  Babinski  reflex  was  absent  on  each  side.  All  venereal 
disease  was  denied.  There  was  certainly  sufiicient  here  to  suggest  a 
cerebellar  lesion,  but  none  existed. 


TUMOKS  AND  OTHER  BRAIN  LESIONS  395 

On  removing  the  skullcap,  which  was  extremely  thin,  the  brain  and 
its  membranes  bulged  outward.  On  the  internal  surface  of  the  skullcap 
the  moulding  of  the  convolutions  of  the  brain  could  be  distinctly  made 
out.  The  third  and  lateral  ventricles  were  distended  with  clear  fluid, 
and  the  aqueduct  of  Sylvius  was  closed  by  proliferated  neuroglia,  while 
the  fourth  ventricle  was  of  normal  size.  In  microscopical  sections  a 
very  small  opening  where  the  aqueduct  should  be,  was  found,  but  dur- 
ing life  this  may  have  been  closed,  and  it  is  doubtful  whether  it  ex- 
tended all  the  way  from  the  third  to  the  fourth  ventricle. 

In  a  case  of  echinococcus  cysts  of  the  left  occipital  lobe  reported  by 
Mingazzini,  lateral  homonymous  hemianopsia  was  not  mentioned  by 
the  patient  and  was  not  searched  for  by  the  physicians.  Choked  disks, 
much  diminished  patellar  reflexes,  intense  headache  occurring  periodi- 
cally, and  tinnitus  in  the  left  ear  were  rather  suggestive  of  the  cere- 
bellum as  the  seat  of  the  tumor,  and  Mingazzini  says  that  if  lateral 
homonymous  hemianopsia  had  existed  one  might  still  think  of  cere- 
bellar tumor,  as  this  phenomenon  as  a  distant  symptom  has  been 
observed  in  tumor  of  the  cerebellum. 

Lumbar  puncture  was  made  in  this  case  and  the  patient  went  into 
•collapse  the  same  evening  and  died  the  next  morning.  This  procedure 
is  dangerous  in  cases  of  tumor  of  the  occipital  lobe,  cerebellum  or 
medulla  oblongata,  and  probably  also  when  the  tumor  is  situated  else- 
where in  the  brain. 

A  similar  case  has  been  observed  by  Mann.  A  tumor  of  the  occipital 
lobe  was  found.  Ilemiataxia  and  the  Babinski  sign  occurred  on  the 
same  side  as  the  tumor,  and  there  was  much  in  this  case  to  suggest  a 
tumor  of  the  cerebellum, 

Oppenheim  speaks  of  a  case  of  Schultze  in  which  a  cerebellar  tumor 
by  pressure  on  the  occipital  lobe  caused  symptoms  of  a  lesion  in  the 
occipital  lobe.  He  refers  to  a  case  of  his  own  in  which  a  tumor  of  the 
precuneus  caused  cerebellar  symptoms  by  pressure.  A  tumor  of  the 
•cerebellum  was  observed  by  him  to  compress  the  descending  horn  of 
the  lateral  ventricle,  and  thereby  to  cause  distention  of  this  horn  and 
interference  with  the  function  of  the  occipital  lobe.  I  have  seen  dis- 
tention of  one  posterior  horn  of  the  lateral  ventricle  caused  by  a  tumor 
Avithin  the  pons. 

TUMORS    OF    THE    PARIETAL    LOBE. 

Tumors  in  this  area  of  the  brain  may  cause  convulsions  in  the  limbs 
and  face  of  the  opposite  side  by  irritation  of  the  adjoining  motor  area, 
Aveakness  of  the  limbs  of  the  opposite  side  from  pressure  upon  the 


396  THE    EYE    AND    NERVOUS    SYSTEM 

cortical  motor  centres,  loss  of  stereognostic  perception  and  of  the  sense 
of  position,  and  loss  or  impairment  of  tactile  sensation  with  less  implica- 
tion of  pain  sensation,  and  awkwardness  of  movements  in  the  limbs  of 
the  other  side.  The  astereognosis  and  loss  of  the  sense  of  position  may 
be  so  great  that  the  patient,  as  in  a  case  under  my  care,  may  be  unable 
to  recognize  any  object  placed  in  his  hand  of  the  opposite  side  when  the 
eyes  are  closed,  or  have  any  knowledge  of  the  position  of  the  limbs  of 
the  opposite  side,  and  may  take  the  hand  of  the  examiner  believing  it  to 
be  his  own.  In  the  case  referred  to  the  meningitis  in  plaques  was  found 
over  and  almost  confined  to  the  opposite  parietal  lobe.  Ataxia  of  the 
limbs  on  the  side  opposite  the  lesion  is  to  be  expected  if  the  sense  of 
position  is  so  impaired  that  the  patient  has  no  knowledge  of  the  posi- 
tion of  his  limbs.  I  have,  in  several  cases  of  lesion  of  the  parietal  lobe, 
observed  the  patient  place  the  normal  hand  on  his  shoulder  or  some 
part  of  the  opposite  upper  limb  in  searching  for  the  hand  of  this  side, 
and  by  following  the  limb  down  reach  his  hand. 

Although  I  believe  that  a  lesion  of  the  parietal  lobe  may  cause  ataxia, 
loss  of  stereognostic  perception,  and  loss  of  the  sense  of  position  and 
some  disturbance  of  tactile  and  possibly  of  pain  sensation  in  the  limbs 
of  the  opposite  side,  I  have  not  been  convinced  that  a  lesion  of  the 
motor  area,  or  at  least  of  the  post-central  convolution,  will  give  none  of 
these  symptoms.  The  tendency  now  is  to  regard  the  post-central  con- 
volution as  having  no  motor  function. 

Marinesco  remarks  that  Jonesco  removed  a  part  of  the  cortical  centre 
for  the  left  upper  limb  in  a  man.  After  the  operation  the  patient  could 
move  his  shoulder  and  elbow  very  well,  but  not  his  hand.  Nine  months 
later  the  movements  of  the  left  hand. were  very  imperfect,  and  tactile 
sensation,  the  sense  of  position,  and  stereognostic  perception  were 
diminished  in  the  left  hand.  In  another  case  Jonesco  removed  the 
centres  of  the  face  and  upper  and  lower  limbs  of  the  right  cerebral 
cortex.  A  year  after  the  operation  there  was  much  weakness  of  the  left 
side  of  the  body,  and  the  sense  of  position,  stereognostic  perception,  and 
tactile  sensation  were  affected  in  the  left  upper  limb.  Thermal  and 
painful  sensations  were  preserved.  These  operations  were  done  for  epi- 
lepsy, and  in  one  case,  at  least,  and  probably  in  both,  the  cortical  motor 
areas  were  determined  by  the  electric  current. 

I  have  observed  loss  of  stereognostic  perception  caused  by  lesions 
much  below  the  cortex  of  the  brain, — viz.,  in  a  case  of  tumor  of  the  pons 
(Potts  and  Spiller)  and  in  a  case  of  tumor  compressing  the  medulla 
oblongata  (case  of  Dercum)  ;  but  where  the  symptoms  indicate  a  tumor 
of  the  cerebrum  that  probably  is  cortical,  I  believe  the  loss  of  stereog- 


TUMORS  AND  OTHER  BHAm   LESI0:N'S     397 

nostic  perception  and  of  the  sense  of  position  and  ataxia  of  the  upper 
limb  of  the  side  opposite  to  that  in  which  is  the  lesion  indicate  much 
implication  of  the  parietal  lobe. 

In  1898,  at  a  meeting  of  the  Philadelphia  Xeurological  Society, 
I  ^  referred  to  ?)  case  that  I  had  seen  in  the  service  of  Dr.  Lloyd,  at  the 
Philadelphia  Hospital,  which  seemed  to  show  that  the  cortical  represen- 
tation of  the  sense  of  position  is  in  the  parietal  lobe.  A  man  complained, 
during  the  night  preceding  his  attack,  of  fatigue,  headache,  and  inability 
to  sleep.  The  following  morning  he  fell  to  the  floor  on  attempting  to 
rise  from  his  bed,  but  was  not  unconscious.  When  he  was  found  he  had 
paresis  of  the  left  limbs  and  of  the  left  side  of  the  face  and  tongue. 
Later  the  patient  could  raise  his  left  upper  limb  above  his  head,  but 
every  movement  of  this  limb  was  ataxic  in  an  extreme  degree,  though 
the  limb  was  not  paralyzed.  The  mental  condition  of  the  patient  pre- 
vented an  examination  of  the  condition  of  sensation.  At  the  necropsy  a 
hemorrhage  was  found  in  the  right  parietal  lobe.  The  hemorrhagic 
area  was  about  2  centimetres  in  diameter,  and  extended  inward  in  the 
form  of  a  cone  to  the  lateral  ventricle,  having  its  base  in  the  cortex.  It 
was  situated  about  4  centimetres  from  the  longitudinal  fissure  and 
about  2  or  3  centimetres  behind  the  Rolandic  fissure.  The  brain 
had  been  cut  into  frontal  sections  about  1  centimetre  apart,  when 
I  had  an  opportunity  to  examine  it,  and,  although  the  injury  to  the 
cerebral  tissue  prevented  an  exact  determination  of  the  location  of 
the  hemorrhage,  it  was  evidently  in  or  very  close  to  the  supra- 
marginal  gyrus.  The  ataxia  was  probably  caused  by  a  loss  of  the  sense 
of  position. 

This  was  probably  one  of  the  first  cases  reported  in  this  country, 
showing  that  ataxia  from  a  loss  of  the  sense  of  position  may  be  caused 
by  a  lesion  of  the  parietal  lobe.  The  case  of  Starr  and  McCosh 
preceded  it. 

A  case  that  Dr.  C.  K.  Mills  and  I  saw  together,  June  3,  1901,  in 
consultation,  the  case  being  under  the  charge  of  Dr.  Ida  Richardson, 
affords  evidence  also  of  the  localization  of  the  stereognostic  perception 
and  of  the  sense  of  position  in  the  parietal  lobe.  The  patient's  left  upper 
limb  was  decidedly  ataxic,  paretic,  and  astereognostic,  and  the  head  was 
retracted  and  rigid.  The  necropsy  was  made  by  Dr.  W.  F.  Hendrick- 
son.  An  area  of  depression,  indefinite  in  outline,  but  about  6  centi- 
metres in  diameter,  was  found  in  the  parietal  lobe  of  the  right  cerebral 
hemisphere  just  posterior  to  the  fissure  of  Rolando.  Palpation  revealed 
less  resistance  at  this  point  than  over  the  surrounding  tissue.    On  section 

'  Journal  of  Nervous  and  Mental  Disease,  January,  1899,  p.  43. 


398  THE    EYE    AND    NERVOUS    SYSTEM 

this  area  was  found  to  be  the  seat  of  extensive  softening.  The  process 
implicated  practically  the  entire  right  parietal  lobe. 

The  case  reported  by  Dr.  C.  K.  Mills,^"  which  was  seen  by  me  and 
others  in  consultation,  was  one  of  the  first  to  establish  the  localization 
of  the  stereognostic  perception  in  the  parietal  lobe.  A  diagnosis  of 
tumor  of  this  lobe  was  made  because  of  the  loss  of  the  sense  of  position, 
impaired  cutaneous  sensibility,  astereognosis,  and  ataxia.  The  case 
has  been  so  fully  reported  by  Dr.  Mills  and  is  so  well  known  that  I  need 
not  refer  to  it  further. 

Oppenheim,*'  after  giving  a  description  of  his  case  of  parietal  tumor 
with  operation,  remarks  that  lesions  of  the  parietal  lobe  cause  ataxia 
of  one  or  both  limbs  of  one  side.  Irritative  or  paralytic  motor  symptoms 
are  absent,  or  when  they  occur  they  are  the  result  of  pressure.  The 
sensory  disturbance  is  always  a  partial  hemiana?sthesia,  in  that  pain  and 
temperature  sensations  are  preserved,  while  tactile  sensation  and  espe- 
cially the  sense  of  position  and  the  stereognostic  perception  are  impaired 
or  lost.  Such  a  symptom-complex  occurs  when  the  lesion  does  not 
extend  far  into  the  white  matter  of  the  parietal  lobe.  He  says,  also,  that 
the  cases  in  which  tumor  of  the  jiarietal  lobe  has  been  so  accurately  diag- 
nosed that  a  radical  operation  was  possible  are  very  few. 

This  case  of  Oppenheim  was  reported  by  him  February  12,  1900, 
and  published  in  the  third  number  of  the  Mitteilungen. 

In  his  monograph  ^^  on  tumors  of  the  brain  he  says  that  in  the  ma- 
jority of  eases  of  tumor  of  the  parietal  lobe  sensory,  irritative  and 
paralytic  phenomena  were  observed.  He  refers  to  Mills's  case,  but  adds 
that  the  cases  are  npt  sufficient  to  permit  us  to  regard  the  above-men- 
tioned phenomena  as  positive  evidence  of  lesions  of  the  parietal  lobe. 
The  evidences  of  the  localization  value  of  these  symptoms  are,  however, 
rapidly  accumulating. 

In  March,  1901,  Dr.  C.  W.  Burr  V^  reported  a  case  in  which  there 
were  slight  hemiplegia,  slight  tactile  anaesthesia,  astereognosis,  mind- 
blindness,  loss  of  the  tempierature  sense  on  one  side,  sensory  aphasia 
varying  in  intensity,  and  mental  dullness.  In  this  case  a  tumor  was 
found  in  the  parietal  lobe,  pressing  against  but  not  invading  the  ascend- 
ing parietal  convolution,  partially  destroying  the  angular  gyrus,  and 
involving  almost  the  entire  posterior  parietal  lobule. 

"  Mills,  Keen,  and  Spiller,  Journal  of  Nervous  and  Mental  Disease,  May,  1900, 
p.  244. 

"  Mitteilungen  aus  den  Grenz^bieten  der  Medizin  und  Chirurgie,  1900,  vol.  vi. 
pp.  382,  383. 

"Oppenheim,  Die  Geschwiilste  des  Gehirns,  second  edition,  p.   116. 

"The  Amer.  Journ.  of  the  .Med.  Sci..  March,  1901,  p.  306. 


/■ 


-^'.r^ 


Figs.  2,  8.— Extreme  muscular  atrophy,  hemiplegia,  and  bilateral  contractures  from  internal  hydroce- 
phalus confined  to  one  side  of  brain. 


TUMOKS  AND  OTHER  BRAIX  LESIOIS^S  399 

A  tumor  of  the  parietal  lobe  by  growing  downward  may  involve  the 
internal  capsule  and  the  optic  radiations,  and  thereby  cause  hemiplegia, 
hemiana^sthesia,  and  hemianopsia. 

TUMORS  OF  THE  TEMPORAL  LOBE. 

When  the  tumor  is  situated  in  the  left  temporal  lobe,  sensory  aphasia 
from  implication  of  the  posterior  part  of  the  first  temporal  convolution 
may  be  expected.  The  patient  becomes  word-deaf  and  is  unable  to 
speak  connectedly;  he  may  also  be  word-blind.  The  latter  symptom, 
according  to  some  neurologists,  is  to  be  explained  by  implication  of  the 
left  angular  g}'rus.  The  right  temporal  lobe  is  a  much  more  "  silent 
area"  than  the  left,  at  least  in  right-handed  people,  and  a  tumor  here 
may  grow  to  a  considerable  size  before  causing  localizing  symptoms.  A 
tumor  in  either  the  right  or  left  temporal  lobe  may  by  its  gro\vth  cause 
pressure  upon,  or  direct  involvement  of,  the  posterior  limb  of  the  inner 
capsule,  with  hemiplegia  of  the  opposite  side  of  the  body. 

TUMORS    OF    THE    MOTOR    AREA. 

A  growth  in  this  region  is  more  easily  diagnosticated  than  one  in  any 
other  part  of  the  brain.  Jacksonian  motor  epilepsy  with  paresis  or 
paralysis  of  the  face  and  limbs,  and  with  motor  aphasia,  if  the  tumor  is 
on  the  left  side  in  a  right-handed  person,  forms  a  symptom-group  veiy 
characteristic  of  tumor  in  the  central  gyri.  I  have  observed  a  case  in 
which  a  gradually  developing  hemiplegia,  with  paresis  of  the  other 
lower  limb,  resulted  from  internal  hydrocephalus  confined  to  one  lateral 
ventricle.  Such  a  case  might  be  difficult  to  diagnose  from  one  of  sub- 
cortical tumor  of  the  motor  region  (Figs.  2,  3). 

TUMORS    OF    THE    FRONTAL    LOBE. 

The  frontal  lobe  may  be  seriously  involved  by  a  tumor  without 
causing  localizing  symptoms,  but  it  is  probable  that  a  growth  is  more 
likely  to  cause  derangement  of  the  higher  intellectual  functions  when  it 
is  situated  in  this  portion  of  the  brain,  especially  in  the  left  cerebral 
hemisphere.  Judgment,  power  of  concentration  of  thought,  ability  to 
understand  what  is  read,  etc.,  may  show  early  impairment  in  cases  of 
frontal  tumor. 

Jacksonian  convulsions  in  the  limbs  of  the  opposite  side  are  not 
uncommon,  because  of  the  proximity  of  the  motor  area,  and  hemiplegia 
or  monoplegia  of  the  opposite  side  may  also  be  produced  by  pressure 
upon,  or  direct  implication  of,  the  motor  region  of  the  brain.     According 


400  THE    EYE    AND    NERVOUS    SYSTEM 

to  the  investigations  of  Griinbaum  and  Sherrington,  the  centres  for  the 
limbs  and  face  are  entirely  in  front  of  the  central  fissure. 

TUMORS   OF   THE   OPTIC   THALAMUS. 

According  to  some  neurologists,  we  may  expect  as  signs  of  a  lesion 
of  the  optic  thalamus  a  hemiplegia  which  in  some  cases  may  be  slight,  in 
others  intense,  but  even  then  is  likely  to  diminish  in  intensity,  and  often 
is  less  pronounced  than  is  the  hemianaesthesia  on  the  same  side  as  the 
paralysis.  These  symptoms  are  explained  by  the  theory  that  all  sensory 
fibres  pass  through  the  optic  thalamus,  and  therefore  a  tumor  growing  in 
this  ganglion  directly  implicates  the  sensory  tract,  and  causes  a  dis- 
turbance of  motion,  at  first  by  pressure  upon  the  internal  capsule,  and 
later  by  direct  implication  of  this  system  of  fibres.  The  case  that  I  had 
the  opportunity  of  studying  wnth  Dr.  Dercum  some  years  ago,  in  which 
objective  disturbance  of  sensation  had  existed  many  years,  and  a  cyst 
was  found  in  the  carrefour  sensitif  of  Charcot  and  in  the  lenticular 
nucleus,  without  any  implication  of  the  optic  thalamus,  has  led  me  to 
believe  that  the  sensory  fibres  probably  do  not  all  terminate  in  the  optic 
thalamus.  In  addition  to  the  disturbances  of  motion  and  sensation 
mentioned,  subjective  disturbances  of  sensation, — viz.,  pain,  also  chorei- 
form or  athetoid  movements,  forced  laughter,  hemianopsia,  disturb- 
ances in  the  emotional  innervation  of  the  opposite  side  of  the  face  in 
laughing  and  crying,  and  forced  movements, — may  be  expected. 

In  a  case  of  abscess  of  the  right  optic  thalamus,  C  E.  Beevor^^  ob- 
served severe  pain  in  the  occipital  region  of  the  same  side,  vomiting, 
double  optic  neuritis,  with  some  weakness  of  the  face  and  limbs  of  the 
opposite  side,  a  tendency  of  the  patient  to  rotate  to  the  side  of  the 
lesion,  in  the  direction  of  screwing-in,  pleurosthotonus  and  a  tendency 
to  fall  backward  and  to  the  right. 

He  regards  as  symptoms  of  tumor  of  the  optic  thalamus  occipital 
pain  on  the  same  side  as  the  tumor,  impaired  mentality,  weakness  and 
nystagmus  of  ocular  movements  to  the  same  side,  weakness  of  the 
opposite  limbs,  rigidity  of  the  limbs  of  the  same  side,  staggering  back- 
ward and  to  the  same  side,  and  attacks  of  rotation  of  the  trunk  with  the 
face  towards  the  same  side. 

The  involuntary  movements  found  in  cases  of  lesion  of  the  thalamus 
may  be  ataxic,  choreic,  or  of  the  nature  of  intention  tremor. 

Charcot  believed  that  hemiehorea  is  produced  by  a  lesion  of  certain 
fibres  of  the  corona  radiata,  or  of  a  portion  of  the  posterior  limb  of  the 
inner  capsule  in  front  of  the  carrefour  sensitif.     Gowers  attributed  the 

"  Beevor,  Brain,  1898,  vol.  xxi. 


TUMORS  AND  OTHER  BRAIX  LESIONS  401 

hemicliorea  to  a  lesion  of  the  optic  thalamus;  Kahlcr  and  Pick  to  irri- 
tation of  the  pyramidal  tract,  but  Bonhoeffer  ^^  believes  that  hemichorea 
depends  upon  a  lesion  of  the  superior  cerebellar  peduncle,  and  he  at- 
tempts to  show  that  in  most  of  the  cases  with  necropsy  in  which  hemi- 
chorea was  present  these  peduncles  were  implicated. 

The  diagnosis  of  tumor  of  the  optic  thalamus  not  infrequently  is 
one  of  great  difficulty  and  in  some  cases  it  is  impossible.  The  symptoms 
may  be  those  of  a  cerebellar  tumor  as  in  a  case  observed  by  Douglas 
Stanley,^  ^  in  which  a  large  glio-sarcoma  was  found  occupying  the  whole 
of  the  left  optic  thalamus,  causing  compression  of  the  internal  capsule 
and  displacement  outward  of  the  lenticular  nucleus,  and  projecting 
into  the  lateral  ventricle,  so  as  to  cause  slight  pressure  on  the  opposite 
optic  thalamus.  The  patient  was  a  little  weak  in  the  right  arm  and 
leg,  and  had  difficulty  in  controlling  volitional  movements  of  these  limbs. 
He  had  a  coarse,  irregular  tremor  of  the  right  arm.  His  gait  was  un- 
steady and  he  reeled  to  the  right  side.  On  attempting  to  stand  with  his 
feet  together  he  was  liable  to  fall  to  the  right  side.  Occasionally  con- 
vergent strabismus  appeared  on  the  right  side,  and  occasionally  he  had 
internal  squint  with  the  left  eye  varying  in  degree,  so  that  there  was 
alternating  converging  strabismus,  and  it  was  often  difficult  to  say  which 
external  rectus  was  most  affected.  This  case  shows  that  paralysis  of  an 
external  rectus  may  be  a  distant  symptom,  and  I  have  seen  it  in  a  case 
of  tumor  of  the  parietal  lobe. 

The  occurrence  of  Jacksonian  epilepsy  from  a  tumor  situated  deeply 
in  the  brain  may  cause  a  mistake  in  diagnosis,  but  in  rare  instances  con- 
vulsions of  this  character  may  occur,  as  in  a  case  reported  by  J.  M. 
Clarke,  in  which  a  tumor  was  confined  to  the  optic  thalamus.  Paralysis 
nowhere  was  absolute,  and  in  the  face  was  slightly  marked,  and  there 
was  no  deviation  of  the  tongue.  There  was  rigidity  but  no  ankle  clonus, 
nor  exaggeration  of  reflexes.  Loss  of  control  over  bladder  and  rectum 
existed.  In  these  respects  the  group  of  symptoms,  he  says,  corresponded 
to  those  observed  by  Bastian.  In  additioli  there  was  left  ptosis,  dilated 
pupils,  with  loss  of  reaction  to  light,  turning  of  the  head  first  to  the  left 
and  afterward  to  the  right  side,  with  mobile  spasm  of  the  right  arm  and 
leg,  and  vasomotor  phenomena  of  the  right  arm. 

In  Clarke's  case  the  head  looked  towards  the  side  of  the  lesion  in  the 
earlier  stages  and  only  subsequently  was  directed  towards  the  healthy 
side. 

According  to  the  experiments  of  Schiff  and  Nothnagel  in  unilateral 


"  Bonhoeffer,  Monatsschrift  fur  Psychiatrie  und  Neurologie,  vol.  i.  p.  6. 
^'Stanley,  Brain,  1898,  vol.  xxi. 
26 


402  THE    EYE    AND    NERVOUS    SYSTEM 

lesions  of  the  thalamus  the  head  looks  towards  the  healthy  thalamus, 
while  on  the  side  of  the  healthy  thalamus  the  flexors  of  the  upper 
extremity,  and  on  the  side  of  the  lesion  the  extensors  of  the  upper 
extremity  appear  contracted. 

In  Clarke's  case  the  positions  of  the  right  arm  and  leg  exactly  corre- 
sponded to  those  commonly  observed  in  ordinary  cases  of  hemiplegia 
from  hemorrhage  into  the  internal  capsule,  and  he  believes  they  were 
accounted  for  by  pressure  of  the  tumor  on  the  inner  capsule.  During 
the  short  time  the  patient  lived  after  an  operation  the  symptoms  of 
paralysis  and  spasm  disappeared  in  the  face  and  arm,  and  almost 
entirely  in  the  leg.  Any  effect  obtained  by  operation  was  believed  to  be 
by  relief  of  pressure,  and  would  seem  to  indicate  that  pressure  on  the 
internal  capsule  was  the  essential  agent  in  the  production  of  paralysis 
and  spasm. 

Clarke  ^^  describes  in  this  case  convulsive  seizures,  beginning  in  the 
right  arm  and  affecting  the  right  arm  and  leg,  lasting  for  two  or  three 
minutes  and  followed  by  rigidity  of  these  limbs  for  the  same  length  of 
time,  or  of  spasm  confined  to  the  right  arm  occurring  at  irregular  in- 
tervals. These  attacks  seem  to  have  been  of  the  Jackson ian  type, 
although  the  lesion  was  in  the  optic  thalamus.  Later  the  spasmodic 
movements  of  the  right  upper  limb  were  continuous.  He  describes  also 
an  intention  tremor  among  the  early  symptoms. 

Usually  Jacksonian  motor  epilepsy  indicates  a  lesion  in  or  near  tlio 
motor  cortex,  but  I  have  found  this  sign  in  a  case  of  my  own,  caused  by 
a  tumor  deep  within  the  white  matter  of  the  brain  but  above  the  internal 
capsule. 

A  tumor  of  the  optic  thalamus  does  not  always  cause  disturbances 
'of  vision,  although  it  would  be  expected  to  do  so  because  of  the  probable 
situation  of  visual  fibres  within  the  pulvinar,  and  because  of  the 
liability  to  interference  with  the  flow  of  cerebrospinal  fluid  through 
the  aqueduct  of  Sylvius.  In  a  case  observed  by  Byrom  Bramwell,^* 
a  diagnosis  of  cortical  gumma  in  the  position  of  the  right  lower  limb 
centre  was  made  on  account  of  tenderness  over  the  leg  and  arm  centres 
on  the  right  side;  and  of  attacks  of  Jacksonian  epilepsy  occurring 
several  times  a  day,  the  spasm  beginning  in  the  left  big  toe;  and 
because  of  exaggeration  of  the  left  knee-jerk.  Other  symptoms  of 
tumor  were  present.  An  examination  at  the  necropsy  revealed  a  glio- 
sarcoma  about  the  size  of  a  small  egg  in  tlie  right  optic  thalamus  and 
encroaching  slightly  upon  the  motor  fibres  of  the  pyramidal  tract.     The 

"J.  M.  Clarke,  Lancet,  1891,  p.  1283. 

"Byrom  Bramwell,  Brain,  1899,  vol.  xxii.  p.  49. 


TUMORS  AND  OTHEE  BRAII^  LESIONS  403 

tumor  was  thought  to  have  produced  irritation  of  the  leg  fibres  of  the 
internal  capsule,  but  it  seems  very  strange  that  a  tumor  so  deep  within 
the  brain  as  in  this  and  in  Clarke's  case  should  have  caused  Jacksonian 
epilepsy.  Bramwell  says  that  in  four  cases  in  which  the  optic  thalamus 
was  invaded  by  a  new  growth  he  was  unable  to  detect  any  definite  symp- 
toms due  to  a  lesion  of  this  area,  unless  paroxysms  of  flushing,  which 
were  very  marked  in  one  case,  were  due  to  this  cause. 

In  a  case  of  tumor  of  the  posterior  portion  of  the  left  optic  thalamus 
and  pressing  on  the  inner  capsule,  reported  by  Major,^^  there  was  no 
optic  neuritis  and  no  color-blindness.  The  symptoms  were  paralysis 
of  the  right  limbs  and  right  side  of  the  face,  numbness  of  the  right 
side,  dilatation  of  the  right  pupil  and  right  homonymous  hemianopia. 

TUMORS    OF    THE    CORPORA    QUADRIGEMINA 
AND    CEREBRAL    PEDUNCLES. 

If  we  recall  the  structure  of  the  region  of  the  corpora  quadrigemina 
we  can  understand  that  ocular  palsies  are  usually  among  the  early  mani- 
festations of  tumor  of  this  part  of  the  brain.  The  nuclei  of  the  oculo- 
motor and  trochlear  nerves  lie  near  the  aqueduct  of  Sylvius,  and  there- 


KiG.  4.— Fibroma  on  the  corpora  quadrigemina.     The  right  ctrebral  hemisphere,  and  the  posterior 
part  of  the  pons  have  been  removed. 

fore  pressure  upon  these  nuclei,  or  direct  implication  of  them  by  a 
tumor  causes  partial  paralysis  of  the  muscles  of  the  eyeball  on  each  side. 
Ataxia  may  be  attributed  to  the  involvement  of  the  superior  cerebellar 


Brit.  Med.  Joiirn.,  April  16,  1892,  p.  818. 


404  THE    EYE    AND    NERVOUS    SYSTEM 

peduncles  which  decussate  below  the  aqueduct  and  form  the  red  nuclei. 
Disturbance  of  hearing  may  also  be  expected,  and  may  be  explained  by 
the  implication  of  the  posterior  colliculi  of  the  corpora  quadrigemina. 
The  inner  muscles  of  the  eye,  the  iris  and  ciliary  muscles,  may  escape, 
because  the  position  of  the  nuclei  for  these  muscles  is  probably  in  ad- 
vance of  those  for  the  external  muscles  of  the  eyeball,  in  fact,  rigidity 
of  the  iris  is  not  a  common  sign  of  tumor  in  this  part  of  the  brain,  and 
ptosis  from  involvement  of  the  levator  palpebrae  also  may  be  absent 
Inequality  of  the  pupils  has  been  observed  from  tumor  of  the  corpora 
quadrigemina.  Occasionally  absence  of  convergence-reaction  and  paral- 
ysis of  accommodation  have  been  seen.  Oppenheim  reports  recurrent 
ophthalmoplegia. 

In  Kolisch's  case  of  tubercle  of  the  corpora  quadrigemina  and  pons 
both  oculomotor  nerves  were  paretic,  but  the  pupillary  reaction  was 
preser\'ed.  The  tumor  did  not  extend  forward  beyond  the  corpora 
quadrigemina,  and  probably  for  this  reason  the  pupillary  reaction  was 
preserved. 

In  a  case  of  tuberculous  tumor  confined  to  the  corpora  quadrigemina 
reported  by  Bristowe,  the  tumor  lay  between  the  upper  surface  of  these 
bodies  and  the  aqueduct  of  Sylvius.  All  the  movements  of  the  eyes  were 
more  or  less  imperfect,  except  possibly  downward  movement.  The 
irides  reacted  well  to  light,  but  reaction  to  accommodation  could  not  be 
determined.  Mr.  Nettleship,  who  made  the  ophthalmological  examina- 
tion, thought  that  the  centres  for  the  iris  and  ciliary  muscles  had 
escaped.  This  case  also  seems  to  show  that  these  centres  are  in  advance 
of  the  other  oculomotor  centres. 

In  B.  Sachs's  case  of  tubercle  of  the  corpora  quadrigemina  there 
was  almost  complete  bilateral  external  ophthalmoplegia,  but  during  the 
entire  period  of  observation  the  ciliary  muscles  and  irides  remained 
exempt.  Sachs  regards  this  as  striking  proof  that  the  nuclei  of  these 
muscles  are  at  some  distance  from  those  governing  the  external  ocular 
muscles. 

The  internal  ophthalmoplegia  in  Gordinier's  case  from  a  lesion  far 
back  in  the  oculomotor  nuclei  is  remarkable,  as  the  nuclei  for  the  in- 
ternal ocular  muscles  are  usually  placed  well  forward,  by  L.  Bruns,  in 
the  median  side  of  the  optic  thalami. 

In  a  case  of  tumor  which  seems  to  have  been  confined  to  the  aque- 
duct of  Sylvius,  and  was  reported  by  J.  Collins,^"  the  gait  was  like  that 
of  an  intoxicated  person,  but  there  was  no  ocular  palsy  and  hearing  was 


'  Collins,  Amer.  Jour,  of  the  Med.  Sci.,  1895,  vol.  ex,  p.  420. 


Fig.  5. — Glioma  of  the  right  side  of  the  pons.  Attempt  to  converge.  Left  eye  is  moved  more  toward 
the  right  than  when  the  attempt  is  made  to  move  both  eyeballs  to  the  right  (  Fig.  7).  Marked 
deviation  of  lower  jaw  to  the  right  on  account  of  paralysis  of  the  right  pterygoid  muscles.  Associated 
ocular  palsies. 


Fig.  6.— Glioma  of  right  side  of  the  pons.  Attempt  to  look  upward  and  to  protrude  the  tongue. 
The  eyeballs  move  very  slightly  upward.  The  lower  jaw  goes  to  the  right,  the  tongue  to  the  left  when 
protruded.    Figs.  5,  7,  8  and  18  are  from  the  same  case. 


FiQ.  7. — Glioma  of  the  right  side  of  the  pons.   Attempt  to  look  to  the  right  and  to  separate  the  lips  with 
the  teeth  together ;  weakness  in  the  lower  distribution  of  the  seventh  nerve  on  each  side. 


FlQ.  8.— Glioma  of  the  right  side  of  the  pons. 


Fig.  9.— Case  seen  with  Dr.  J.  w.  McConnell.    Lesion  probably  near  the  aqueduct  of  Sylvius;  attempt 
to  converge ;  power  of  convergence  lost. 


Fig.  10.— Same  case  as  shown  in  Fig.  9.  Attempt  to  look  upward  shows  failure  of  eyes  to  move 
upward ;  wrinkling  of  forehead  in  the  attempt  is  well  shown  ;  paralysis  of  upward  associated  move- 
ments. 


TUMORS  A:N^D  other  BRAI:N^  lesions  405 

• 

good.  We  may  assume  that  pressure  from  the  tumor  was  not  sufficient 
to  cause  many  symptoms.  The  third  and  lateral  ventricles  were  much 
distended,  but  the  fourth  ventricle  contained  no  fluid  and  presumably 
was  not  dilated.  The  case  resembles  one  to  which  I  have  referred  in  my 
section  on  tumors  of  the  occipital  lobe,  of  occlusion  of  the  aqueduct  of 
Sylvius  by  proliferation  of  the  neuroglia. 

Associated  Ocular  Palsies.^^  —  Disturbances  in  the  associated 
movements  of  the  eyes  (Blicklahmung)  afford  a  sign  of  localizing  value 
in  lesions  of  the  brain,  and  yet  this  subject  has  received  comparatively 
little  attention.  Von  Kornilow,  who  has  written  the  best  recent  paper 
on  these  palsies,  remarks  that  clinical  cases  carefully  studied  are  rare, 
and  even  more  so  are  those  with  necropsy.     (See  Chapter  V,  p.  266.) 

The  opinion  of  Parinaud  and  Sauvineau  that  upward  or  downward 
associated  ocular  paralysis  is  always  associated  with  paralysis  of  con- 
vergence is  incorrect,  as  shown  by  a  case  reported  by  Parinaud  himself, 
by  a  case  reported  by  von  Kornilow,  and  by  other  cases.  It  is  true,  how- 
ever, as  Parinaud  pointed  out  in  1883,  that  in  paralysis  of  associated 
ocular  movements  diplopia  often  is  absent.  Indeed  when  in  such  palsy 
diplopia  later  appears  it  may  be  a  sign  of  improvement,  as  it  was  in 
a  case  observed  by  me,  where  the  disappearance  of  the  palsy  in  one 
eye  caused  diplopia,  soon  followed  by  complete  recovery.  Although 
paralysis  of  associated  ocular  movement  of  any  form  is  usually  a  sign 
of  grave  significance,  one  should  be  careful  about  giving  a  fatal  prog- 
nosis. In  a  case  reported  by  Raymond  and  Cestan  ^^  the  paralysis 
lasted  ten  years,  and  in  a  number  of  cases  complete  recovery  has  oc- 
curred, while  in  others  improvement  has  been  very  marked,  as  in  some 
of  the  cases  studied  by  me. 

The  evidence  is  strong  that  paralysis  of  associated  lateral  movements 
of  the  eyeballs  is  indicative  of  a  lesion  of  the  posterior  longitudinal 
bundle  near  the  sixth  nucleus.  The  external  rectus  muscle  on  the  side 
of  the  lesion  may  be  more  affected  than  the  internal  rectus  of  the  other 
eye,  and  this  according  to  Parinaud  ^^  is  the  rule.  He  mentions  also 
that  the  movement  of  convergence  may  be  greater  in  the  eye  in  which 
the  external  rectus  is  paralyzed,  because  of  the  paralysis  of  the  external 
rectus. 

The  disturbances  of  the  upward  or  downward  associated  movements 
have  been  studied  less  than  those  of  lateral  associated  movement.     The 

"  Adapted  from  the  author's  Presidential  Address,  read  at  the  meeting  of  the 
American  Neurological  Association,  June,  1905,  and  published  in  the  Journal  of 
Nervous  and  Mental  Disease.  July  and  August,  1905. 

"Eevue  Neurologique,   1901,  p.  70. 

^Archives  de  Neurologic,  vol.  v.  No.  14,  1883,  p.  145. 


40G  THE    EYE    AND    XERVOUS    SYSTEM 

former,  though  usually  acquired,  may  be  congenital,  and  Oppenheim  ^* 
has  observed  congenital  paralysis  of  associated  upward  movement. 
Uhthoff  -^  speaks  of  paresis  of  upward  associated  movement  with  slight 
impairment  of  movement  in  other  directions,  occurring  in  multiple 
sclerosis. 

We  have  reason  to  believe  that  the  cerebral  cortex  exerts  some  con- 
trol over  the  associated  movements  of  the  eyeballs;  especially  strong 
is  the  evidence  concerning  lateral  conjugate  movements.  Conjugate 
deviation  of  the  eyes  has  been  studied  within  the  past  few  years  by 
Klaas,^''  and  from  his  investigations  of  the  literature,  it  appears  that 
this  phenomenon  was  first  observed  by  Andral  (1834).  It  was  studied 
by  Duplay,  Durand-Fardel,  and  others,  but  Prevost  showed  that  con- 
jugate deviation  of  the  eyes  often  occurs  in  hemiplegia  and  has  nothing 
to  do  with  strabismus,  as  Andral  believed ;  he  showed  further  that  it 
is  often  associated  with  deviation  of  the  head;  that  the  deviation  is 
toward  the  lesion  when  the  lesion  is  cerebral,  and  away  from  the  lesiou 
when  it  is  in  the  isthmus.  Landouzy  believed  that  irritation  and 
paralysis  produce  deviation  in  opposite  directions  when  the  lesion  is 
in  the  cerebrum,  and  Grasset  showed  that  the  patient  looks  at  his 
paralyzed  limb  when  the  lesion  is  a  paralyzing  one  in  the  brain  stem, 
and  at  the  lesion  when  it  is  an  irritating  one,  and  in  this  part  of  the 
brain.  Wernicke  assumed  that  the  rapid  disappearance  of  conjugate 
deviation  of  the  head  and  eyes  is  owing  to  the  partial  control  of  the 
lateral  ocular  movements  by  the  cerebral  hemisphere  of  the  same  side, 
and  to  the  action  of  this  hemisphere  in  place  of  the  injured  opposite 
cerebral  hemisphere.  Wernicke  believed  that  conjugate  deviation  of 
the  eyes  is  caused  by  a  lesion  of  the  lower  parietal  lobe,  and  he  reported 
three  cases  of  lesion  in  the  lower  part  of  each  parietal  lobe  in  which 
voluntary  lateral  associated  ocular  movements  were  lost,  pseudo-ophthal- 
nioplegia,  although  in  another  case  with  similar  lesions  disturbance 
of  the  ocular  movements  was  not  observed. 

The  evidence  concerning  a  centre  for  lateral  movements  of  the 
eyes  in  the  lower  parietal  region  or  angular  gyrus  is  conflicting.  There 
is  more  evidence  that  a  centre  for  lateral  associated  ocular  movements 
is  situated  in  the  posterior  part  of  the  frontal  lobe. 

The  recent  investigations  of  Griinbaum  and  Sherrington  by  elec- 
trical   irritation   have    again    demonstrated    that    a    centre   for   ocular 


**  Lehrbuch  der  Nervenkrankheiten,  4th  edition,  vol.  ii.  p.  706. 
"Archiv.  fiir  Psychiatric,  vol.  xxi.  1890,  p.  379. 

"Uber    konjugierte    Augenablenkung    bie    Gehirnkrankheiten,    Inaugural  Dis- 
sertation,  Marburg,   1898. 


TUMOES  AND  OTHEE  BEAIN  LESIOI^S  407 

movements  probably  exists  in  the  frontal  lobe.  Pathological  alteration 
of  the  frontal  lobe  has  given  evidence  to  the  same  effect.  In  a  case 
of  cerebral  syphilis  with  severe  lesion  of  the  right  frontal  lobe,  reported 
by  Karl  Schaffer,^^  paralysis  of  associated  movement  of  the  eyeballs 
to  the  left  occurred,  and  Schaffer  concludes  from  a  study  of  this  case 
that  a  centre  for  lateral  associated  movements  exists  in  the  posterior 
part  of  tlie  second  frontal  convolution,  although  he  does  not  deny  that 
there  may  be  another  and  similar  centre  in  the  supramarginal  and 
angular  gy ri. 

Hemianopsia  cannot  be  regarded  as  the  cause  of  conjugate  devia- 
tion in  all  eases,  although  much  has  been  attributed  to  it  recently  by 
certain  French  writers.  Dejerine  and  Eoussy  ^^  have  demonstrated 
that  it  is  not  necessarily  the  cause  of  the  deviation,  inasmuch  as  devia- 
tion existed  in  a  patient  of  theirs  who  had  been  born  blind  and  whose 
■cortical  visual  zone  had  not  been  developed. 

So  far  the  knowledge  gained  relates  chiefly  to  associated  lateral 
movements,  but  we  know  much  less  concerning  a  cortical  centre  for 
upward  or  downward  associated  movements. 

The  views  of  Parinaud  and  some  other  writers  on  this  subject  lack 
•confirmation.  They  believed  that  there  is  a  centre  for  ocular  move- 
ments in  the  occipital  lobe  and  a  more  important  one  in  the  frontal 
lobe.  The  inferior  part  of  the  latter  centre  controls  the  upward  move- 
ment of  the  eyes,  the  superior  part  controls  the  downward  movement 
of  the  eyes,  the  intervening  part  controls  the  lateral  movements.  It  is 
true  that  lateral  associated  movements  have  been  impaired  with  paraly- 
sis of  upward  or  downward  associated  movements  in  a  number  of 
instances,  as  they  were  in  some  of  my  cases,  but  a  lesion  in  the  vicinity 
of  the  corpora  quadrigemina  will  better  explain  this  form  of  paralysis 
than  a  lesion  of  the  cerebral  cortex.  Teillais  says,  regarding  his  own 
case,  it  would  be  unreasonable  to  regard  the  lesion  as  cortical,  because 
if  the  lower  part  of  the  cortical  centre  controls  the  upward  move- 
ments, and  the  upper  part  the  downward  movements,  then  the  whole 
.of  this  centre  except  the  middle  portion  must  have  been  destroyed, 
as  the  lateral  movements  were  preserved.  Such  a  view  cannot  be 
accepted. 

Paralysis  of  lateral  associated  movement  from  cortical  lesion,  as 
seen  in  the  conjugate  deviation  of  the  eyes,  is  always,  so  far  as  I  know, 
transitory,  and  the  persistence  of  such  paralysis  points  to  a  lesion  of 
the    posterior    longitudinal    bundle.      We   have    little    anatomical    or 


"  Neurologisches  Centralblatt,  Nov.   16,   1904,  p.   1035. 
=^  Revue  Neurologique,  Feb.   15,  1905,  p.   161. 


408  THE    EYE    AND    NERVOUS    SYSTEM 

pathological  evidence  that  is  really  valuable  concerning  the  existence 
of  a  cortical  centre  for  upward  or  downward  associated  movements,  but 
in  reasoning  from  analogy  we  must  assume  that  such  a  centre  or  centres 
exist.  The  clinical  case  that  Parinaud  2"  reported  in  1892  as  indi- 
cating a  lesion  of  the  frontal  centre,  is  in  the  light  of  our  present  knowl- 
edge unsatisfactory  because  of  the  association  of  homonymous  lateral 
hemianopsia. 

Parinaud  reported  the  case  before  the  Society  of  Ophthalmology, 
April,  5,  1892.  The  man  had  paralysis  of  the  lower  left  part  of  the 
face,  of  the  left  half  of  the  tongue,  and  temporarily  of  the  left  hand. 
He  had  almost  complete  paralysis  of  the  elevators  of  the  eyeballs,  with 
a  little  reduction  in  the  extent  of  the  lateral  movements.  Convergence 
was  well  performed.  (Here  Parinaud  presents  a  case  of  paralysis  of 
upward  associated  movement  with  preservation  of  convergence.) 
Downward  movement  was  normal.  Parinaud  believed  there  was  a 
lesion  in  the  right  frontal  lobe,  at  the  level  of  the  lower  part  of  the 
Rolandic  fissure,  involving  the  centres  for  the  face,  tongue,  hand,  and 
associated  movements  of  the  eyeballs. 

This  patient  had  transitory  partial  left  homonymous  hemianopsia, 
and  this  was  supposed  to  be  the  result  of  a  lesion  near  the  visual  zone, 
which  occupies,  according  to  the  teaching  of  Parinaud  in  1892,  all  the 
occipital  lobe,  and  extends  to  near  the  ascending  parietal  convolution. 
This  opinion  we  now  can  hardly  accept. 

Hysteria  may  cause  paralysis  of  the  associated  ocular  movements, 
but  there  is  no  pathognomonic  sign  of  this  form  of  the  palsy,  and  in 
some  instances  a  correct  diagnosis  may  be  extremely  difficult  or  even 
impossible.  No  case  presents  this  difficulty  more  clearly  than  the  one 
reported  by  Crouzon,  Marie,  and  Babinski  ^^  before  the  Neurological 
Society  of  Paris  on  three  different  occasions  in  1900  and  1901. 

Crouzon  presented  the  case  as  one  of  spasmodic  tic,  later  Babinski 
and  Parinaud  expressed  the  opinion  that  it  was  organic  and  caused  by 
a  supranuclear  lesion,  and  still  later  Marie  presented  the  case  with  the 
diagnosis  of  a  purely  functional  disorder,  a  neurosis,  as  a  spasm  of 
the  elevators  of  the  eyeballs.  When  the  patient  threw  his  head  as  far 
backward  as  possible  and  followed  with  his  eyes  a  finger  lowered  slowly 
the  eyeballs  moved  downward,  but  if  his  head  were  erect  and  he  at- 
tempted on  command  to  look  at  his  feet,  he  bent  his  head  forward  and  the 
eyeballs  went  forcibly  upward.  After  thirty  or  forty  seconds  the  spasm 
disa])peared  and  the. eyeballs  returned  to  the  normal  position.     Marie 

"Annales  d'Oculistique,  vol.  cvii.  p.  283,  1892. 
"Revue  Neurologique,  1901,  p.  428. 


TUMORS  AND  OTHER  BRAIX  LESIOXS  409 

concluded  from  this  that  there  must  be  a  spasm  of  the  elevators  and 
not  a  paralysis  of  the  depressors.  The  disorder  had  occurred  after  an 
apoplectic  attack  lasting  seventeen  hours,  and  the  man  had  become 
blind.  During  the  attack  he  had  not  had  stertorous  breathing  nor  pas- 
sage of  urine  or  faeces,  and  this  Marie  regarded  as  remarkable  if  there 
were  an  organic  lesion.  The  coma  disappeared  suddenly,  but  the  patient 
was  delirious  for  several  weeks  and  did  not  recognize  anyone  or  his 
surroundings.  This  Marie  regarded  as  like  hysteria.  The  speech  was 
slow  and  hesitating,  and  the  visual  fields  were  contracted. 

Parinaud  pointed  out  that  the  downward  movement  in  following 
a  finger  is  a  reflex  act  and  better  performed  than  a  voluntary  move- 
ment, and  this  he  sa^'s  he  has  observed  in  hysteria,  but  when  the 
condition  is  hysterical  there  is  no  inconvenience  from  the  ocular  dis- 
turbance, just  as  there  is  no  inconvenience  from  the  contraction  of  the 
visual  fields  in  hysteria,  and  Parinaud  has  never  seen  a  hysterical 
patient  incline  the  head  forward  in  order  to  make  use  of  the  superior 
part  of  the  champ  du  regard  (Dr.  de  Schweinitz  suggests  as  a  suitable 
translation  for  this  term,  field  of  fixation).  The  dissociation  between 
the  voluntary  and  reflex  movements  is  not  always  hysterical.  Pari- 
naud, has  pointed  out  that  spasm  may  occur  in  sound  ocular  muscles 
as  the  result  of  paralysis  of  other  ocular  muscles  when  the  patient  is 
required  to  look  in  different  directions.  A  paralysis  confined  to  one 
eye  may  be  the  cause  of  spasm  in  the  other,  so  that  the  sound  muscles 
may  appear  to  be  affected.  In  these  cases  the  paralysis  is  peripheral, 
but  even  in  associated  palsies  it  is  not  rare  to  see  disturbance  of  the 
antagonistic  muscles.  In  conjugate  lateral  paralysis  from  lesion  of 
one  sixth  nucleus  there  is  almost  always  nystagmus  when  the  patient 
looks  toward  the  sound  side,  and  this  nystagmus  may  be  so  pronounced 
that  it  may  be  difficult  to  say  which  side  is  affected.  This  I  also  have 
noticed  in  a  case  of  complete  paralysis  of  lateral  associated  movements 
on  one  side  and  of  partial  paralysis  on  the  other.  In  the  case  which 
called  forth  so  much  discussion  in  the  ^N'eurological  Society  of  Paris 
the  visual  fields  were  contracted  only  in  the  inferior  portion  and  not 
concentrically,  therefore  the  contraction  was  not  like  that  of  hysteria. 
The  champ  visual  (visual  field)  Parinaud  distingiiishes  from  the  champ 
du  regard  (field  of  fixation).  He  believes  that  if  the  cortical  centre  for 
associated  movements  of  the  eyes  in  the  frontal  lobe  be  destroyed,  the 
voluntary  associated  movements  will  be  lost,  but  the  reflex  associated 
movements  will  be  preserved,  and  yet  little,  if  any,  clinical  evidence 
in  support  of  tliis  opiliion  can  be  found. 

Babinski  pointed  out  that  in  the  case  under  discussion  the  down- 


410  THE    EYE    AND    NERVOUS    SYSTEM 

ward  moveinent  of  the  eyes  was  imperfect  with  the  head  thrown  back. 
He  quoted  the  case  reported  by  Schroder  ^^  in  which  only  paralysis 
of  the  associated  downward  movement  persisted,  and  the  eyes  could 
not  be  lowered  below  the  horizontal  plane.  Any  attempt  to  look  down- 
ward caused  a  spasm  of  the  elevators  and  the  eyes  turned  upward. 

Oppenheim  ^^  has  observed  cases  of  pseudobulbar  paralysis  in  which 
lateral  movements  of  the  eyeballs  were  impaired  in  voluntary  innerva- 
tion, but  were  preserved  when  the  patient  tried  to  follow  an  object  or 
to  turn  toward  the  direction  from  which  a  sound  came. 

All  the  pathological  evidence  that  I  have  been  able  to  obtain  in 
cases  of  persistent  palsy  of  associated  upward  or  downward  movement 
is  indicative  of  a  lesion  near  the  aqueduct  of  Sylvius.  It  is  extremely 
doubtful  whether  a  lesion  confined  to  the  corpora  quadrigemina  and 
causing  no  pressure  on  the  surrounding  parts  ever  causes  paralysis  of 
associated  ocular  movements,  and  those  who  favor  such  a  view  have 
not  produced  proof  of  a  supranuclear  centre  in  this  part.  Bem- 
heimer  ^^  says  Topolanski  showed  that  electrical  irritation  of  the  cor- 
pora quadrigemina  of  rabbits  does  not  produce  ocular  movements,  and 
that  these  parts  could  be  removed  without  producing  symptoms.  He 
believed  that  there  is  a  centre  for  ocular  movements  near  the  oculo- 
motor nucleus. 

Bernheimer,  from  experiments  on  monkeys,  has  shown  that  the 
ocular  movements  do  not  depend  on  the  integrity  of  the  corpora  quad- 
rigemina, that  this  part  of  the  brain  does  not  contain  a  reflex  centre 
for  these  movements,  and  that  after  destruction  of  this  part  the  ocular 
movements  are  normal.  He  obtained  synergic  ocular  movements  from 
irritation  of  the  angular  gj'rus  both  before  and  after  he  had  removed 
the  corpora  quadrigemina.  He  thus  refutes  Prus's  statements  that  a 
centre  for  ocular  movements  exists  in  the  corpora  quadrigemina. 

Cases  have  been  reported  in  which  the  corpora  quadrigemina  have 
been  destroyed  without  disturbance  in  the  movements  of  the  eyeballs 
(Weinland,  Seidel,  Ruel,  Nissen,  cited  by  von  Kornilow). 

In  a  case  that  I  have  had  the  opportunity  of  studying  there  was  a 
complete  paralysis  of  right  associated  lateral  movements,  and  great 
impairment  of  left  and  of  upward  associated  movements.  The  paraly- 
sis of  lateral  associated  movements  is  to  be  explained  by  the  involve- 
ment of  both  posterior  longitudinal  bundles.     If  we  assume  that  the 


"  Quoted  by  Teillais,  Bui.  de  la  Soc.  Fran,  d'  Ophthal.,  1899,  p.  415. 

"  Neurologisches  Ccntralblatt,   1895,  p.  40. 

"  Wiener  klin.  Wochenschrift,  No.  52.  1899,  p.  1310. 


TUMORS  AND  OTHER  BRAIN  LESIONS  411 

nuclei  of  the  superior  rectus  and  inferior  oblique  muscles  are  in  the 
posterior  part  of  the  oculomotor  nucleus,  we  can  understand  why 
marked  impairment  of  upward  movement  was  present  in  this  case,  but 
this  is  contrary  to  the  views  held  by  some  concerning  the  position  of 
these  groups  of  nerve  cells. 

Siemerling^*  has  recently  remarked  that  the  numerous  experi- 
mental studies  of  Bach,  Bernheimer,  van  Gehuchten,  and  van  Biervliet 
have  not  led  to  uniform  conclusions.  The  oculomotor  nucleus  is  merely 
the  place  for  the  transference  of  impulses  and  we  are  not  in  a  position 
to  state  which  parts  are  concerned  with  the  innervation  of  the  individual 
muscles. 

Another  of  my  cases  is  further  evidence  that  the  nuclei  of  the 
superior  rectus  and  inferior  oblique  muscles  may  be  in  the  posterior 
part  of  the  oculomotor  nucleus,  because  there  was  paresis  of  upward 
associated  movement  and  the  nerve-cells  of  the  oculomotor  nuclei  were 
not  diseased,  but  the  posterior  part  of  the  nuclei  was  affected  by  press- 
ure from  the  tumor  in  the  pons,  and  the  aqueduct  of  Sylvius  was  com- 
pressed upon  the  left  side.  The  tumor  did  not  invade  the  nuclei  of 
the  third  and  fourth  nerves.  The  paralysis  of  left  associated  lateral 
movement  is  explained  by  the  almost  complete  destruction  of  the  left 
half  of  the  pons  by  a  tubercle,  and  thereby  involvement  of  the  posterior 
longitudinal  bundle.  The  left  sixth  nerve  was  also  much  degenerated.  In 
this  and  a  similar  case  studied  by  me,  downward  movement  was  normal. 
This  would  seem  to  be  evidence  that  the  nucleus  of  the  inferior  rectus 
muscle  must  be  in  advance  of  those  for  the  superior  rectus  and  inferior 
oblique  muscles,  as  in  each  case  the  tumor  grew  from  the  pons  and  did 
not  invade  the  oculomotor  nucleus. 

If  this  is  the  correct  position  of  these  nuclei,  a  lesion  developing 
ventrally  in  the  pons  as  shown  in  two  of  my  cases  may  by  pressure 
upon  the  posterior  part  of  the  oculomotor  nuclei  cause  paralysis  of 
associated  upward  movement,  as  a  result  of  injury  of  the  nuclei  for 
the  superior  rectus  and  inferior  oblique  muscles,  and  it  would  not  be 
at  all  necessary  to  assume  the  existence  of  a  co-ordinating  centre  for 
upward  associated  movement.  Paralysis  of  downward  associated  move- 
ment depends  on  impairment  of  the  inferior  rectus  and  superior  ob- 
liqr.G  muscles,  these  having  cells  of  origin  in  two  distinct  nuclei.  There 
must  be  a  close  association  between  the  nucleus  of  the  superior  oblique 
and  that  of  the  inferior  rectus  muscle  of  the  same  side,  and  possibly 
of  the  opposite  side.  It  is  probable  therefore  that  a  lesion  of  the  nuclei 
of  the  inferior  rectus  muscles  and  of  the  fibres  connecting  them  with 
"Archiv.  fur  Psychiatrie,  vol.  xl.  No.  1,  p.  61. 


412  THE    EYE    AND    NERVOUS    SYSTEM 

the  nuclei  of  the  trochlear  nerves  would  cause  paralysis  of  associated 
downward  movement,  and  it  seems  unnecessary  to  assume  that  a  co-ordi- 
nating supranuclear  centre  for  upward  or  downward  movements  exists 
in  the  corpora  quadrigemina  or  their  vicinity.  Indeed,  two  of  my 
cases  show  that  such  a  centre  is  improbable,  because  in  each  case  the 
lesion  causing  paresis  of  upward  associated  movement  was  posterior 
to  the  oculomotor  nucleus,  and  did  not  implicate  the  corpora  quadri- 
gemina. Three  of  the  cases  of  the  series  studied  by  me  afford  further 
evidence  against  a  centre  for  co-ordinate  ocular  movements  in  the  cor- 
pora quadrigemina.  In  one  the  paralysis  at  first  was  only  in  upward 
movements,  later  also  in  lateral  associated  and  in  downward  associated 
ocular  movements.  In  this  case,  therefore,  a  paralysis  at  first  confined 
to  one  form  of  associated  movement  later  extended  to  other  forms.  In 
another  case  the  paralysis  of  upward  associated  ocular  movements  dis- 
appeared, leaving  only  paresis  of  the  left  superior  rectus,  and  finally 
this  also  disappeared.  In  another  case  the  associated  palsy  dis- 
appeared, leaving  paralysis  of  muscles  only  on  one  side.  It  is  very 
common  to  have  a  palsy  of  one  or  more  branches  of  the  oculomotor 
nerve  in  association  with  paralysis  of  associated  upward  or  downward 
movement,  and  this  is  more  easily  explained  by  a  lesion  of  the  oculo- 
motor nucleus  than  by  a  cortical  lesion.  Cortical  centres  control  asso- 
ciated movements,  not  isolated  muscles. 

The  paralysis  of  the  sixth  nerve  occurring  with  paralysis  of  asso- 
ciated upward  or  downward  movements  has  been  attributed  by  Licht- 
heim  to  pressure  upon  the  nerve  at  the  base  of  the  brain.  The  opinion 
held  by  Parinaud,  although  supported  by  Duval,  Laborde,  and  Graux, 
can  hardly  be  accepted, — viz.,  that  the  sixth  nucleus  gives  nerve  fibres 
to  the  internal  rectus  muscle,  and  that  this  muscle  has  a  double  inner- 
vation, the  sixth  nerve  for  lateral  movement,  the  third  for  convergence. 

The  assumption  of  a  basal  co-ordinating  centre  above  the  oculomotor 
nucleus  for  ocular  movements  seems  forced.  The  lateral  associated 
ocular  movements  can  be  w^ell  explained  by  the  connection  formed  by 
the  posterior  longitudinal  bundle  between  the  nucleus  of  the  branch 
to  the  internal  rectus  muscle  and  the  nucleus  of  the  sixth  nerve.  It 
is  reasonable  to  suppose  that  certain  fibres  connecting  the  different  parts 
of  the  oculomotor  nucleus  with  one  another,  or  connecting  the  group 
of  nerve  cells  innervating  the  inferior  rectus  with  the  nucleus  of  the 
trochlear  nerve  has  a  co-ordinating  function  similar  to  that  of  the  pos- 
terior longitudinal  bundle,  and  that  this  function  may  be  disturbed 
by  a  small  lesion.  It  is  striking  that  the  paralysis  of  downward  asso- 
ciated movement  (necessitating  the  implication  of  two  separate  nuclei 


TUMOES  AND  OTHER  BEAIN  LESIOI^S  413 

of  ocular  muscles)  without  paralysis  of  upward  associated  movement 
is  exceedingly  rare,  far  more  so  than  is  the  isolated  paralysis  of  upward 
associated  movement. 

Conjugate  deviation  of  the  eyeballs  from  lesions  of  the  pons  is  not 
common  when  the  lesion  is  confined  to  one  side  of  the  pons.  In  a  case 
of  hemorrhage  into  the  tegmentum  of  the  pons  that  I  have  had  the 
opportunity  to  study  and  the  brain  from  which  I  have  examined,  in- 
ward deviation  of  only  the  right  eye  occurred  from  paralysis  of  the 
sixth  nerve.  One  gets  the  impression  that  conjugate  deviation  of  the 
eyeballs  away  from  the  side  of  the  lesion  is  common  in  lesions  of  one 
side  of  the  pons,  but  in  four  cases  of  lesions  of  this  part  observed  by 
me  (three  of  tumor  and  one  of  hemorrhage)  causing  paralysis  of  the 
external  rectus  muscle  of  the  same  side,  I  have  looked  for  the  conjugate 
deviation,  but  failed  to  find  it.  It  is  probable  that  in  most  cases  the 
side  of  the  pons  opposite  to  the  lesion  is  directly  or  indirectly  involved. 

Von  Monakow  ^^  believes  that  when  the  lesion  implicates  the  sixth 
nucleus  of  one  side,  apart  from  the  paralysis  no  disturbance, — i.e., 
no  deviation  occurs,  unless  it  is  the  result  of  secondary  contracture. 
I  believe  that  conjugate  deviation  is  rare  even  as  a  late  sign  of  paraly- 
sis of  one  sixth  nucleus.  Of  course  any  involvement  of  both  sixth 
nuclei  would  make  conjugate  deviation  impossible,  and  in  one  of  my 
cases  both  these  nuclei  were  affected. 

In  a  case  of  softening  of  the  dorsal  part  of  the  left  side  of  the  pons 
reported  by  Albert  Eansohoff  ^^  there  was  complete  paralysis  of  the 
associated  ocular  movement  to  the  left  and  of  convergence  movement 
of  the  right  eye.  The  right  external  rectus  muscle  was  not  overactive, 
but  the  left  internal  muscle  was  in  marked  spasm.  The  left  abducens 
nucleus  was  involved  in  the  lesion,  the  right  escaped,  but  the  right 
posterior  longitudinal  bundle  was  involved.  Eansohoff  mentions  that 
Wernicke  and  Jolly  have  shown  that  paralysis  of  the  external  rectus 
muscle  does  not  in  itself  cause  contraction  of  its  antagonist,  the  internal 
rectus,  and  that  in  the  cases  of  these  authors,  as  well  as  in  his  own,  there 
was  a  lesion  of  the  opposite  posterior  longitudinal  bundle.  In  Wer- 
nicke's case  a  tumor  had  destroyed  the  left  adducens  nucleus  and  invaded 
the  right  side  of  the  pons.  Both  eyeballs  were  persistently  turned  to 
the  right,  especially  the  left  eyeball. 

According  to  the  study  of  the  literature  by  Eansohoff,  contracture 
of  the  unparalyzed  antagonistic  muscles,  but  not  always  in  both  eyes, 
and  more  in  the  internal  rectus  muscle  on  the  side  of  the  lesion  than 

^  Gehirnpathologique,  p.  335. 

^Archiv.  fiir  Psychiatrie,  vol.  xxxv.  1901,  p.  416. 


414  THE    EYE    AND    NERVOUS    SYSTEM 

in  the  external  rectus  muscle  on  the  side  opposite  to  the  lesion,  was 
observed  in  the  cases  of  Wernicke,  Jolly,  Graux,  Meyer  (the  lesion 
did  not  extend  to  the  mid-line),  Spitzer  (slight,  and  after  one-half 
year  duration  of  the  lesion),  Mierzejewsky  and  Rosenbach,  Gebhard, 
and  RansohoflF. 

Ransohoff  says  that  if  the  posterior  longitudinal  bundle,  as  is  as- 
sumed by  most  authors,  contains  a  part  of  the  voluntary  fibres  for  the 
internal  rectus  muscle  of  the  opposite  side,  a  lesion  of  these  fibres,  as 
of  the  pyramidal  fibres,  must  cause  spastic  contracture  of  the  muscle 
they  innerv^ate.  Therefore,  according  to  Ransohoff,  contracture  of  the 
internal  rectus  muscle  on  the  side  of  the  lesion  indicates  that  the  pos- 
terior longitudinal  bundle  of  the  opposite  side  is  also  involved,  but 
this  contracture  probably  could  not  exist  if  the  nucleus  of  each  abdu- 
cens  were  destroyed. 

In  four  of  ray  cases  both  posterior  longitudinal  bundles  must  have 
been  much  affected  and  conjugate  deviation  did  not  occur  in  any. 

In  one  case  in  which  paralysis  of  lateral  movements  was  present, 
the  movement  of  either  eyeball  to  the  right  or  left  was  distinctly  greater 
when  one  eye  was  covered  than  in  associated  lateral  movements.  The 
individual  movements  of  the  eyeballs  were  therefore  greater  than  the 
associated  movements. 

One  of  my  cases  was  especially  interesting  in  that  syphilitic  infec- 
tion was  positive.  The  paralysis  of  associated  movement  was  peculiar; 
the  downward  movement  was  completely  lost  in  the  right  eye  and  much 
impaired  in  the  left;  the  upward  movement  was  slight  in  the  right 
eye  and  completely  lost  in  the  left ;  the  lateral  associated  movements 
were  nearly  normal.  Almost  all  the  ocular  movements  were  regained 
except  the  downward  movement  of  the  right  eye.  It  is  remarkable 
that  the  complete  paralysis  in  the  associated  movements  was  in  the 
downward  direction  in  one  eye  and  in  the  upward  direction  in  the 
other,  although  the  associated  movement  was  very  feeble.  It  is  re- 
markable that  the  associated  palsy  disappeared  leaving  paralysis  of 
downward  movement  of  the  right  eye. 

In  four  cases  of  my  series  with  necropsy  the  pupil  was  smaller  on 
the  same  side  as  the  lesion  of  the  pons.  This  alteration  of  the  size 
of  the  pupil  from  a  lesion  of  the  pons  is  difficult  to  explain,  but  the 
presence  of  the  sign  in  four  cases  shows  that  it  is  of  diagnostic  signifi- 
cance, and  also  that  the  fibres  controlling  the  iris  pass  through  the  pons. 

In  at  least  two  cases  nystagmus  was  present  in  looking  away  from 
the  side  of  the  chief  lesion.  This  was  caused  by  weakness  of  the  oppo- 
site external  rectus  muscle  from  pressure  upon  or  direct  involvement 


TUMOES  AND  OTHER  BRAIN  LESIONS  415 

of  the  nucleus  of  the  abducent  nerve.  This  sign  may  be  expected  when 
a  lesion  affects  the  dorsal  part  of  the  pons  and  destroys  the  function 
of  one  abducent  nucleus  and  weakens  that  of  the  other.  Nystagmus 
may  surely  be  the  result  of  paresis  of  ocular  muscles. 

I  have  studied  thirty-eight  cases  of  paralysis  of  upward  or  down- 
ward associated  movement  reported  in  the  literature,  not  including 
nine  of  my  own.  In  many  cases  certain  symptoms  are  not  referred  to, 
so  that  statements  regarding  the  frequency  of  symptoms  are  incomplete. 
Von  Kornilow  used  only  twenty  cases  of  the  twenty-seven  to  which  he 
found  references  in  drawing  his  conclusions.  As  a  result  of  my  study 
of  these  forty-seven  cases, — 

Paralysis  of  upward  associated  movement  without  paralysis  or 
paresis  of  downward  associated  movement  was  found  in  twenty-six 
cases  (Henoch,  Priestley  Smith  (2),  Nieden,  Gowers,  Parinaud  (2), 
Ormerod,  Reinhold,  Thomson,  Lichtheim  (3),  Hoesslin,  Bruner,  Mills, 
Nogues  and  Sirol,  Kornilow,  Posey,  Parkinson,  Cases  1,  2,  4,  5,  6,  8 
of  my  series). 

Paralysis  of  upward  associated  movement  without  paralysis  or  pare- 
sis of  downward  associated  movement  was  found  in  sixteen  cases  (Wer- 
nicke, Parinaud,  Hope,  Nothnagel,  Eisenlohr,  Verrey,  Sharkey,  Sau- 
vineau,  Teillais,  Gordinier,  Raymond  and  Cestan  (slight),  Kornilow, 
Cases  3,  4,  7,  9  of  my  series). 

Paralysis  of  upward  associated  movement  with  impairment  of 
lateral  movement,  often  developing  later,  was  found  in  fifteen  cases 
(Henoch,  Priestley  Smith  (2),  Lichtheim  (3),  Parinaud,  Poulard,  Ray- 
mond and  Cestan,  Kornilow,  Parkinson,  Cases  1,  2,  3,  and  4  of  my 
series). 

Paralysis  of  upward  associated  movement  without  impairment  of 
lateral  movement  was  found  in  twenty-two  cases  (Wernicke,  Nieden, 
Gowers,  Parinaud  (2),  Ormerod,  Hope,  Thomson,  Sharkey,  Sauvineau, 
Hoesslin,  Bruner,  Teillais,  Gordinier,  Nogues  and  Sirol,  Kornilow, 
Posey,  Cases  5,  6,  7,  8,  and  9  of  my  series). 

Paralysis  of  downward  associated  movement  without  paralysis  of 
upward  associated  movement  was  found  in  five  eases  (Gee,  Poulard, 
Curzon-Marie  and  Babinski,  Basevi,  Schroder),  but  in  none  of  my  nine 
cases. 

The  reaction  of  the  iris  was  found  to  be  impaired  in  fourteen  cases 
(Henoch,  Gowers,  Parinaud,  Thomson,  Eisenlohr,  Lichtheim,  Sharkey, 
Sauvineau,  Hoesslin,  Gordinier,  Mills,  Kornilow,  Posey,  Case  9  of  my 
series),  and  is  said  to  have  been  normal  in  four  cases  (Parinaud,  Teil- 
lais, Poulard,  Raymond  and  Cestan),  but  in  many  cases  no  statements 


416  THE    EYE    AND    NERVOUS    SYSTEM 

regarding  the  iritic  reflex  are  made,  and  in  all  my  nine  eases  the  iritic 
reflex  was  preserved. 

The  optic  nerve  was  found  to  be  affected  in  fifteen  cases  (Gowers, 
Thomsen,  Nothnagel,  Gee,  Eisenlohr,  Lichtheim  (3),  Sharkey,  Hoess- 
lin,  Gordinier,  Cases  1,  5,  7,  and  9  of  my  series),  and  normal  in  twelve 
cases  (Wernicke,  Nieden,  Parinaud'  (2),  Ormerod,  Reinhold,  Sauvi- 
neau,  Teillais,  Raymond  and  Cestan,  Mills,  Nogues  and  Sirol,  Posey). 

Convergence  was  impaired  in  fifteen  cases  (Parinaud  (2),  Sharkey, 
Sauvineaii,  Teillais,  Poulard,  Gordinier,  Raymond  and  Cestan,  Korni- 
low,  Basevi,  Cases  2,  4,  5,  8,  and  9  of  my  series),  and  normal,  or 
nearly  normal,  in  nine  cases  (Hope,  Parinaud,  Nogues  and  Sirol,  Kor- 
nilow,  Posey,  Cases  1,  3,  6,  and  7  of  my  series). 

Ptosis  was  found  in  seven  cases  (Reinhold,  Eisenlohr,  Gordinier, 
Kornilow,  Nothnagel,  Cases  2  and  8  of  my  series),  and  was  said  to 
be  absent  in  thirteen  cases  (Wernicke,  Parinaud,  Gee,  Sauvineau,  Teil- 
lais, Raymond  and  Cestan,  Cases  1,  3,  4,  5,  6,  7,  and  9  of  my  series). 

Necropsy  was  obtained  in  nineteen  cases  (Henoch,  Wernicke, 
Gowers,  Hope,  Reinhold,  Thomsen,  Gee,  Eisenlohr,  Lichtheim  (3), 
Sharkey,  Hoesslin,  Gordinier,  Nothnagel,  Basevi,  Cases  1,  2,  and  3 
of  my  series). 

In  all  of  these  cases  except  one  (Thomsen  ^^)  parts  about  the  aque- 
duct of  Sylvius  were  implicated,  and  in  Thomsen's  case  a  gumma  was 
found  in  the  cerebral  peduncles.  Thomsen  cannot  explain  why  the 
intense  degeneration  of  the  right  oculomotor  nerve  and  the  slight  de- 
generation of  the  left  oculomotor  nerve  caused  paralysis  of  only  up- 
ward movement.  It  is  possible  that  the  Nissl  method,  if  it  had  been 
employed,  would  have  shoAvn  marked  alteration  of  the  oculomotor 
nuclei,  possibly  as  a  toxic  effect  from  the  adjacent  gumma,  and  in  this 
way  the  paralysis  of  upward  associated  movement  may  have  been 
caused.  Such  was  the  conditon  in  one  of  my  cases,  unless  perhaps  the 
alteration  of  the  nerve  cells  was  caused  by  pressure.  Until  at  least  an- 
other case  similar  to  Thomsen's  is  reported  we  may  well  hesitate  to 
believe  that  a  peripheral  lesion  of  one  oculomotor  nerve  may  cause 
paralysis  of  only  upward  associated  movements. 

Tumor  was  found  in  fourteen  cases  (Henoch,  Gowers,  Hope,  Rein- 
hold, Gee,  Lichtheim  (3),  Sharkey,  Hoesslin,  Gordinier,  Nothnagel, 
Cases  1  and  2  of  my  series),  a  bullet  wound  in  one  case  (Eisenlohr), 
apoplectic  cyst  in  one  case  (Wernicke),  hemorrhage  in  one  case  (my 
Case  3),  and  lesion  uncertain  in  one  case  (Basevi). 


"Archiv.  fUr  Psychiatric,  vol.  xviii.  1887,  p.  616. 


TUMOES  AND  OTHEK  BEAIN  LESIONS  417 

Eeeovery  or  partial  recovery  occurred  in  seven  cases  (Priestley 
Smith,  jSTieden,  Verrey,  Kornilow,  Cases  5,  8,  and  9  of  my  series). 
Important  symptoms  other  than  ocular  palsies  were  found  in  forty-one 
cases  (Henoch,  Wernicke,  Priestley  Smith  (2),  Gowers,  Parinaud  (3), 
Hope,  Eeinhold,  Thomsen,  Nothnagel,  Gee,  Eisenlohr,  Lichtheim  (3), 
Yerrey,  Sharkey,  Sauvineau,  Hoesslin,  Bruner,  Teillais,  Poulard,  Gor- 
dinier,  Cruzon-Marie  and  Babinski,  Eaymond  and  Cestan,  Mills, 
Nogues  and  Sirol,  Kornilow  (2),  Schroder,  Posey, .  Parkinson,  Cases 
1,  2,  3,  4,  6,  8,  and  9  of  my  series). 

As  a  result  of  my  studies  I  believe  that  persisting  paralysis  of  asso- 
ciated lateral  movement  indicates  a  lesion  of  the  posterior  longitudinal 
bundle;  that  persistent  paralysis  of  associated  upward  or  downward 
movement  indicates  a  lesion  in  the  vicinity  of  the  oculomotor  nucleus, 
and  that  paralysis  of  associated  ocular  movements  is  not  the  result  of 
a  lesion  of  extracerebral  nerve  fibres.  Lesions  of  the  cerebral  cortex 
may  certainly  cause  paralysis  of  lateral  associated  ocular  movements, 
and  possibly  of  upward  or  downward  associated  ocular  movements,  but 
cortical  paralysis  of  associated  ocular  movements  is  transitory,  unless 
possibly  where  the  centre  on  each  side  of  the  brain  is  destroyed.  Paraly- 
sis of  associated  ocular  movements  may  be  caused  by  hysteria.  Any 
case  in  which  associated  ocular  palsy  is  persistent  and  is  of  organic 
nature,  is  unsuitable  for  operation  unless  the  operation  is  merely  pal- 
liative, as  the  lesion  is  probably  within  the  posterior  part  of  the  pons 
or  cerebral  peduncle,  according  to  the  form  of  the  associated  palsy,  or 
else  causes  much  pressure  upon  the  dorsal  portions  of  these  structures. 
The  paralysis  of  associated  ocular  muscles  may  be  produced  by  inflam- 
matory lesions  or  lesions  of  a  similar  character  (alcohol,  syphilis),  as 
well  as  by  tumor,  and  may  disappear  later  in  the  course  of  the  disease. 
Syphilitic  ependymitis  and  cellular  infiltration  must  be  considered  in 
diagnosing  the  lesion  causing  paralysis  of  associated  ocular  movements. 
Most  congenital  associated  palsies  are  probably  nuclear  in  origin. 

Drowsiness,  Nystagmus,  and  Impairment  of  Vision  as  Signs  of 
Tumor  of  the  Corpora  Quadrigemina. — Drowsiness  and  repeated 
yawning  are  not  uncommon  signs  of  a  tumor  of  the  corpora  quadri- 
gemina, but  are  not  pathognomonic. 

Nystagmus  also  occurs  with  tumor  of  the  corpora  quadrigemina  and 
sometimes  is  obtained  only  by  looking  upward. 

A  peculiar  form  of    nystagmus  is  described  by  Bielschowsky  as 

occurring  in  cases  of  tumor  of  the  corpora  quadrigemina.     It  consists 

of  clonic  movements  of  both  internal  rectus  muscles,  and  seems  to  have 

been  much  the  same  form  of  movement  as  that  observed  by  Eansom. 

27 


418  THE    EYE    AND    NERVOUS    SYSTEM 

Bielschowsky  refers  to  Blanquinque  as  having  described  in  1871  con- 
vulsive movements  of  the  eyeballs  as  a  sign  of  tumor  of  the  corpora 
quadrigeniina. 

Vision  may  not  be  disturbed  at  all  by  a  tumor  of  the  corpora  quad- 
rigemina,  and  when  disturbed  vision  results  from  optic  neuritis  the 
latter  probably  is  more  frequently  caused  by  internal  hydrocephalus. 
Implication  of  the  anterior  colliculi  of  the  corpora  quadrigemina  prob- 
ably does  not  cause  marked  disturbance  of  vision,  but  the  external 
geniculate  body  of  one  side  may  be  involved  and  give  rise  to  hemianopsia 
if  the  tumor  grows  forward. 

In  a  case  of  tubercle  of  the  corpora  quadrigemina  with  tuberculous 
meningitis,  reported  by  Nissen,  the  failure  of  vision  seemed  to  be 
caused  by  the  optic  neuritis  from  meningitis,  and  not  by  the  tubercle  of 
the  corpora  quadrigemina. 

In  W.  Goldzieher's  case  of  tubercle  of  the  corpora  quadrigemina 
the  symptoms  were:  ataxic  gait,  drowsiness,  complete  paralysis  of  all 
external  muscles  supplied  by  each  oculomotor  nerve  (the  movements 
of  the  iris  were  normal  and  accommodation  also  seemed  to  be  normal, 
as  the  child  recognized  small  objects  held  near  its  eyes;  the  eye- 
grounds  were  normal),  weakness  of  the  lower  limbs,  frequent  yaA\Tiing, 
sudden  and  severe  involuntary  jerkings  of  the  whole  body,  etc.  The 
child  was  five  years  old. 

The  necropsy  showed  a  tubercle  of  the  corpora  quadrigemina  about 
the  size  of  a  hazel  nut.  The  aqueduct  of  Sylvius  was  somewhat  nar- 
rowed by  pressure,  but  the  gray  matter  about  the  ventricles  did  not 
appear  to  be  implicated. 

The  case  shows,  according  to  Goldzieher,  that  a  tumor  involving  the 
corpora  quadrigemina  entirely  may  cause  no  blindness.  He  refers  to 
Nothnagel  as  having  spoken  of  this  fact  in  his  Topischen  Diagnostik  der 
Gehirnkrankheiten,  pp.  211-214.  Goldzieher's  patient  saw  well,  and 
accommodated. 

In  Bielschowsky's  case  the  anterior  portion  of  the  corpora  quadri- 
gemina was  completely  degenerated,  and  each  pulvinar  was  much  com- 
pressed, but  vision  was  only  a  little  impaired,  and  the  visual  fields  were 
not  contracted.  The  external  geniculate  bodies  were  preserved,  and  to 
the  integrity  of  these  structures  was  due  the  preservation  of  vision. 

In  some  cases  of  tumor  of  the  corpora  quadrigemina  the  abducens 
nerve  has  been  paralyzed,  as  in  a  case  observed  by  W.  B.  Ransom,^  in 
which  there  was  a  large  oval-celled  sarcoma  of  the  corpora  quadrigemina. 


Lancet,  May  4.   1805,  p.   111.5. 


TUMOKS  AND  OTHER  BRAIN  LESIONS  419 

Ophthalmoplegia  was  well  developed  long  before  marked  ataxia  set  in. 
In  the  earlier  stage  the  diagnosis  between  growth  from  the  base  of  the 
skull,  of  the  corpora  quadrigemina  or  pineal  gland,  and  of  the  cere- 
bellum, was  not  certain.  Paralysis  of  the  sixth  nerves,  loss  of  knee- 
jerks,  clonic  spasm  of  the  upper  lids  and  internal  rectus  muscles,  espe- 
cially of  the  left,  were  observed.  The  disturbance  of  vision  in  this  case 
was  regarded  by  Ransom  as  unusual,  as  in  most  cases  such  loss  has  been 
slight  or  very  late  in  appearing.  It  could  not  in  his  case  have  been  due, 
he  says,  to  implication  of  the  optic  thalamus  or  optic  radiations.  It  is 
possible  that  the  optic  neuritis  caused  it,  but  a  similar  amount  of 
effusion  is  frequently  seen  witli  little  or  no  loss  of  vision.  The 
paralysis  of  each  external  rectus  is  interesting  as  the  pons  was  normal. 
An  explanation  for  this  paralysis  is  not  given,  but  the  case  is  another 
instance  of  the  paralysis  of  the  abducent  nerve  when  the  lesion  is  at  a 
distance  from  its  nucleus. 

Optic  neuritis  was  absent  in  a  case  of  tumor  in  the  corpora  quadri- 
gemina observed  by  Frederick  Taylor,^^  in  which  the  cerebellum  was 
perfectly  healthy,  but  the  corpora  quadrigemina  were  flattened,  broader, 
and  gray  and  gelatinous  in  appearance,  and  were  the  seat  of  a  new 
growth,  regarded  as  a  glio-sarcoma.  The  symptoms  were:  j^tosis,  stag- 
gering gait,  drowsiness,  ataxia  of  the  upper  limbs,  nearly  complete 
double  ophthalmoi^legia,  lateral  nystagmus  and  later  unconsciousness. 
The  j)upils  were  slightly  and  unequally  dilated  and  reacted  to  light, 
but  the  child  could  not  see. 

Nissen  ^°  has  made  the  peculiar  observation  that  tumor  of  the  brain 
in  children  is  less  likely  to  cause  choked  disks  than  is  tumor  in  adults. 
In  his  five  cases  of  tumor  of  the  corpora  quadrigemina  or  cerebral 
peduncle,  optic  neuritis  was  present  only  in  one,  and  in  this  case  there 
was  meningitis.  Xissen  mentions  as  cases  of  tumor  of  the  corpora  quad- 
rigemina with  normal  eyegrounds  those  of  Taylor,  Goldzieher,  Bruns, 
and  Ilberg. 

Resemblance  between  the  S3nnptoms  of  Tumor  of  the  Corpora 
Quadrigemina  and  those  of  Multiple  Sclerosis. — The  symptoms  of 
tumor  of  the  corpora  quadrigemina  may  resemble  closely  those  of  mul- 
tiple sclerosis,  and  the  differential  diagnosis  may  be  exceedingly  difli- 
cult.  For  example,  in  Bielschowsky's  case  *^  the  temporal  side  of  each 
disk  was  pale  as  in  multiple  sclerosis.  This  pallor  was  explained  as 
secondary  atrophy  from  pressure  on  the  chiasm  by  the  distended  third 

=»  Taylor,  Lancet,  November  18,  1893,  vol.  ii.  p.  1252. 

*°W.  Nissen  Jahrbuch  fiir  Kinderheilkunde,  1901,  vol.  liv. 

*' Bielschowsky,  Deutsche  Zeitschrift  fiir  Nervenheilkunde,  xxii.  1  and  2,  p.  54. 


420 


THE    EYE    AND    NERVOUS    SYSTEM 


ventricle.  Pressure  on  the  chiasm  may  cause  atrophy  without  first  pro- 
ducing choked  disks.  This  temporal  pallor  of  the  disks  is  confusing, 
especially  in  cases  of  tumor  of  the  corpora  quadrigeniina,  as  Bielschow- 
sky  points  out,  because  incoordination  is  also  present,  and  the  diagnosis 
of  multiple  sclerosis  is  likely  to  be  made. 

Nystagmus  and  coarse  intention  tremor  in  Gordinier's  case  were  sug- 
gestive of  multiple  sclerosis,  but  Gordinier  believed  that  severe  head- 
ache, optic  neuritis,  ophthalmoplegia,  and  the  cerebellar  gait,  together 


Fio.  11.— Tumor  of  orbit  causing  ptosis  of  left  upper  eyelid,  almost  immobile  pupil,  complete  paral- 
ysis of  superior  rectus  and  inferior  rectiis,  partial  paralysis  of  internal  rectus,  some  protrusion  of  eye- 
ball, and  blurring  of  optic  disk  on  the  left  side.  The  photograph  shows  an  attempt  to  look  to  the  right. 
The  only  sign  of  intracranial  disturbance  was  exaggeration  of  the  patellar  reflexes.  Had  the  tumor 
grown  more  within  the  cranium,  the  limbs  of  the  right  side  would  have  been  paralyzed  in  association 
with  the  left  oculomotor  paralysis. 

with  the  absence  of  scanning  speech,  of  epileptiform  attacks,  and  of 
evidence  of  motor-tract  involvement,  negatived  such  a  diagnosis.  This 
opinion  can  not  be  accepted  unconditionally.  To  my  mind  the  absence 
of  involvement  of  the  motor  tract  was  the  best  clinical  evidence  that 
the  disease  was  not  multiple  sclerosis.     Headache,  optic  neuritis,  cere- 


TUMORS  AND  OTHER  BR'AIN  LESIONS 


421 


bellar  gait  may  all  occur  in  multiple  sclerosis,  and  I  have  shown  by  a 
case  with  necropsj-  that  ophthalmoplegia  may  also  be  a  sign  of  the 
disease.  Scanning  sjieech  and  epileptiform  attacks  are  not  present  in 
every  case  of  multiple  sclerosis. 

Symptoms  from  Implication  of  the  Cerebral  Peduncle. — When  the 
tumor  extends  far  enough  ventrally  to  involve  the  foot  of  the  cerebral 
peduncle  it  may  cause  paresis  or  paralysis  of  the  limbs  on  the  side 
opposite  to  the  lesion,  with  involvement  of  the  oculomotor  nerve  on 


Fig.  12.— Same  patient  as  shown  in  Fig.  11.    Complete  left-sided  ptosis.    (Case  seen  by  author  with 

Dr.  S.  D.  Risley.) 

the  same  side  as  the  lesion.  Various  forms  of  tremor  may  be  present 
in  the  paretic  limbs,  the  involuntary  movements  may  have  the  charac- 
teristics of  intention  tremor,  or  may  be  choreiform  or  athetotic. 

In  a  case  that  B.  Sachs  *^  reports,  one  of  thrombosis  of  the  posterior 
cerebral  artery,  the  right  cms  was  small  and  very  soft,  and  the  right 
oculomotor  nerve  was  as  thin  as  a  thread.  Left  hemiplegia  had  been 
present.     The  left  upper  limb  ha^i  shown  the  wildest  ataxic  movements, 


"  Sachs,  Amer.  Jour,  of  the  Med.  Sci.,  1891,  vol.  ci.  p.  233. 


422  THE    EYE    AND    NERVOUS    SYSTEM 

reminding  one  of  the  extreme  incoordination  of  the  lower  limbs  in 
the  last  stages  of  tabes  dorsalis.  If  the  patient  were  asked  to  use  the 
paralyzed  hand  the  paretic  limb  was  whirled  about  so  violently  as  to  be 
a  source  of  some  danger  to  those  near.  After  a  little,  the  arm  would  fall 
down  from  exhaustion,  and  remain  quiet  until  aroused  again. 

In  a  case  observed  by  Bristowe,  *^  all  the  limbs  were  weak  but  not 
paralyzed,  and  were  tremulous  on  movement;  this,  according  to  Bris- 
towe, seemed  to  indicate  compression  and  irritation  of  the  motor  tracts. 

In  another  case  a  tuberculous  growth  occupied  the  third  ventricle 
and  implicated  both  optic  thalami.  Tremors  like  those  of  disseminated 
sclerosis  and  drowsiness  were  present  in  this  case. 

Kolisch  **  says  (1893)  that  ataxic  gait  has  been  observed  in  two- 
thirds  of  the  cases  of  tumor  of  the  corpora  quadrigemina.  Peculiar 
persisting  choreiform  movements  of  the  left  upper  and  lower  extremi- 
ties, but  ceasing  during  sleep,  were  observed  even  when  his  patient,  a 
child,  was  lying  on  her  back,  and  were  more  intense  when  she  was  ex- 
cited, sat  up,  or  attempted  to  walk.  These  movements  were  attributed 
by  Kolisch  in  his  case  to  lesion  of  the  tegmentum  of  the  cerebral 
peduncle  and  irritation  of  the  pyramidal  tract. 

Gordinier  says  that  in  five  of  the  eleven  cases  in  literature  (Bruns, 
Weinland,  Eisenlohr,  Ilberg,  Bonhoffer)  in  which  lesions  of  the  corpora 
quadrigeminal  region  were  accompanied  by  tremor  or  choreiform  move- 
ments, the  superior  cerebellar  peduncles  were  involved.  Both  Bruns  and 
Weinland  attribute  the  tremor  to  irritation  of  the  motor  fibres  of  the 
pyramidal  tracts,  although,  as  Gordinier  mentions,  in  the  cases  they 
report  the  necropsies  showed  the  motor  tracts  to  be  normal,  while  the 
superior  cerebellar  peduncles  were  diseased.  Gordinier  refers  to  tlie  ex- 
periments of  Ferrier  and  Turner,  who  described  similar  tremors  result- 
ing from  division  of  the  superior  cerebellar  peduncles  in  monkeys.  They 
found  that  if  a  peduncle  were  divided  between  the  cerebellum  and  its 
decussation  in  the  tegmentum,  the  tremor  was  confined  to  the  side  of 
the  lesion.  I  have  referred  in  my  section  on  tumors  of  the  optic  thala- 
mus to  involuntary  movements  caused  by  implication  of  the  superior 
cerebellar  peduncles. 

S3miptom-Complex  of  Benedickt. — Paralysis  of  the  oculomotor 
nerve  on  one  side  and  of  the  limbs  on  the  opposite  side,  with  tremor 
of  any  variety,  has  been  called  the  symptom-complex  of  Benedickt,  but 
is  hardly  to  be  expected  in  a  typical  form  in  tumor  of  the  corpora 
quadrigemina,  as  tlie  ocular  nuclei  are  situated  so  close  together  that 

"Bristowe,  Brain,  vol.  vi.  p.  167. 

"  R.  Kolich,  Deutsche  Zeitschrift  fiir  Nervenheilkunde,  vol.  iv.  p.  14. 


TUMORS  AND  OTHER  BRAIN  LESIONS  423 

the  paralysis  of  the  ocular  muscles  is  usually  bilateral,  although  not 
commonly  symmetrical.  In  order  to  have  paralysis  of  the  limbs  the 
tumor  must  in  some  way  affect  the  motor  fibres  in  the  foot  of  the 
cerebral  peduncle. 

In  a  very  interesting  case  of  tubercle  of  the  right  cerebral  peduncle, 
reported  by  Blocq  and  Marinesco,"*"^  there  were  symptoms  like  those  of 
paralysis  agitans.  The  diagnosis  of  paralysis  agitans  was  made  during 
the  life  of  the  patient.  The  immobile  face  was  like  that  occurring  in 
this  disease.  A  rhythmical  tremor  of  small  oscillations  was  seen  in  the 
left  hand,  the  left  fingers  were  half  flexed,  the  left  lower  limb  trembled 
also  but  less  severely.  Voluntary  effort  arrested  the  tremor  temporarily. 
The  authors  believe  that  this  was  not  paralysis  agitans  occurring  in  a 
case  of  tumor  of  the  cerebral  peduncle,  because  of  the  exaggeration  of 
the  reflexes  on  the  left  side  and  the  exact  correlation  of  the  symptoms 
with  the  localization  of  the  tumor.  Exaggeration  of  tendon  reflexeS; 
however,  does  occur  in  paralysis  agitans. 

They  refer  to  a  case  of  Mendel  in  which  intention  tremor  of  the  right 
arm  was  found  associated  with  a  tubercle  of  the  left  cerebral  peduncle. 
Charcot  observed  a  case  in  which  tremor  like  that  of  Parkinson's  disease, 
with  typical  attitude  of  one  of  the  upper  limbs,  was  associated  with  a 
tumor  compressing  one  of  the  cerebral  peduncles. 

Deafness  as  a  Sign  of  Lesion  of  the  Corpora  Quadrigemina. — 
Weinland  *^  has  made  an  important  study  concerning  the  effect  of 
lesion  of  the  posterior  coUiculi  of  the  corpora  quadrigemina  upon  hear- 
ing. In  nineteen  cases  of  tumor  of  the  corpora  quadrigemina  disturb- 
ance of  hearing  was  observed  in  nine  cases, — i.e.,  in  about  half  of  the 
cases  (Duffin,  Klebs-Fischel,  Gowers,  Ferrier,  Fischer,  Hope,  Noth- 
nagel,  Ruel,  Weinland).  Disturbance  of  hearing  was  observed  in  four 
cases  out  of  nine  of  some  other  form  of  lesion  of  the  corpora  quadri- 
gemina (Hintz,  Reinhold,  Henschen,  Zenner).  In  five  of  the  nine  cases 
of  tumor  af  this  region,  with  disturbance  of  hearing,  the  deafness 
was  bilateral,  although  not  always  equal  on  the  two  sides.  In  three 
cases  (Ferrier,  Ruel,  Weinland)  it  was  shov^Ti  that  diseases  of  one  pos- 
terior colliculus  causes  disturbance  of  hearing  on  the  opposite  side. 
This  is  explained  by  the  course  of  the  fibres  concerned  in  hearing. 
Fibres  connecting  with  the  cochlear  nerve  form  the  lateral  fillet,  which 
passes  to  the  posterior  colliculi  of  tlie  corpora  quadrigemina;  from 
here  other  fibres  pass  to  the  internal  geniculate  body  and  the  first  and 
possibly  the  second  temporal  convolutions. 

"  Blocq  and  Marinesco,  Memories  de  la  Soc.  de  Biologie,  1893,  p.  105. 
"  Weinland.  Archiv.  fiir  Psych.,  1894,  vol.  xxvi.  p.  353. 


424  THE    EYE    AND    NERVOUS    SYSTEM 

Weinland  shows  that  the  cases  of  tumor  of  the  corpora  quadrigemina, 
in  which  deafness  was  not  observed,  do  not  lessen  the  importance  of  the 
posterior  colliculi  in  the  function  of  hearing. 

In  Gordinier's  case  "'^  hearing  was  normal  although  the  posterior 
colliculi  of  the  corpora  quadrigemina  were  almost  destroyed.  The 
patient  heard  ordinary  conversation  distinctly  and  the  watch  three 
feet  away  from  each  ear.  This  was  explained  by  the  absence  of  impli- 
cation of  the  lateral  fillet  of  either  side  or  of  the  nucleus  about  which 
its  fibres  terminate.  In  Bielschowsky's  case  also  the  posterior  col- 
liculi of  tlie  corpora  quadrigemina  were  much  compressed,  and  yet 
hearing  was  not  positively  affected  thereby,  so  far  as  could  be  deter- 
mined, although  the  patient  felt  as  though  the  hearing  had  become  less 
acute. 

Diagnosis  between  Lesions  of  the  Corpora  Quadrigemina  and 
those  of  the  Cerebellum — Extension  of  the  tumor  forward  so  as  to 
involve  the  visual  system,  either  the  optic  tract,  external  geniculate 
body,  or  the  optic  thalamus,  causes  homonymous  lateral  hemianopsia. 
Extension  backward  into  the  pons  may  result  in  paralysis  of  the  motor 
and  sensory  portions  of  the  trigeminal  nerve,  and  of  the  facial  and 
abducens. 

L.  Bruns  ^^  emphasizes  the  difficulty  in  diagnosing  between  tumors 
of  the  corpora  quadrigemina  and  those  of  the  cerebellum,  as  ataxia  and 
ophthalmoplegia  may  be  caused  by  a  tumor  in  either  location.  A  tumor 
of  the  vermis  is  very  likely  to  cause  cerebellar  ataxia,  and  by  pressure 
upon  the  corpora  quadrigemina  may  cause  ocular  palsies. 

He  believes  on  theoretical  grounds  it  is  possible  that  a  tumor  else- 
where within  the  brain,  for  example  in  the  frontal  lobe,  may  cause 
ataxia  with  paralysis  of  ocular  muscles.  ^Yhere  the  symptoms  begin 
with  ataxia  Bruns  thinks  the  tumor  is  probably  in  the  cerebellum ;  where 
they  begin  with  ophthalmoplegia  the  tumor  is  probably  in  the  corpora 
quadrigemina.  This  is  entirely  contrary  to  Nothnagel's  teaching,  as  the 
latter  regards  ataxia  occurring  as  the  first  symptom  before  the  ophthal- 
moplegia as  especially  diagnostic  of  tumor  of  the  corpora  quadrigemina. 
Bruns  mentions  as  a  diagnostic  point  that  the  escape  of  the  abducent 
nerves  would  be  more  in  favor  of  tumor  of  the  corpora  quadrigemina 
than  of  tumor  of  the  cerebellum,  but  paralysis  of  these  nerves  may  occur 
with  tumor  in  either  place.  Implication  of  other  cranial  nerves,  as  of 
the  facial,  would  indicate  rather  tumor  of  the  vermis.    Ataxia  or  chorei- 


"  Gordinior,  Journal  of  Nervous  and  Mental  Disease,  October,  1901,  p.  543. 
*"  Bruns,   Die   GeschwOlste   des   Nervensystems,   Archiv.   fiir   Psych.,    1894,   vol. 
xxvi.  p.  299. 


TUMORS  AND  OTHER  BRAIN  LESIONS  425 

form  movements  would  indicate  rather  tumor  of  the  corpora  quadri- 
gemina. 

Nissen  says  that  Nothnagel  believed  the  posterior  colliculi  of  the 
corpora  quadrigemina  are  concerned  in  co-ordination.  Others  believe 
the  ataxia  is  caused  by  involvement  of  the  red  nucleus  or  superior  cere- 
bellar peduncle ;  still  others  attribute  the  ataxia  to  pressure  on  the  cere- 
bellum. In  Nissen's  case  there  was  no  ataxia  and  a  tubercle  was  con- 
fined to  the  corpora  quadrigemina  and  implicated  only  the  upper  part 
of  the  tegmentum.  There  was  also  no  disturbance  of  hearing,  although 
the  posterior  colliculi  of  the  corpora  quadrigemina  were  implicated. 
Nissen  thinks  that  the  corpora  quadrigemina  may  be  a  reflex  area, 
in  which  the  acoustic  system  is  connected  with  the  nuclei  for  ocular 
movements.  He  thinks  also  that  at  least  in  some  of  the  cases  of  tumor 
of  the  posterior  colliculi  with  deafness  the  tumor  may  have  pressed 
upon  the  internal  geniculate  body,  or  may  have  caused  the  deafness  by 
distant  pressure  upon  the  medulla  oblongata  and  acoustic  nerve. 

In  this  remarkable  case  of  Xissen  there  vv'as  neither  ataxia  of  limbs 
nor  paralj'sis  of  the  ocular  nerves,  and  Nissen  shows  that  the  symptoms 
recognized  by  Nothnagel  (ataxic  gait  and  ophthalmoplegia)  depend 
upon  implication  of  the  tegmentum. 

In  two  cases  of  tumor  of  the  corpora  quadrigemina  observed  by  W. 
Aldren  Turner,^^  staggering  gait  combined  with  diplopia  and  headache 
varying  in  position  were  the  chief  symptoms.  In  one  case  the  inclina- 
tion was  invariably  backward,  so  that  the  patient  was  unable  to  stand 
or  even  to  sit  up  in  bed  without  support  from  behind.  The  ocular  symp- 
toms consisted  in  paresis  of  the  third  and  sixth  nerves  in  one  case,  and 
defect  in  the  upward  and  downward  movements  of  the  globes  in  the 
other.  Optic  neuritis  was  present  in  both  cases.  In  one  case  the  knee- 
jerks  were  lost  and  in  the  other  they  w^ere  diminished.  Both  of  these 
cases  showed  ataxia  of  cerebellar  character  with  impairment  in  the 
movements  of  the  eyeballs. 

Persisting  subnormal  temperature  was  observed  in  a  case  of  glioma 
of  the  mid-brain  and  left  optic  thalmus  reported  by  Lloyd.^^  The 
paralysis  was  crossed,  the  left  third  nerve  being  involved,  with  right 
brachial  monoplegia  and  right  crural  monoparesis.  The  patient  had  a 
remarkable  course  of  subnormal  temperature,  the  lowest  point  reached 
being  95.4°.  The  temperature  was  subnormal  for  twenty  days,  with 
two  exceptions. 


*»  Turner,  Brain,  1898,  vol.  xxi. 

«>  Lloyd,  Med.  News,  January  30,  18«)2,  p.  129. 


426  THE    EYE    AND    NERVOUS    SYSTEM 

The  headache  in  tumor  of  the  corpora  quadrigemina  is  not  so  severe 
and  frequent  as  in  cerebellar  tumor,  according  to  Nissen,  and  is  not  so 
well  localized  in  the  occiput.  Vomiting  seems  to  be  one  of  the  early 
symptoms.  Only  in  a  few  cases  were  forced  positions  and  forced  move- 
ments observed. 

LESIONS   OF   THE   PINEAL   GLAND. 

Oppenheim  '^^  is  very  skeptical  regarding  the  relation  of  the  pineal 
gland  to  the  sexual  organs.  Ileubner,  Slawzk,  and  Oestreich  believe 
that  early  and  excessive  development  of  the  penis  or  mammary  gland 
occurs  with  tumor  of  the  pineal  gland.  Sexual  excitement  and  priapism 
are  mentioned  by  Biancone,  Gutzeit,  and  Henrot.  Neumann  also  be- 
lieves a  relation  exists  between  the  pineal  gland  and  the  male  sexual 
apparatus. 

Tumors  of  the  pineal  gland,  Oppenheim  says,  cause  almost  the 
same  symptoms  as  tumors  of  the  corpora  quadrigemina,  only  in  the 
former  the  oculomotor,  trochlear,  and  abducent  nerves  are  not  so  often 
affected,  but  nystagmus  is  more  common. 

In  Zenner's  case  ^^  of  tumor  of  the  pineal  gland,  rapid  onset  of  blind- 
ness, deafness,  blunted  intelligence,  without  motor  or  sensory  paralysis, 
were  attribuated  to  acute  internal  hydrocephalus.  The  blindness  was 
not  believed  to  be  due  to  optic  neuritis,  and  the  optic  tracts  appeared  to 
be  somewhat  flattened.  Vomiting,  speech  disturbances  and  difficulty  in 
swallowing  were  attributed  to  pressure  on  the  pons  and  medulla 
oblongata. 

TUMORS    OF   THE    PITUITARY    BODY. 

Tumors  of  the  pituitary  body  not  infrequently  cause  bitemporal 
hemianopsia,  which  later  may  give  place  to  blindness.  Choked  disks 
may  be  associated  with  tumor  of  this  part  of  the  brain,  but  not  infre- 
quently the  optic  neuritis  is  of  mild  grade,  or  there  may  be  optic  atrophy 
from  the  pressure  on  the  chiasm  without  previous  neuritis.  Polyuria 
and  polydipsia,  even  diabetes  mellitus,  have  been  observed  as  signs  of 
such  a  growth.  The  tumor  may  develop  very  slowly.  Acromegaly  has 
been  found  so  frequently  in  cases  in  which  the  pituitary  body  was  en- 
larged that  some  relation  between  this  disease  and  the  alteration  of  the 
pituitary  body  probably  exists.  Sufficient  cases,  however,  have  been 
reported  to  show  that  tumor  of  the  pituitary  body  does  not  always  cause 
acromegaly.    Bitemporal  hemianopsia  is  a  common  sign  of  acromegaly. 

"  Die  Geschwiilste  des  Gehirns,  Oppenheim,  p.   142  et  seq. 

"  Philip  Zenner,  Alienist  and  Neurologist,  1892,  vol.  xiii.  p.  470. 


TUMOES  A:^rD  OTHEE  BEAIX  LESIONS  427 

If  a  tumor  at  the  chiasm  grows  to  sufficient  size  it  may  implicate  the 
ocular  nerves  in  its  neighborhood  and  cause  ocular  palsies.  Often  the 
hitemporal  hemianopsia  is  followed  by  complete  blindness  of  the  eye  on 
the  side  of  the  greater  development  of  the  tumor  and  still  later  by  com- 
plete blindness  of  the  other  eye.  Sometimes  when  a  tumor  implicates 
one  optic  tract  near  the  chiasm  it  causes  homonymous  lateral  hemi- 
anopsia, followed  by  complete  blindness  in  the  eye  on  the  same  side  as 
the  tumor  and  increasing  failure  of  sight  in  the  other  eye.  This  is 
caused  by  extension  of  the  tumor  to  the  inner  side  of  the  other  optic 
tract. 

Bitemporal  hemianopsia  can  be  caused  only  by  a  lesion  at  the  chiasm. 
Binasal  hemianopsia,  according  to  Harris,  has  been  observed  in  tabes 


Fig.  13.— Adenoma  of  the  pituitary  body.  Case  of  acromegaly  reported  by  Dr.  F.  A.  Packard, 
from  a  specimen  in  my  collection.  Necropsy  by  Dr.  Cattell.  (See  also  illustrations  of  this 
case  in  article  by  Dr.  0.  W.  Burr. ) 

find  in  one  or  two  obscure  cases  probably  as  a  result  of  symmetrical 
neuritis  of  the  outer  parts  of  the  chiasm.  He  says  further  that  homony- 
mous hemianopsia  may  occur  in  tabes,  as  he  has  seen  in  two  cases  with 
optic  atrophy,  and  in  multiple  sclerosis  also  with  optic  atrophy,  probably 
due  to  a  patch  of  sclerosis  affecting  the  optic  tract. 

It  is  improbable  that  a  lesion  at  the  optic  chiasm  can  cause  nasal 
hemianopsia  as  shown  by  Wilbrand  and  Saenger,  because  the  crossed 
and  uncrossed  fibres  are  mingled  at  the  lateral  part  of  the  chiasm,  but 
von  Monakow  says  the  lateral  part  of  the  chiasm  contains  chiefly  un- 


428  THE    EYE    AND    NERVOUS    SYSTEM 

crossed  fibres.  A  lesion  on  the  outer  side  of  each  optic  nerve  near  the 
chiasm,  or  on  the  outer  side  of  each  optic  tract,  might  cause  binasal 
hemianopsia.  This  subject  has  recently  been  carefully  studied  by  W.  T. 
Shoemaker. '^^ 

The  definition  of  hemianopsia  he  gives  is  "  Half  defect  in  the  field 
of  vision  of  each  eye,  symmetrically  placed,  and  caused  by  a  common 
intracranial  lesion."  He  believes  that  binasal  hemianopsia  can  never 
result  from  a  lesion  of  the  chiasm  on  account  of  the  anatomical  arrange- 
ment of  the  fibres,  and  that  in  most  instances  it  is  a  symptom  of  optic 
nerve  disease.  In  eighteen  cases  reported  in  the  literature  and  in  one 
of  his  own  of  binasal  hemianopsia  demonstrable  inflammatory  disease  of 
the  optic  nerves  was  found  in  twelve  cases.  Shoemaker  refers  to  five 
cases  which  seem  to  have  exhibited  true  binasal  hemianopsia  (Lang  and 
Beevor,  Graefe,  Daae,  Herschel,  Mooren's  second  case). 

Tumor  growing  from  the  pituitary  body  sometimes  causes  intense 
drowsiness.  It  was  present  in  the  case  of  acromegaly  reported  by  A.  E. 
Witmer  from  my  clinic  and  also  in  the  case  of  the  same  disease  reported 
by  F.  A.  Packard.  F.  W.  Mott^*says  that  in  three  cases  of  tumor  of 
the  third  ventricle,  which  started  probably  from  the  pituitary  body,  and 
in  which  during  life  symptoms  of  such  a  growth  were  present,  a  condi- 
tion of  drowsiness  was  observed  and  was  so  intense  that  these  patients 
would  fall  asleep  while  eating  their  meals,  and  in  two  of  the  cases  this 
phenomenon  was  so  marked  a  feature  as  to  be  the  most  prominent  and 
early  objective  symptom. 

Tumor  in  this  situation  I  believe  also  may  cause  frequent  ya^vning, 
but  I  have  seen  this  sign  when  the  tumor  was  in  the  cerebellum. 

I  have  seen  the  entire  third  ventricle  filled  by  a  tumor  growing  from 
the  pituitary  body  in  a  case  in  which  the  symptoms  of  acromegaly  were 
pronounced  (case  of  F.  A.  Packard,  Fig.  13).  Other  illustrations  of 
Dr.  Packard's  case  are  given  in  Dr.  Burr's  article. 

TUMORS   OF  THE   OPTIC   NERVE. 

Sometimes  a  tumor  occurs  in  the  intracranial  portion  of  the  optic 
nerves,  as  in  a  remarkable  case  reported  by  A.  Pick.*^^  Braunschweig 
collected  cases  of  tumor  of  the  optic  nerves  and  concluded  that  exoph- 
thalmos was  never  absent  in  such  cases.  Pick  shows  by  the  report  of 
his  0A\'n  case  that  the  word  "  never"  should  have  been  omitted.     At  the 


**  Shoemaker,  New  York  Med.  Journal  and  Philadelphia  Med.  Journal  February 
4,  1905. 

"Mott,  Brain,  1898,  vol.  xxi. 

"A  Pick,  Brain,  1901,  vol.  xxiv.  p.  502, 


Fig.  14. — Paralysis  of  right  upper  and  lower  limbs  and  of  right  side  of  face ;  paralysis  of  left  muscles 
of  mastication  and  of  left  side  of  tongue.  Tongue  deviated  to  the  left  when  protruded.  Diplopia  in 
looking  to  the  left.    A  lesion  of  the  pons  was  probably  present  in  this  case. 


Fig.  15.— Same  case  as  represented  in  Fig.  14.  The  lower  jaw  deviated  toward  the  left  when  the 
mouth  was  opened  because  of  the  paralysis  of  the  left  muscles  of  mastication.  The  tongue  deviated 
toward  the  right  when  within  the  mouth.    Paralysis  of  left  side  of  tongue. 


Fig.  Kj.— Paralysis  of  each  external  rectus  and  of  the  left  muscles  of  mastication  from  a  tubercle  in 

the  left  half  of  the  pons. 


Fig.  17.— Same  case  as  shown  in  Fig.  16.    Tubercle  of  the  left  half  of  the  pons. 


TUMORS  AlsD  OTHER  BRAIN  LESIONS  429 

necropsy  in  Pick's  case  the  optic  nerves  appeared  very  gray,  but  were 
not  diminished  in  size.  The  optic  nerves,  chiasm,  and  stump  of  the 
optic  tract  did  not  stain  Avell  by  the  Weigert-Pal  method,  and  on  super- 
ficial examination  seemed  to  be  merely  atrophied ;  but  more  careful 
examination  showed  numerous  cells  among  the  atrophied  nerve-fibres. 
The  tumor  was  a  myxosarcoma.  Early  failure  of  vision  is  to  be  ex- 
pected in  tumors  of  the  optic  nerve,  and  the  reflex  pupillary  response 
may  be  present  at  one  time  and  absent  at  another.  The  latter  sign  may 
not  always  be  present,  and  is  caused  probably,  as  Pick  says,  by  the  soft 
tumor  tissue  within  the  nerve  and  by  varying  degrees  of' vascular  con- 
gestion. Pick's  patient  had  hallucinations  of  sight,  similar  to  those 
sometimes  occurring  in  disease  of  the  retina  or  optic  nerves. 

TUMORS  OF  THE  PONS  AND  MEDULLA  OBLONGATA.   . 

Ataxia  is  generally  very  pronounced  in  tumors  of  the  pons,  because 
of  implication  of  the  cerebellar  peduncles,  but  I  have  had  a  case  in 
which  a  large  tumor  on  the  pons  caused  no  ataxia.  Many  important 
structures  are  crowded  together  in  the  pons,  and  even  a  small  growth  may 
cause  very  striking  symptoms^  When  the  tumor  is  confined  to  one  side 
of  the  pons,  it  may  cause  paralysis  of  motion  in  the  muscles  of  masti- 
cation and  loss  of  sensation  in  the  face  on  the  same  side  as  the  tumor, 
because  of  involvement  of  the  trigeminal  nerve ;  paralysis  of  the  ex- 
ternal rectus  and  of  the  muscles  of  the  face  on  the  same  side  as  the 
tumor,  because  of  involvement  of  the  abducent  and  facial  nerves;  in 
some  cases  also  when  the  tumor  extends  low  into  the  pons,  deafness  on 
the  same  side  from  involvement  of  the  acoustic  nerve,  or  of  the  intra- 
pontile  fibres  belonging  to  the  acoustic  system.  The  tongue  and  limbs 
may  be  paralyzed  on  the  opposite  side  because  of  implication  of  the 
central  motor-fibres  for  the  hypoglossus  nucleus,  and  of  those  for  the 
anterior  horns  of  the  cervical  and  lumbar  region  above  their  decussa- 
tion. Sensation  may  be  impaired  in  the  limbs  of  the  opposite  side  of 
the  body  and  even  in  the  opposite  side  of  the  face,  because  of  implication 
of  the  sensory  fibres  above  their  decussation.  Occasionally  the  tongue 
is  paralyzed  on  the  same  side  as  the  muscles  of  mastication  (Figs. 
14  and  15). 

In  a  case  of  tubercle  of  the  pons  observed  by  Dr.  C.  S.  Potts  and 
myself,  the  symptoms  were  weakness  of  the  muscles  of  the  left  side  of 
the  face,  excepting  the  orbicularis  palpebrarum;  weakness  of  the 
muscles  of  mastication  on  the  left  side,  and  fibrillary  tremors  of  these 
muscles;  weakness  of  the  left  external  rectus,  with  the  loss  of  power 
of  associated  movement  of  the  eyes  to  the  left,  but  with  preservation  of 


430 


THE    EYE    AND    NERVOUS    SYSTEM 


the  power  of  convergence;  diminution  of  the  appreciation  of  touch, 
pain,  heat,  and  cold  in  the  right  upper  and  lower  limbs,  right  side  of 
the  trunk  and  neck,  occipital  region  and  ear;  loss  of  stereognostic  per- 
ception and  of  the  sense  of  position  on  the  right  side;  diminution  of 
the  appreciation  of  touch,  pain,  heat,  and  cold  on  the  left  side  of  the 
face  and  head ;  slight  loss  of  appreciation  of  heat  and  cold  on  the  right 
side  of  the  face;  anaesthesia  of  the  conjunctiva,  mucous  membrane  of 
the  nose,  mouth,  and  tongue  on  the  left  side ;  deafness  in  the  left  ear ; 
loss  of  taste  in  the  left  anterior  half  of  the  tongue,  and  ataxia  of  both 


yio.  l.s  — Photoffraph  showing  the  tendency  of  the  head  to  fall  forward  and  to  the  left,  and  the  pres- 
ence of  left  hemiplegia  in  a  case  of  glioma  of  the  right  side  of  the  pons  (Figs.  5-H). 

lower  limbs,  especially  of  the  right.     The  tiiinor  was  confined  to  the  left 
half  of  the  pons. 

I  believe  tliat  this  case  shows  that  paralysis  of  lateral  associated 
movement,  when  the  power  of  convergence  is  preserved,  is  caused  by  a 
lesion  of  the  j)osterior  longitudinal  bundle  between  the  nuclus  of  the 
sixth  nerve  and  that  of  the  thir<l.  T  have  since  the  publication  of  this 
case  studied  two  other  cases  of  tumor  within  the  pons  with  necropsy, 
in  which  the  correct  diagnosis  could  be  made  during  the  life  of  the 
patient.  In  these  cases  the  symptoms  Avere  similar  to  those  described 
above,  and  in  each  case  I  was  able  to  observe  paralysis  of  associated  ocu- 
lar movements  toward  the  side  of  the  tumor. 


TUMORS    OF   THE   CEREBELLUM. 

Marcus  Gunn  "'"  believes  that  intense  double  optic  neuritis,  with 
much  swelling  and  surrounding  retinal  change,  coming  on  quickly,  sug- 

"(Junn,   Brain,   1898,  vol.  xxi. 


Fig.  19  —Glioma  of  the  right  side  of  the  pons.    F  G.  =  the  raphe  of  the  pons.     The  right  portion  of 
the  tumor  is  cystic.    Same  case  represented  in  Fig.  18. 


Fig.  20.— Cyst  and  glioma  of  the  vermis. 


Fig.  -21.— Position  of  standing  in  lesions  of  the  cerebellum. 


TUMORS  AND  OTHER  BRAIN  LESIONS  431 

gests  the  cerebellum ;  and  that  one-sided  optic  neuritis,  or  marked  dif- 
ference in  the  intensity  of  the  neuritis  on  the  two  sides,  suggests  the 
cerebrum,  and  is,  on  the  whole,  in  favor  of  the  tumor  being  on  the  same 
side  as  the  excess  of  neuritis,  where  there  are  other  reasons  for  localizing 
a  tumor  in  the  front  of  the  cerebrum. 

Cerebellar  tumor,  he  says,  very  often  excites  a  peculiarly  intense 
optic  neuritis,  with  great  engorgement  of  the  papillae  and  marked  oedema 
of  the  surrounding  retina,  with,  not  uncommonly,  a  macular  stellate 
figure  similar  to  what  is  seen  in  so-called  albuminuric  retinitis.  This 
condition  is  seen  also  in  basal  meningitis,  and  possibly  the  reason  for 
its  occurrence  with  cerebellar  tumor  is  that  the  latter  is  likely  to  cause 
basal  meningitis.  Mr.  Gunn  says  he  has  found  these  changes  in  the 
eye-grounds  in  at  least  two  cases  of  frontal  tumor,  in  neither  of  which 
was  there  any  evidence  of  basal  meningitis. 

The  changes  resembling  those  of  albuminuric  retinitis  were  present 
in  a  number  of  cases  of  tumor  that  have  come  under  my  observation. 

Tumor  of  the  cerebellum  is  often  in  connection  with  a  cyst 
(Fig.  20),  but  the  tumor  may  be  so  small  that  it  may  be  overlooked, 
imless  careful  microscopical  study  is  made ;  indeed,  some  writers  be- 
lieve that  a  cyst  of  the  cerebellum  is  always  at  some  time  in  its  growth 
in  connection  with  a  tumor. 

The  ataxia  of  cerebellar  tumor  is  often  intense,  and  the  patient 
staggers  from  side  to  side  like  a  drunken  person  (Fig.  21).  Choked 
disks  are  generally  pronounced.  The  headache  may  be  occipital  or 
frontal,  and  in  some  cases  may  be  associated  with  rigidity  of  the  neck. 
A  tendency  for  the  head  and  trunk  to  incline  forward  and  to  one  side 
(Fig.  18)  is  pronounced  in  some  cases,  but  it  is  uncertain  whether 
the  inclination  of  head  and  trunk  is  toward  the  side  of  the  tumor  or 
away  from  it.  Hemiasynergy  is  an  unreliable  sign,  although  I  have 
seen  it  in  three  or  four  cases.  It  may  be  tested  by  having  the  patient 
while  lying  down  draw^  up  each  lower  limb  separately,  and  it  should  be 
observed  then  whether  the  leg  is  extended  on  the  thigh  and  the  thigh 
on  the  trunk  synchronously,  or  whether  there  is  a  dissociation  of  these 
movements.  The  latter  is  supposed  to  indicate  the  presence  of  a  lesion 
in  the  lateral  lobe  of  the  cerebellum  on  the  side  of  the  hemiasynergy. 

Vertigo  is  often  an  early  and  persistent  sign  of  tumor  of  the  cere- 
bellum. 

Staggering  in  walking,  like  the  inclination  of  the  head  and  trunk, 
may  be  toward  or  away  from  the  side  of  the  tumor. 

Deafness,  such  as  may  be  caused  by  involvement  of  the  acoustic 
nerve  may  be  a  valuable  sign  pointing  to  the  side  of  the  cerebellum 


432 


THE    EYE    AND    NERVOUS    SYSTEM 


aflFected,  and  is  likely  to  be  present  when  the  tumor  is  in  the  cerebello- 
pontile  angle. 

In  a  case  of  cerebellar  abscess  I  had  the  opportunity  to  observe  that 
nystagmus  was  greater  when  the  eyes  were  turned  toward  the  side  of 
the  lesion  than  when  they  were  turned  away  from  it.  I  have  not  been 
able  to  make  this  observation  in  any  other  case,  although  the  sign  has 
been  seen  by  others. 

Tenderness  to  pressure  over  one  side  of  the  occipital  region,  and 
increase  or  diminution  of  vertigo  according  as  the  patient  lies  on  one  or 
the  other  side  of  the  body,  are  signs  of  uncertain  value. 

The  patellar  reflex  is  frequently  lost  in  cases  of  cerebellar  tumor,  but 
it  may  be  exaggerated,  or  may  vary  in  intensity  from  time  to  time. 


Fig.  22— Paralysis  of  each  external  rectus  from  a  tumor  in  the  fourth  ventricle,  implicating  the 

abducens  nucleL 


Lesions  Simulating  Tumor. — Other  lesions  of  the  brain  may  cause 
symptoms  resembling  very  closely  those  of  tumor,  and  chief  among  these 
is  abscess.  In  most  cases  some  purulent  focus  may  be  detected  as  the 
cause  of  the  cerebral  abscess,  and  most  frequently  it  is  disease  of  the 
middle  ear.  In  other  cases  pus  in  the  ethmoid  or  sphenoidal  sinuses 
may  be  the  starting  point  for  cerebral  abscess,  or  there  may  have  been 
some  injury  to  the  bones  of  the  skull.  The  symptoms  of  abscess  are 
likely  to  develop  more  rapidly  than  do  those  of  tumor  and  may  be  asso- 


TUMORS  A'NB  OTHER  BRAIK  LESIOXS  433 

ciated  with  fever,  or  subnormal  temperature,  chills,  slowing  of  the  pulse; 
or  the  symptoms  of  meningitis  may  be  added, — viz.,  transitory  ocular 
palsies,  rigidity  of  the  neck,  Kernig's  sign,  etc.  Purulent  meningitis 
often  accompanies  cerebral  abscess. 

When  the  meningitis  is  in  plaques  a  differential  diagnosis  from 
brain  tumor  may  be  impossible.  This  form  of  meningitis,  described 
especially  by  the  French  writers,  is  more  common  in  the  motor  area,  and 
I  have  described  one  of  the  two  cases  reported  in  this  country.  In  my 
patient  the  symptoms  were  precisely  those  of  tumor  of  the  parietal 
lobe. 

Hydrocephalus  in  often  associated  with  tumor  and  I  have  seen  it 
so  mask  the  symptoms  of  a  tumor  on  the  pons  that  the  latter  could  not 
be  diagnosticated.  Again,  I  have  seen  internal  hydrocephalus  without 
tumor  cause  the  symptoms  of  a  cerebellar  neoplasm. 

Hemorrhage  into  the  brain  can  be  easily  distinguished.  The 
rapidity  of  onset  and  the  course  of  the  symptoms  are  unlike  what  is  seen 
with  brain  tumor,  but  sometimes  hemorrhage  into  a  tumor  occurs,  and 
the  diagnosis  then  is  made  chiefly  by  the  sudden  increase  of  symptoms. 

The  differential  diagnosis  between  general  paralysis  of  the  insane 
(chronic  meningo-encephalitis)  and  cerebral  tumor  usually  is  easy,  but 
I  have  known  the  former  to  be  regarded  as  tumor,  and  the  danger  of 
mistake  is  greater  when  the  encephalitis  is  of  rather  rapid  onset.  Usu- 
ally, however,  a  correct  diagnosis  can  be  made. 

Aneurysms  may  give  the  symptoms  of  brain  tumor.  In  some  cases 
the  stethoscope  applied  to  the  head  may  be  of  assistance,  but  I  have 
known  a  supposed  tumor  at  the  chiasm  causing  bitemporal  hemianopsia 
to  be  in  reality  an  aneurysm  in  this  region,  and  to  present  no  signs 
characteristic  of  aneurysm. 


:« 


CHAPTER    X. 

BULBAR  AND  PSEUDOBULBAB  DISEASES 

By  E.  W.  TAYLOR,  A.M.,  M.D. 

The  clinical  significance  of  affections  of  the  oblongata  lies  essen- 
tially in  the  fact  that  in  this  portion  of  the  brain  are  gathered,  in  small 
compass,  nerve  mechanisms  of  vital  importance.  The  hypoglossal  nerve, 
and  the  group  made  up.  by  the  spinal  accessory,  vagus  and  glossopharyn- 
geal, are  situated  here,  in  close  relationship  to  each  other.  Disease 
processes,  small  in  extent,  may  therefore  lead  to  most  serious  disorders 
of  function,  if  localized  in  the  oblongata,  and  particularly  in  its  dorsal 
portion.  Owing  to  the  great  functional  importance  of  this  region,  there- 
fore, a  separate  consideration  of  its  diseases  is  justified,  although  it 
should  be  borne  in  mind  that  a  considerable  number  of  the  bulbar  affec- 
tions are  simply  local  expressions  of  more  general  processes,  either 
•spinal  or  cerebral,  or  involving  higher  levels  of  the  brain  stem.  For 
example,  bulbar  lesions  are  often  associated  with  tabes,  poliomyelitis, 
progressive  muscular  atrophy,  including  amyotrophic  lateral  sclerosis, 
multiple  sclerosis,  or  with  so-called  Landry's  paralysis,  diseases  which 
are  ordinarily  included  in  the  spinal  category.  On  the  other  hand, 
bulbar  symptoms  are  not  infrequently  combined  with  generalized  cere- 
bral lesions,  of  which  arteriosclerosis,  paralytic  dementia,  and  syphilis 
will  serve  as  examples.  More  important  for  our  present  purpose  is  the 
association  with  encephalitis,  and  especially  with  superior  polienceph- 
alitis,  ophthalmoplegia,  and  other  affections  of  cranial  nerve  nuclei 
beyond  the  immediate  limits  of  the  oblongata. 

As  knowledge  of  pathological  processes  grows,  it  becomes  in- 
creasingly evident  that  degenerative  or  inflammatory  conditions  are 
rarely  sharply  limited  to  one  small  area  in  the  nervous  system,  although 
their  clinical  manifestations  may  point  predominantly  toward  such 
localized  involvement.  This  is  somewhat  strikingly  true  of  the  bulbar 
diseases,  which,  as  already  suggested,  in  many  cases  constitute  merely 
the  most  conspicuous  part  of  the  clinical  picture,  owing  to  the  highly 
important  functional  significance  of  this  portion  of  the  brain.  It  is 
still  convenient,  however,  to  use  the  accepted  nomenclature  and  to  de- 
scribe the  bulbar  lesions  under  a  separate  heading. 
434 


BULBAK  AND  PSEUDOBULBAR  DISEASES         435 

General  Symptomatology. — The  symptoms  arising  from  disease  of 
the  oblongata  alone  are  chiefly  dependent  upon  lesions  involving  the 
nuclei  of  the  ninth,  tenth,  eleventh,  and  twelfth  nerves.  Through  in- 
volvement of  the  vagus  breathing  is  interfered  with,  leading  to  varying 
degrees  of  dyspnoea  and  at  times  to  the  Cheyne-Stokes  type.  The  normal 
action  of  the  heart  may  be  disturbed,  with  irregularity  of  the  pulse,  or 
with  an  increased  rapidity  or  abnormal  slowness,  depending  upon  the 
condition  of  paralysis  or  irritation  of  the  heart  regulatory  mechanism. 
A  more  important  and  characteristic  sign  of  bulbar  injury  is  difficulty 
in  deglutition,  brought  about  by  involvement  of  the  accessory-vagus- 
glossopharyngeal  group  of  nerves.  Mastication  may  also  suffer  through 
disordered  innervation  of  the  hypoglossal  nerve,  and  of  the  motor 
branches  of  the  trigeminal  to  the  jaw  muscles.  This  latter  difficulty  is 
usually  not  conspicuous,  since  the  degenerations  in  the  motor  nuclei 
of  tlie  fifth  nerve  lying  in  the  pons  are  usually  slight,  if  not  entirely 
lacking.  The  part  taken  by  the  tongue  in  the  act  of  mastication  is,  how- 
ever, sufficient  in  all  well-marked  cases  to  render  imperfect  the  disposal 
of  food  in  the  mouth.  Paralysis  and  atrophy  of  the  tongue  is  one  of 
the  most  constant  disturbances  in  the  course  of  bulbar  disease.  This 
leads  to  difficulty  in  forming  a  bolus  of  food,  and  in  passing  it  backward, 
and  also  to  a  characteristic  speech  disturbance  which  leads  very  early  to 
difficulties  in  articulation,  progressing  from  an  imperfect  enunciation  of 
certain  sounds  (dysarthria)  to  an  ultimate  complete  disability  to  articu- 
late (anarthria).  This  is  due  not  only  to  the  paralysis  of  the  tongue, 
but  also  to  disturbances  in  the  innervation  of  cheeks,  lips,  and  palate, 
which  determine  the  shape  of  the  mouth  cavity.  Owing  to  difficulty  of 
deglutition,  as  well  as  to  probable  disturbance  in  the  centre  in  the  oblon- 
gata, presiding  over  secretion,  the  saliva  is  apparently  secreted  in  in- 
creased amount,  and  constantly  flows  from  the  imperfectly  closed  mouth. 
This  combination  of  symptoms,  more  or  less  developed  in  various  cases, 
constitutes  the  essential  picture  of  the  primary  lesions  of  the  oblongata. 

A  fairly  sharp  distinction  may,  in  general,  be  made  between  those 
more  chronic  lesions  which  are  sharply  limited  to  the  oblongata,  and 
others  of  more  acute  onset  and  more  extensive  distribution.  In  the  latter 
the  general  symptomatology  becomes  much  wider  in  range,  owing  to  the 
fact  that  the  lesions  are  no  longer  limited  to  the  bulb,  but  extend  to  the 
pons,  and  even  in  certain  cases  to  the  mid-brain  region  of  the  corpora 
quadrigemina  and  crura  cerebri.  This  widening  of  the  scope  of  so- 
called  bulbar  diseases  is  not  strictly  accurate,  but  is  justified  and  necessi- 
tated by  the  diffuse  character  of  many  of  the  underlying  lesions,  and  on 
the  clinical  side  by  the  frequent  uncertainty  of  accurate  focal  diagnosis 


436  THE    EYE    AND    NERVOUS    SYSTEM 

of  disease  involving  the  pons-oblongata  region.  In  general,  the  symp- 
toms are  dependent  upon  lesions  of  the  nerves  already  mentioned, 
having  their  nuclei  in  the  oblongata,  and  also  upon  the  seventh,  sixth, 
and  fifth  nerves  located  in  the  pons,  together  with  the  fourth  and  third 
nerves,  lying  in  the  quadrigerainal  region.  Paralysis  of  the  face,  dis- 
turbance in  the  innervation  of  the  jaw,  and  various  paralyses  of  the 
ocular  muscles,  are  therefore  frequent.  Added  to  these  are  disorders  of 
motility  of  the  extremities  from  involvement  of  the  ventrally  lying 
pyramidal  tracts,  as  well  as  varying  alterations  of  sensibility,  dependent 
upon  interruption  of  sensory  tracts.  Except  for  the  symptoms  referable 
to  lesions  at  a  distance  or  of  unknown  cause,  which  will  receive  detailed 
discussion  later,  the  symptomatology  of  this  complicated  region  is 
wholly  dependent  upon  a  disturbance  of  function,  either  through  irrita- 
tion or  destruction  of  its  component  parts,  or  through  the  cutting  off  of 
its  central  connections. 

A  distinction  may  in  general  be  made  between  those  affections  which 
are  degenerative  in  character  and  chronic  in  course,  and  those  of  acute, 
or  sub-acute  onset,  in  which  the  nerve  elements  are  involved  secondarily 
to  vascular  changes.  Chronic  progressive  bulbar  paralysis  is  a  type  of 
the  first  class,  and  so-called  apoplectiform  bulbar  paralysis,  brought 
about  by  embolism  or  thrombosis  of  arteries,  a  type  of  the  second.  A 
third  general  group  includes  those  conditions  in  which  the  lesions 
apparently  producing  bulbar  symptoms  lie  at  a  distance  from  the  oblon- 
gata,— pseudobulbar  palsy, — and  finally  those  cases  in  which  no  lesions 
have  been  found  sufficient  to  account  for  symptoms  predominantly  of 
bulbar  type, — bulbar  paralysis  without  anatomical  basis;  myasthenia 
gravis  in  some  of  its  forms. 

The  following  discussion  will  elucidate  these  more  or  less  distinct 
processes,  all  of  which  have  been  differentiated  within  the  past  half 
century. 

PROGRESSIVE  BULBAR  PALSY. 

Terminology. — Various  terms,  suggestive  of  the  location  and  of  the 
course  of  this  disease,  have  been  used  since  the  earliest  description  by 
Duchenne.  The  more  important  of  these  are:  Progressive  muscular 
paralysis  of  the  tongue,  palate,  and  lips  (Duchenne)  ;  chronic  pro- 
gressive bulbar  paralysis  (Wachsmuth)  ;  progressive  nuclear  bulbar 
paralysis  (Kussmaul) ;  labio-glosso-laryngcal  paralysis  (Trousseau)  ; 
progressive  amyotrophic  bulbar  paralysis  (Leyden). 


History Following  an  observation  of  Diimesnil   in   1859,  in   which   the   sug- 
gestion  was   made   that  bulbar   lesions   and   progressive   muscular   atrophy   might 


BULBAK  AND  PSEUDOBULBAR  DISEASES         437 

co-exist,  Duchenne'  in  1860-61  gave  the  first  satisfactory  description  of  bulbar 
paralysis.  He  critcised  Dumesnil's  observation  on  the  ground  that  progressive 
bulbar  paralysis  and  progressive  (spinal)  muscular  atrophy  were  absolutely  dis- 
tinct diseases,  the  first  being  characterized  by  paralysis  without  atrophy,  and  the 
second  by  atrophy  without  paralysis,  a  false  assumption,  as  later  demonstrated. 
Observations  by  Romberg  and  Trousseau,  previous  to  Duchenne's  description,  did 
not  indicate  a  close  conception  of  the  character  or  manifestations  of  the  disease. 
In  1868  Trousseau,  on  the  basis  of  cases  with  somewhat  unsatisfactory  autopsies, 
completed  Duchenne's  earlier  description,  but  maintained  the  essential  identity  of 
the  disease  with  progressive  muscular  atrophy.  Microscopic  examination  revealed 
certain  degenerations  of  the  hypoglossal  and  accessory  nerves,  but  threw  no  definite 
light  on  the  changes  in  the  nuclei.  The  investigations  of  Baerwinkel,  Wachsmuth, 
and  Schultz  definitely  increased  knowledge  of  the  subject  in  the  years  up  to  1865. 
Baerwinkel  was  struck  with  the  apparent  retention  of  electrical  reactions  in 
affected  muscles,  and  also  with  the  sharp  localization  to  bulbar  nerves.  Wachs- 
muth in  1864  described  certain  alterations  in  the  oblongata,  which  were,  in  part, 
correctly  brought  into  relation  with  the  clinical  symptoms.  The  pathological 
anatomy  was  placed  on  a  firm  basis  by  the  work  of  Charcot  and  Joffroy  in  1869, 
verified  and  carried  further  by  Charcot's  pupils,  as  well  as  by  Leyden  (1870). 
The  degeneration  of  certain  motor  nuclei  in  the  oblongata,  with  the  associated 
and  resultant  alterations  of  nerves  and  muscles,  was  determined  as  the  essential 
lesion.  Kussmaul  (1871)  urged  the  neuropathic  character  of  the  degeneration  and 
established  the  analogy  to  progressive  (spinal)  muscular  atrophy  on  a  firmer 
basis.  Cases  thereafter  were  rapidly  reported,  with  increasingly  accurate  descrip- 
tions, both  on  the  clinical  and  anatomical  side,  by  Gombault,  Duchenne,  Joffroy, 
Hun,  Dejerine,  Freund,  Richie,  Reinhold,  Hoffman,  Tooth  and  Turner,  Remak,  Turner 
and  Bullock,  and  many  others.  At  the  present  time  the  affection  and  its  relations 
are  adequately  understood.  The  work  of  the  past  fifteen  or  twenty  years  has  been 
to  differentiate  from  the  chronic  progressive  type  of  bulbar  paralysis  other  varieties 
of  bulbar  disease  which  will  be  considered  in  detail  later  in  this  chapter. 

Symptomatology. — Bulbar  paralysis  of  the  progressive  type,  both  be- 
cause it  was  first  described  and  also  because  it  presents  a  most  charac- 
teristic picture  of  bulbar  disease  in  general,  may  serve  as  a  type  of 
lesions  of  this  region. 

The  symptoms,  as  observed  in  a  typical  case,  are  as  follows:  The 
onset  is  gradual,  usually  indicated  by  a  slight  disturbance  of  speech,  to 
which  small  importance  is  at  first  attached.  A  persistence  of  this  symp- 
tom, combined  with  a  sensation  of  pressure  about  the  neck,  finally  leads 
the  patient  to  a  realization  of  the  abnormal  condition.  As  in  many 
forms  of  disease  of  insidious  onset,  a  slight  exacerbation  of  symptoms 
may  give  rise  to  the  idea  of  a  sudden  onset,  which  in  this  affection  may 
be  assumed  never  actually  to  occur.  With  the  progress  of  the  disease 
the  tongue  is  affected;  its  movements  are  hindered;  subjective  sensa- 
tions of  weight  or  stretching  in  the  tongue  and  throat  are  noticed,  which 
may  extend  downward.  In  conjunction  with  these  symptoms  the  speech 
becomes  more  difficult,  more  indistinct  and  slower,  and  accompanied  by 

^Duchenne:  De  I'electrisaH^ion  localiz§e,  2d  ed.,  p.  621,  1861;  also  Arch.  G6n. 
de  Med.,  xvi.  283,  1860. 


438  THE    EYE    AND    NERVOUS    SYSTEM 

feelings  of  fatigue.  The  indistinctness  of  utterance  is  brought  about 
first  by  difficulty  in  the  enunciation  of  certain  letters,  in  general,  in- 
versely to  the  process  of  learning  to  speak.  The  sounds  "  r"  and  "  sch" 
are  early  lost,  as  in  the  child  they  are  last  to  be  acquired.  Later,  as  the 
movements  of  the  tongue  become  more  and  more  hindered,  enunciation 
of  the  lingual  consonants  "  1,  r,  n,  and  t,"  and  afterwards  "  s,"  is  lost 
(Gowers,  Leyden,  and  Goldscheider,  quoting  Kussmaul,  find  that  "  a" 
is  early  lost)  ;  "  t  and  d"  are  involved  when  the  tongue  can  no  longer 
be  brought  firmly  against  the  palate.  Succeeding  or  associated  with  the 
palsy  of  the  tongue,  the  lips  are  involved,  with  the  result  that  speech  is 
still  further  hindered  in  the  pronunciation  of  the  labials,  "  o,  u,  p,  b, 
m."  Even  when  the  disorder  has  reached  this  stage  words  may  still  be 
correctly  enunciated  by  special  effort  and  deliberation.  The  vowel 
sound  "  a"  is  longest  retained.  Finally,  with  the  advent  of  weakness  of 
the  palate,  complete  paralysis  of  the  tongue  and  inability  to  use  the  lips, 
speech  is  reduced  to  a  few  meaningless  sounds,  which  bear  only  a  distant 
similarity  to  articulate  language. 

Before  this  final  stage  is  reached,  difficulties  in  deglutition  super- 
vene, due  to  the  combined  weakness  of  the  tongue,  soft  palate  and  pha- 
ryngeal muscles.  Food  can  no  longer  be  properly  moved  into  the  back 
of  the  mouth  cavity  by  the  tongue,  and  the  act  of  swallowing  is  likewise 
difficult.  Mastication  also  suffers  from  the  palsy  of  the  tongue.  Liquids 
are  regurgitated  through  the  nose,  and  with  the  progressive  weakness  of 
the  muscles  of  the  epiglottis  particles  of  solid  food  enter  the  larynx. 
Swallowing  becomes  increasingly  difficult,  and  semisolids  are  found  to 
be  more  readily  taken  than  either  solids  or  liquids,  owing  to  the  lesser 
likelihood  of  finding  lodgement  in  the  larynx  or  being  regurgitated 
through  the  nose.  Along  with  the  difficulty  in  swallowing,  saliva  col- 
lects in  quantity,  and  finally  runs  in  a  constant  stream  from  the  mouth, 
which  offers  small  resistance  on  account  of  the  palsy  of  the  lips.  The 
patient  at  this  stage  presents  the  characteristic  picture  of  bulbar  disease, 
practically  speechless,  swallowing  with  the  utmost  difficulty,  and  con- 
tinually wiping  away  saliva  as  it  flows  from  the  imperfectly  closed  lips. 

As  the  disease  still  further  advances,  the  muscles  governing  the  vocal 
cords  are  apt  to  suffer;  paralysis  of  the  cords  results  and  phonation 
finally  becomes  impossible.  Coughing  ultimately  becomes  difficult,  with 
the  accompanying  danger  of  inhalation  pneumonia.  With  such  a  grad- 
ual increase  of  paralysis  in  tongue,  lips,  pharynx,  and  finally  larynx, 
the  disease  runs  its  course.  As  Gowers  puts  it,  "  the  symptoms  are 
grouped  about  the  tongue  as  a  centre,"  but  they  extend  far  beyond  the 
limits  of  this  one  organ,  before  life  is  actually  threatened. 


BULBAK  AND  PSEUDOBULBAR   DISEASES         439 

Other  symptoms  which  occur,  but  do  not  dominate  the  typical  clin- 
ical picture,  should  be  mentioned.  Although  there  is  no  loss  of  sensi- 
bility, and  taste  is  usually  unaffected,  the  throat  reflexes  ordinarily  fail, 
owing  to  the  motor  involvement,  though  this  is  not  invariably  the  case. 
This  tendency  to  loss  of  reflex  activity  naturally  renders  deglutition 
more  difiicult  and  increases  the  danger  of  the  entrance  of  food  into  the 
larynx.  The  jaw  reflexes,  on  the  contrary,  may  be  increased,  no  doubt 
due  to  a  degeneration  of  the  pyramidal  tracts.  (See  Pathological 
Anatomy.)  Dyspnoea,  rapid  pulse,  oedema  of  the  lungs,  have  been 
attributed  to  late  involvement  of  the  motor  vagus  (accessory).  Except 
for  an  undue  emotionalism,  laughter  or  tears  from  insufficient  cause, 
the  mental  faculties  remain  unimpaired,  and  the  patient  is  painfully 
•conscious  of  his  sufferings  up  to  the  very  end  of  the  disease. 

Physical  Signs. — The  physical  signs  of  the  affection  are  as  unmis- 
takable and  characteristic  as  its  symptomatic  course.  The  nerves  in- 
volved are  the  hypoglossus,  the  motor  portions  of  the  vagus-glossopha- 
ryngeal-accessory  group,  rarely  the  motor  portion  of  the  trigeminus,  the 
facial,  and  ocular  nerves.  The  involvement  of  these  latter  nerves  is 
rather  to  be  regarded  as  a  complication  than  as  a  component  part  of  the 
symptom-complex,  assuming,  as  we  do  somewhat  arbitrarily,  that  pro- 
gressive bulbar  paralysis  is  a  disease  sui  generis^  limited  to  the 
oblongata.  As  a  direct  result  of  the  primary  degeneration  of  the  hypo- 
glossal nerve,  the  tongue  atrophies;  as  the  disease  progresses,  it  be- 
comes wrinkled,  and  furrowed,  often  swollen  in  appearance,  and  lies 
practically  immovable  on  the  floor  of  the  mouth.  A  similar  atrophy 
•occurs  in  the  lips ;  a  complete  closing  of  the  mouth  finally  becomes  im- 
possible, and  the  lower  portion  of  the  face  assumes  a  characteristic  ex- 
pressionless appearance  in  marked  contrast  to  the  usually  alert  and 
distressed  aspect  of  the  eyes  and  upper  portion  of  the  face. 

The  fact  that  the  upper  distribution  of  the  seventh  nerve  is  spared, 
whereas  the  lower  branches  are  apparently  involved  in  the  degenerative 
process,  has  led  to  considerable  speculation  as  to  the  course  of  the  lower 
fibres,  with  the  general  conclusion,  as  just  stated,  that  the  orbicularis 
oris  is  supplied  either  directly  or  indirectly  by  fibres  of  the  hypoglossal 
nerve,  but  connected  wdth  the  facial  through  the  dorsal  longitudinal 
fasciculus.  The  close  physiological  association  of  lips  and  tongue  in 
phonation  renders  probable  the  assumption  that  the  innervation  is  from 
a  common  source,  or  from  areas  in  intimate  anatomical  relationship.  It 
is,  at  least,  certain  that  atrophy  of  the  lip  muscles  is  a  constant  feature 
of  progressive  bulbar  palsy,  whereas,  in  exceptional  instances  only  are 
the  upper  facial  muscles  involved.    In  certain  cases  of  bulbar  paralysis, 


440  THE    EYE    AND    NERVOUS    SYSTEM 

however,  forming  part  of  a  general  amyotrophic  lateral  sclerosis,  the 
facial  nerves  may  be  involved  in  all  branches ;  in  other  cases,  as  reported 
by  Remak,"  Tooth  and  Turner,^  Turner  and  Bullock,"*  and  others,  the 
evidence  goes  to  show  that  in  spite  of  nuclear  and  other  degeneration  of 
the  seventh  nerves,  the  upper  branches  have  still  been  spared.  Follow- 
ing an  earlier  suggestion  of  Mendel,®  that  the  upper  facial  muscles  were 
supplied  by  fibres  of  the  third  nerve,  through  the  dorsal  longitudinal 
fasciculus  and  the  trunk  of  the  facial  nerve.  Tooth  and  Turner  were 


Fig.  1.— Progressive  bulbar  paralysis,  patient  of  39,  six  months  duration.  D^Iutdtlon  very  diflBcult ; 
speech  wholly  impossible ;  palsy  of  tongue,  lips,  and  muscles  of  deglutition ;  exaggerated  deep  reflexes 
The  photograph  shows  an  extreme  attempt  to  open  the  mouth  and  protrude  the  tongue. 

able  to  demonstrate  a  complete  atrophy  of  the  nucleus  of  the  seventh 
nerve  and  also  of  its  dorsally  directed  fibres  and  the  knee,  whereas  the 
outgoing  nerve  contained  normal  fibres.  From  this  observation,  and 
others  of  similar  character,  it  has  been  assumed  that  the  upper  facial 
muscles  derive  their  supply  indirectly  from  the  nucleus  of  the  third 
nerve.  This  would  explain  the  sparing  of  the  upper  branches  in  those 
cases  of  bulbar  palsy  in  which  the  lower  branches  of  the  seventh  nerve 
were  manifestly  involved  without  assuming  the  intervention  of  the 
hypoglossal.  It  would  also  fall  in  with  the  physiological  hypothesis, 
as  already  suggested,  that  muscles  acting  in  association  have  a  closely 


'Remak:     Arch.  f.  Psych.,  xxiii.,  H.  3,  919,  1892;    Neurol.  Ctb.,  vii.  62,  1888; 
idem,  x.  28,  1891;    idem,  xi.  753,  1892;    Berl.  klin.  VVoch.,  xxxii.  29,  1895. 
'Tooth  and  Turner:    Brain,  xiv.  473,  1891. 
♦Turner  and  Bullock:    Brain,  xvii.  093,  1894. 
'Mendel:    Berl.  klin.  VVoch.,  xxiv.  913,  1887;    Neurol.  Ctb.,  vi.  537,  1887. 


BULBAE  AKD  PSEUDOBULBAE  DISEASES         441 

related  nerve  supply, — lips  and  tongue  in  articulation,  opening  and 
closing  of  the  lids  through  the  action  of  the  levator  palpebrse  (third 
nerve)  and  the  orbicularis  palpebrarum  (seventh  nerve,  through  fibres 
derived  from  the  third  nucleus). 

On  the  other  hand,  Wilbrand  and  Saenger  ^  are  of  the  opinion  that 
the  nucleus  of  the  third  nerve  has  nothing  whatever  to  do  with  muscles 
supplied  by  the  upper  facial.  In  support  of  this  opinion  they  quote  a 
careful  examination  by  Cassirer  and  Schiff  in  which  there  was  no  dis- 
turbance in  the  function  of  the  upper  facial  in  spite  of  complete  de- 
generation of  the  oculomotor  nucleus,  including  its  dorsal  portion,  to 
which  Mendel  and  others  had  attached  particular  significance.  Siemer- 
ling  and  Boedecker  also  oppose  the  idea  that  the  facial  distribution  to 
the  eye  muscles  has  any  relation  whatever  with  the  nucleus  of  the  third 
nerve.  Evidence  is,  therefore,  as  yet  conflicting  as  to  the  course  of  the 
fibres  innervating  the  upper  portion  of  the  face,  although  the  fact  must 
be  accepted  that  in  nearly  all  cases  of  chronic  bulbar  paralysis  the  lower 
facial  muscles  are  alone  involved.  The  possibility  of  at  least  a  partial 
nerve  supply  from  the  region  of  the  hypoglossal  nucleus  for  these 
muscles  must  be  considered,  as  already  suggested. 

In  those  cases  of  otherwise  uncomplicated  bulbar  paralysis,  in  which 
the  upper  branches  of  the  seventh  nerve  have  been  involved  in  the 
paralysis,  it  is  probable  that  we  have  to  do  with  a  family  type  of  the 
disease,  as  observed  by  Londe  "^  and  Eemak.®  Londe  found  in  a  child 
bulbar  paralysis,  with  general  involvement  of  the  upper  branches  of  the 
facial  nerve,  together  with  palsy  of  the  levator  palpebral  superioris, 
but  without  other  ophthalmoplegia.  Eemak  has  also  reported  a  number 
of  cases  of  involvement  of  the  upper  facial  muscles  in  children,  which 
led  to  the  suggestion  of  a  special  type  of  family  bulbar  paralysis,  having 
analogies  to,  but  not  identical  with,  the  adult  form. 

In  general,  regarding  the  greater  or  less  involvement  of  the  upper 
cranial  nerves,  lying  in  the  pons  or  above,  and  including  the  facial 
nerve,  it  is  now  sufficiently  evident  that  the  underlying  process  is  by  no 
means  a  sharply  limited  one,  but  that,  aa  already  suggested,  it  may 
extend  both  upward  and  downward  from  its  point  of  earliest  develop- 
ment in  the  nuclei  of  the  bulb  proper.  This  fact  will  receive  further 
comment  in  the  discussion  of  the  pathological  anatomy. 

Electrical  Reactions. — The  electrical  alterations  in  bulbar  paralysis. 


'Wilbrand  and  Saenger:    Die  Neurologie  des  Auges,  Bd.  I.,  Abt.  1,  p.  114. 
'Londe:    Eev.  de  MM.,  xiii.  1020,  1893;    xiv.  212,  1894. 
'  Remak :    loc.  cit. 


442  THE    EYE    AND    NERVOUS    SYSTEM 

as  in  other  analogous  affections,  depend  upon  degenerative  changes  in 
the  peripheral  motor  neurones  with  consequent  muscular  atrophy.  The 
early  confusion  inaugurated  by  Duchenne,  and  later  set  at  rest  by 
Trousseau,  Kussmaul  and  all  later  observers  regarding  atrophy  and 
paralysis,  and  the  relations  of  bulbar  palsy  to  progressive  muscular 
atrophy,  was  enhanced  by  the  apparent  failure  of  electrical  changes  in 
the  tongue.  It  is,  however,  undoubtedly  true  that  if  the  muscles  of  the 
tongue  could  be  stimulated  with  the  same  ease  as  other  muscles,  elec- 
trical alterations  of  greater  or  less  degree  would  always  be  found.  The 
deposition  of  fat  in  the  atrophying  tongue  is  one  of  the  chief  hindrances. 
Observations  hitherto  made  have  not  demonstrated  a  complete  reaction 
of  degeneration,  but  partial  degenerative"  reaction  (Erb),  and  various 
stages  between  that  and  the  normal  have  been  observed  so  frequently 
as  to  substantiate  the  a  priori  assumption  of  electrical  alterations  of 
some  degree  in  all  cases  in  which  the  hypoglossal  nerve  is  degenerated. 
Paralysis  of  the  tongue  and  neighboring  parts  from  other  lesions  than 
those  affecting  the  peripheral  motor  neurones  naturally  would  not  give 
rise  to  electrical  changes,  and  no  doubt  it  is  these  cases  which  have  led 
to  the  confusion, 

Duchenne  and  later  Dejerine  ^  have  drawn  attention  to  the  fact  that 
the  paralytic  symptoms  of  bulbar  paralysis  may  be  explained  by  de- 
generation of  the  pyramidal  tracts.  To  this  may  be  associated  degenera- 
tion of  peripheral  neurones,  giving  rise  to  a  bulbar  amyotrophic  lateral 
sclerosis,  or  an  amyotrophic  lateral  sclerosis  of  descending  course.  A 
paralytic  and  atrophic  form  have  been  distinguished.  It  is  in  general 
evident  that  the  atrophic  element,  with  altered  electrical  reactions,  will 
predominate  when  the  peripheral  neurones  are  involved ;  that  the  spastic 
element  will  be  conspicuous  if  the  central  neurones  (pyramidal  tracts) 
are  involved,  and  that  transitional  forms  may  occur  with  varying  de- 
grees of  degeneration  of  the  two  sets  of  neurones.  The  analogy  is  com- 
plete to  spinal  progressive  muscular  atrophy  of  the  flaccid  and  spastic 
(amyotrophic  lateral  sclerosis)  types  respectively.  Finally  it  should 
be  remembered  that  progressive  bulbar  paralysis,  if  not  too  quickly 
fatal,  represents  either  the  starting  of  a  degenerative  process,  which 
tends  to  progress  beyond  the  confines  of  the  bulb,  or  else  develops  as  the 
final  stage  of  analogous  affections  originating  in  the  upper  spinal  cord. 

Pathological  Anatomy. — Progressive  bulbar  paralysis  is  characterized 
by  a  chronic,  primary  degeneration  of  the  cells  of  the  motor  nuclei  of 
the  oblongata.     This  degeneration  is  not  necessarily  associated  with 


•Dejerine:    Arch,  de  Physiol.,  xv.  180,  1883. 


BULBAR  AND  PSEUDOBULBAR   DISEASES 


443 


primary  alterations  of  the  vessels,  nor  with  evidences  of  inflammatory 
reaction,  and  should  be  distinguished  from  those  affections  of  the 
oblongata  which  are  dependent  upon  vascular  disorders  or  inflammatory 
processes.  (See  following  sections.)  The  cells  of  the  hypoglossal 
nuclei  suffer  most  extensively;  many  of  them  show  marked  degenera- 
tive changes,  are  shrunken  or  entirely  disappear.  The  accessory  hypo- 
glossal nuclei  are  spared.  Less  marked,  but  still  evident,  are  degenera- 
tions in  the  motor  nuclei  of  the  accessory  nerve  and  of  the  cells  of  origin 
of  the  motor  vago-glossopharyngeal  roots.  Degenerations  have  at  times 
been  found  in  the  nucleus  ambiguus,  also  in  the  nuclei  of  the  seventh 


"ifSfc" 


Fig.  2.— Progressive  bulbar  paralysis ;  nucleus  ot  hypoglossal  nerve  in  centre  of  field,  showing  loss 
of  myelinated  fibres  and  almost  complete  disappearance  of  nerve  cell  bodies;  x  45.  (Photograph  by 
W.  S.  Greene.) 


pair  and  motor  nucleus  of  the  fifth  nerve  in  those  not  infrequent  cases 
in  which  the  disease  progresses  beyond  the  oblongata  into  the  pons.  Un- 
less we  regard  the  dorsal  nucleus  of  the  vagus  and  glossopharyngeus  as 
motor,  at  least  in  part,*^  it  is  difficult  to  account  for  the  paralyses  of 
larynx  and  pharynx,  in  those  cases  in  which  the  nucleus  ambiguus  has 
been  found  intact,  unless  their  supply  be  wholly  through  the  accessory 
nerve.  These  matters  require  further  investigation  on  the  pathological- 
anatomical  side. 

Corresponding  to  these  cell  degenerations  the  hypoglossal  network  of 


'Spiller:    Univ.  of  Penna.  Med.  Bulletin,  March.  1903. 


444 


THE    EYE    AND    NERVOUS    SYSTEM 


myelinated  fibres  is  degenerated,  and  the  intra-  and  extra-bulbar  axones 
of  the  affected  cells.  The  ensuing  muscular  atrophy  shows  no  peculiar- 
ity. The  muscle-bundles,  particularly  of  the  tongue,  are  thinned,  the 
connective  tissue  increased,  granular  and  fatty  degeneration  occur  often 
with  an  excessive  deposit  of  fat,  which  may  somewhat  mask  the  atrophy. 
The  tip  of  the  tongue  suffers  most. 

A  very  frequent,  though  not  absolutely  constant,  accompaniment  of 
the  muscular  alterations  is  degeneration  of  the  pyramidal  tracts. 
Opinions  differ  as  to  the  frequency  of  this  association.  A  number  of 
cases  have  been  described  in  which  no  alterations  in  the  pyramidal 
tracts  were  found;    they  may,  nevertheless,  have  been  present  in  very 


Fig.  3.— Progressive  bulbar  paralysis;  tongue;  atrophy  of  muscle  bundles;  increase  of  fat;  x  45. 

(Photograph  by  W.  S.  Greene.) 

slight  degree,  as  might  have  been  shown  had  the  Marchi  method  been 
used.  It  has  been  suggested  (Remak)  that  rapidly  fatal  cases  are  less 
likely  to  show  degeneration  of  the  pyramidal  tracts,  but  that  such  de- 
generation would  probably  have  developed  had  life  been  prolonged. 
This  question  is  precisely  analogous  to  that  long  discussed  with  regard 
to  progressive  muscular  atrophy  with  or  without  degeneration  of  the 
pyramidal  tracts.  In  general,  the  evidence  goes  to  show  that  the 
peripheral  neurones  may  be  degenerated  independently  of  the  central, 
but  that  the  converse  very  rarely  occurs. 

Etiology,  Course,  and  Treatment. — The  cause  of  the  affection  is  wholly 
unknown.     Syphilis  and  alcohol  have  no  direct  bearing  on  its  develop- 


BULBAR  AND  PSEUDOBULBAR  DISEASES         445 

ment.  It  occurs  more  frequently  among  men  than  women — 34  men 
and  19  women  in  a  series  of  53,  as  observed  by  Kussmaul.  Apart  from 
the  rare,  apparently  congenital  type,  observed  in  children,  it  is  dis- 
tinctly a  disorder  of  the  latter  years  of  life,  most  frequent  between  the 
fiftieth  and  the  seventieth  years.  I  have,  however,  recently  observed 
two  cases  in  men,  each  thirty-nine  years  old. 

The  course  is  from  one  to  three  years,  and  in  certain  cases  much 
longer.  In  general,  it  is  steadily  progressive  and  death  results  from 
paralysis  of  respiration  and  deglutition,  or  from  inhalation  pneumonia 
in  consequence  of  pharyngeal  j)aralysis.  The  prognosis  is,  therefore, 
wholly  bad,  although  occasional  remissions  may  occur. 

The  treatment  must  be  directed  towards  proper  diet,  especially 
in  the  later  stages.  Semisolid  food  is  usually  indicated,  and  when 
swallowing  becomes  too  difficult  or  impossible,  the  stomach  tube  becomes 
a  necessity.  General  hygienic  measures  are  essential,  and  encourage- 
ment serves  better  than  drugs.  Strychnia  in  increasing  doses  may  be 
used ;  its  efficiency  is  doubtful.  Kussmaul  and  later  Remak  have  urged 
the  value  of  the  galvanic  current  for  the  purpose  of  stimulating  the 
muscles  concerned  in  swallowing.  Remak  advises  that  the  anode  be 
placed  at  the  back  of  the  neck,  and  the  cathode  passed  over  the  neck 
on  either  side  of  the  trachea,  using  a  current  of  3-6  milliamperes. 

ACUTE  (APOPLECTIFORM)  BULBAR  PARALYSIS. 

Hemorrhage,   Embolism,   Thrombosis   of   Arteries   of   Bulb   and   Pons 

Under  this  general  heading  may  be  included  those  affections  of  the 
oblongata  and  pons,  which  are  characterized  by  relatively  sudden  onset, 
and  are  either  rapidly  fatal  or  else  show  a  tendency  towards  improve- 
ment. They  are  to  be  distinguished  from  the  foregoing  progressive 
bulbar  paralysis  on  the  clinical  side  by  their  non-progressive  course,  and 
on  the  anatomical  side  by  their  wholly  different  pathological  altera- 
tions.^^ 

Inasmuch  as  the  lesions  producing  the  symptoms  of  acute  bulbar 
paralysis  are  mainly  dependent  upon  the  blood  supply  of  the  oblongata- 
pons  region,  the  following  anatomical  facts  should  be  borne  in  mind: 
The  two  vertebral  arteries  unite  to  form  the  basilar  artery  at  the  lower 
border  of  the  pons,  having  given  off,  among  others  which  do  not  now 


"Van  Voort  (Deuts.  Ztschft.  f.  Nervenheilk.,  viii.  137,  1896)  has  published  a 
valuable  discussion,  with  an  elaborate  table,  on  Acute  Bulbar  Paralysis,  and  very 
recently  Dana  (Trans.  Ass'n  American  Physicians,  xviii.  446,  1903)  has  published 
an  excellent  and  comprehensive  paper  on  the  same  subject. 


446  THE    EYE    AND    NERVOUS    SYSTEM 

concern  ns,  branches  to  the  oblongata.  The  basilar  artery  before  di- 
viding into  the  posterior  cerebral  arteries  gives  off  transverse  branches 
to  the  pons.  These  arteries  run  for  a  distance  on  the  surface  of  the 
pons  and  then  penetrate  its  substance.  From  studies  made  by  Duret, 
it  appears  that  the  nuclei  of  the  hypoglossal  and  accessory  nerves  receive 
their  blood  supply  from  the  anterior  spinal  artery  and  the  vertebrals; 
the  nuclei  of  the  vagus,  glossopharyngeal  and  auditory  from  branches 
from  the  upper  portion  of  the  vertebrals,  or  lower  part  of  the  basilar; 
the  facial,  trigeminal,  and  ocular  nerves  from  the  branches  of  the 
basilar.  Clinical  experience,  however,  has  not  as  yet  justified  complete 
reliance  on  these  facts. 

Pathological  Anatomy. — The  lesions  underlying  a  large  proportion  of 
the  acute  bulbar  affections  are  alterations  in  the  blood-vessels,  which 


Fig.  4.— Acute  (ajxiplectiform)  bulbar  paralysis ;  pons :  lighter  areas  softening,  as  result  of  tbromboeis 
of  basilar  artery.    (Photograph  by  W.  S.  Greene.) 

lead  secondarily  through  weakness  of  their  walls  to  hemorrhage,  or 
through  roughening  or  narrowing  of  the  lumen  to  thrombosis,  and  when 
the  proper  conditions  are  present,  to  embolism.  The  sources  of  the 
vessel  changes,  and  their  consequent  failure  to  carry  on  their  normal 
function,  are  those  recognized  in  arterial  disease  generally, — cardio- 
renal  affections,  syphilis,  senility  and  the  arteriosclerosis  which  is 
common  in  middle  life  and  almost  physiological  in  old  age.  Emboli 
may  also  reach  this  region  from  diseased  heart  valves.  The  basilar 
artery  is  a  frequent  seat  of  marked  sclerotic  change,  and  often  shows  a 


BULBAR  AND   PSEUDOBULBAR  DISEASES         447 

tortuous  course,  irregularly  thickened  walls,  and  aneurismal  dilatations 
which  may  exert  destructive  pressure  on  the  overlying  oblongata.  The 
same  is  true,  though  perhaps  to  less  degree,  of  the  vertebrals.  The  pons 
and  oblongata  must,  therefore,  both  suffer  from  such  lesions,  since  their 
immediate  blood  supply  is  wholly  from  these  larger  trunks.  The  soften- 
ings, or  more  rarely,  hemorrhages,  which  may  result,  do  not  differ  his- 
tologically from  such  lesions  observed  elsewhere  in  the  brain,  and 
require  no  detailed  description  here.  The  character  of  the  blood  supply 
naturally  gives  rise  to  the  possibility  of  multiple  lesions.  The  pons  and 
oblongata,  for  example,  are  often  simultaneously  involved,  and  it  is 
frequently  impossible  to  localize  the  lesion  accurately  in  one  or  the 
other  region,  hence  the  general  inclusion  of  the  symptoms  under  the 
head  of  bulbar  paralysis.  A  higher  level  than  the  pons  may  also  at 
times  be  involved,  still  further  extending  the  inclusiveness  of  the  term. 
Ow'ing  to  the  facts  that  the  lesions,  particularly  of  softening,  may  be 
multiple,  that  they  are  often  extensive,  and  that  they  show  no  special 
predilection  for  the  gray  matter,  it  follows  that  the  symptoms  produced 
are  far  less  definite  than  in  the  circumscribed  chronic  progressive  form, 
and  therefore  that  wide-spread  disorders  of  motility  and  sensibility  may 
result. 

BULBO-PONTINE    HEMORRHAGE— ACUTE    BULBAR    APOPLEXY. 

Symptomatology. — Hemorrhage  of  the  pons  is  rare ;  it  has  been  esti- 
mated that  hemorrhage  of  the  brain  (cerebrum)  is  about  fifty  times  as 
frequent.  Hemorrhage  of  the  oblongata  is  still  less  common.  It  occurs 
most  frequently  in  the  fourth  decade  of  life,  and  is  usually  due,  as  in 
the  brain,  to  the  results  of  cardiac  hypertrophy  in  the  course  of  renal 
degeneration  associated  with  arteriosclerosis.  The  branches  of  the 
basilar  are  the  usual  sites  of  rupture;  the  basilar  itself  is  rarely  in- 
volved. 

The  symptoms  in  a  typically  severe  and  fatal  case,  following  in  gen- 
eral Dana's  description,  are:  Prodromal  neck  pain,  headache,  vertigo, 
vomiting,  succeeded  at  varying  intervals  by  an  apoplectic  seizure  often 
of  extreme  suddenness.  This  is  usually  followed  by  profound  coma, 
twitching  of  face  and  extremities,  but  rarely  a  general  convulsion.  The 
face  is  flushed ;  the  temperature  subnormal  at  first ;  pulse  irregular ; 
respiration  slow,  irregular  and  of  the  Cheyne-Stokes  type.  The  pupils 
are  contracted ;  there  may  be  convergent  strabismus  or  conjugate  devia- 
tion. Later  a  rise  of  temperature  occurs,  often  reaching  a  high  point ; 
swallowing  is  impossible ;  dyspnoea,  rapid  pulse,  cyanosis,  due  to  paral- 
ysis of  respiration,  lead  to  death  in  from  six  to  twenty  hours  after  the 


448  THE    EYE    AND    NERVOUS    SYSTEM 

onset.     In  less  violent  eases  paraplegia  or  a  crossed  paralysis  manifests 
itself  when  the  acute  stage  is  past,  with  exaggeration  of  reflexes. 

BULBO-PONTINE    SOFTENING. 

The  clinical  differentiation  between  hemorrhage  and  softening  is 
usually  difficult  and  often  impossible  to  make,  as  in  the  case  of  similar 
lesions  in  the  cerebral  hemispheres.  The  suddenness  of  onset,  the 
presence  of  conditions  in  other  organs  predisposing  to  rupture  of  blood- 
vessels, notably  hypertrophied  heart  Vv'ith  elevated  blood-pressure,  are 
suggestive  of  hemorrhage,  though  never  absolutely  conclusive.  Soften- 
ing in  the  pons  and  oblongata  is  about  three  times  as  frequent  as  hemor- 
rhage, and  occurs  usually  in  syphilitic  persons  between  the  ages  of  thirty 
and  fifty.  Arteriosclerosis  in  some  form  is  a  necessary  forerunner  of 
softening,  except  in  the  rare  cases  of  embolism.  Men  are  affected  as 
frequently  as  women. 

In  general,  the  onset  of  severe  symptoms  is  slower  in  softening  from 
any  cause  than  in  hemorrhage,  with  the  possible  exception  of  embolism. 
Following  general  signs  of  arterial  disorders,  such  as  headache,  vertigo, 
tinnitus,  marked  bulbo-pontine  symptoms  develop  with  greater  or  less 
rapidity.  If  the  oblongata  be  particularly  involved,  through  disturb- 
ance in  the  branches  of  the  vertebral  arteries,  disorders  of  speech  and 
difficulty  in  deglutition  occur,  with  possible  paralysis  of  the  pharyngeal 
and  laryngeal  musculature.  Contraction  of  the  pupil,  with  smaller 
palpebral  fissure,  retraction  of  the  globe  and  facial  flushing,  may  occur 
from  involvement  of  a  sympathetic  centre  supposed  to  lie  in  the  oblon- 
gata. Further  disorders  of  sensibility  and  motility  supervene  if  the 
lesion  be  extensive,  due  to  injury  of  the  pyramidal  tracts,  and  interrup- 
tion of  the  sensory  tracts  lying  deep  in  the  oblongata.  In  unilateral 
lesions  hemiplegia  is  a  common  accompaniment.  There  is  also  at  times 
a  dissociation  of  sensation  of  the  usual  character — loss  of  pain  and  tem- 
perature senses,  with  retention  of  the  sense  of  touch,  and  ataxia.  De- 
fective sense  of  position,  astereognosis,  and  forced  movements  have  also 
been  observed. 

Involvement  of  the  pons  to  the  exclusion  of  the  oblongata  implies  a 
freedom  of  the  nuclei  and  peripheral  distribution  of  the  eighth  to  the 
twelfth  nerves  inclusive.  The  fifth,  sixth,  seventh,  and  often  the  third, 
show  defects  of  various  degree.  Sensory  disorder  in  the  distribution 
of  the  fifth  nerve  is  not  uncommon.  The  pyramidal  tracts,  or  often  one 
tract,  is  involved,  giving  rise  to  hemiplegia,  sparing  the  face  if  the 
facial  nerve  escapes  injury  both  in  its  central  and  peripheral  course. 
The  so-called  alternating  or  crossed  hemiplegia  is  produced  when  the 


BULBAE  AND  PSEUDOBULBAR  DISEASES         449 

lesion  lies  in  such  a  position  as  to  involve  the  peripheral  neurone  of  the 
seventh  or  sixth  nerve,  or  if  higher  up,  of  the  third  nerve,  and  the 
pyramidal  tract  of  the  same  side.  The  crossing  of  the  pyramidal 
tract  at  a  lower  level  leads  to  a  paralysis  of  arm  and  leg  on  the  opposite 
side,  and  of  face  or  certain  eye  muscles  on  the  same  side  as  the  lesion. 
Lesions  involving  the  central  path  of  the  sixth  nerve  may  also  lead  to 
conjugate  deviation  of  the  eyes  towards  or  away  from  the  lesion,  de- 
pending upon  whether  the  lesion  be  paralyzing  or  irritative.  An  injury 
to  the  common  centre  for  the  sixth  nerve  on  one  side,  and  the  third  nerve 
(branch  to  the  internal  rectus  of  the  opposite  side),  if  it  be  destructive, 
will  lead  to  a  conjugate  deviation  away  from  the  lesion.  In  the  absence 
of  knowledge  regarding  the  extent  of  injury,  definite  rules  concerning 
the  diagnostic  focal  value  of  conjugate  deviation  cannot  be  laid  down. 
Convergent  strabismus  is  a  result  of  implication  of  the  sixth  nerve,  one 
or  both.  Nystagmus  also  is  a  common  sign  of  pons  lesion,  to  be  ex- 
plained by  disorder  of  the  coordinative  mechanism.  Quadrigeminal 
lesions  give  rise  to  disorders  in  the  distribution  of  the  third  nerve — 
external  strabismus,  ptosis,  paralytic  dilatation  of  the  pupil — and  of 
the  less  important  fourth  nerve.  Symptoms  of  true  bulbar  paralysis, 
involvement  of  tongue,  larynx,  phar^Tix,  may  occur  from  interruption 
of  the  upper  neurones  of  the  hypoglossal,  and  vago-glossopharyngeal 
group  in  the  pons.  This  effect  is  most  markedly  produced  by  a  bilateral 
lesion.  In  such  a  lesion,  the  element  of  atrophy  will  naturally  be  lack- 
ing in  the  peripheral  portion  of  the  nerves  involved.  Injury  of  one 
fillet  produces  an  imperfect  sensory  loss  on  the  opposite  side  of  the  body, 
which  may  be  associated  with  ataxia.  Ataxia  of  the  cerebellar  type  is 
probably  produced  by  lesions  of  the  superior  peduncles  and  red  nucleus. 
Pain  is  never  a  conspicuous  symptom,  which  is  in  general  true  of  intra- 
cerebral or  spinal  lesions.  Uncontrolled  emotionalism — forced  laughter 
— is  a  frequent  symptom  in  bulbo-pontine  disease,  and  is  perhaps  best 
explained  by  the  theory  of  partial  or  complete  destruction  of  the  paths 
of  control,  which  must  be  closely  associated  if  not  identical  with  the 
upper  neurones  of  the  bulbar  and  pontine  nerves. 

In  general,  the  multiplicity  of  symptoms  which  may  result  from 
lesions  of  the  oblongata  or  pons,  or  both,  is  apparent  when  one  considers 
the  complexity  of  anatomical  structure  in  this  portion  of  the  brain. 
Considering,  also,  the  fact  that  the  ordinary  lesions,  producing  acute 
bulbar  paralysis,  may  vary  widely  in  extent  and  destructiveness,  it 
follows  that  the  symptoms  may  range  from  slight  and  temporary  dis- 
turbances in  the  functions  of  the  bulb  and  pons  up  to  conditions  which 
are  quickly  fatal.  It  should,  however,  not  be  difiicult  to  picture  the 
29 


450  THE    EYE    AND    NERVOUS    SYSTEM 

symptomatology  of  intermediate  stages,  if  one  bears  in  mind  the  func- 
tional significance  of  this  lower  portion  of  the  brain  stem.  The  follow- 
ing case  illustrates  sudden  bulbar  palsy  of  slight  degree : 

A  woman  of  fifty-five,  of  active  life,  well  except  for  phlebitis  some  years  ago 
and  an  unexplained  attack  of  vertigo  several  months  before,  suddenly  without  pro- 
dromata,  noticed  distinct  difficulty  in  speech,  coming  on  without  pain.  This  was 
associated  with  difficulty  in  swallowing,  but  without  actual  regurgitation.  Exam- 
ination four  days  after  the  onset  gave  the  following  results:  No  definite  arterio- 
sclerosis ;  heart  sounds  normal,  except  for  accentuated  aortic  second.  '  The  eyes 
showed  nothing  abnormal,  and  sensibility  was,  in  general,  unimpaired.  The  upper 
cranial  nerves  were  not  involved.  The  tongue  was  protruded  imperfectly,  and  all 
movements  were  possible,  though  weak  and  slow.  Speech  was  thick,  laborious,  but 
understandable.  There  was  paresis  of  the  soft  palate  and  of  the  pharynx.  Swal- 
lowing was  very  difficult,  but  possible,  when  either  liquid  or  solid  was  taken  in 
small  amount.  The  reflexes  were,  in  general,  active,  but  there  was  no  evidence  of 
hemiplegia  or  other  paralysis  of  the  extremities.  Later  reports  showed  rapid 
improvement. 

Such  a  case  must  be  interpreted  as  due  to  a  vascular  bulbar 
disorder,  not  sufficiently  extensive  to  lead  to  irremediable  defect. 
It  serves  as  a  type  of  those  bulbar  affections  which  are  slight  in  them- 
selves, but  are  indicative  of  the  possibility  of  more  serious  results  in  the 
future. 

Course  and  Outcome. — As  already  suggested,  if  not  immediately  fatal, 
the  tendency  of  this  group  of  affections  is  towards  improvement,  usually 
leaving  greater  or  less  functional  defect,  except  in  the  slightest  cases, 
as  for  example,  that  quoted  above.  The  reason  for  this  is  obvious  from 
the  character  of  the  underlying  pathological  process,  and  is  perfectly 
analogous  to  such  processes  elsewhere.  Inasmuch  as  the  conditions 
which  lead  to  the  onset  of  the  symptoms  are  usually  not  materially 
influenced  by  treatment,  the  danger  of  subsequent  attacks  of  similar 
character  is  evident. 

Treatment. — The  treatment  of  acute  bulbar  paralysis  should  pri- 
marily be  directed  to  the  underlying  cause,  hemorrhage  or  softening, 
and  should  be  chiefly  preventive.  If  syphilis  be  thought  a  possible  cause, 
and  it  should  be  suspected,  especially  in  relatively  young  patients,  an 
antisyphilitic  cure  should  be  administered  vigorously.  In  other  cases 
of  arterial  lesions,  care  should  be  taken  to  provide  for  adequate  nutri- 
tion and  to  avoid  the  dangers  of  inhalation  pnuemonia,  as  for  the  chronic 
progressive  form.  More  important  than  the  treatment  of  the  actual 
condition  are  prophylactic  measures,  care  of  the  kidneys  and  of  the 
whole  circulatory  system.  A  strict  dietary  regime,  avoidance  of  exer- 
tion and  a  restrained  manner  of  life  generally  should  be  insisted  upon 
in  those  cases  which  show  the  often  vague  signs  of  cerebral  circulatory 


Figs.  5,  6.— Oblongata ;  gumma  involving  region  of  bulbar  nerves,  fourth  ventricle  and  a  part  of 
the  cerebellum;  various  somewhat  irregular  bulbar  and  general  symptoms ;  basilar  artery  normal. 
(Photographs  by  L.  S.  Brown.) 


BULBAK  AND  PSEUDOBULBAK  DISEASES         451 

disturbance,  or  which  have  undergone  a  slight  apoplectic  attack  either  of 
the  brain  or  pons-oblongata  region.  Drugs  are  not  serviceable,  except  to 
meet  indications  as  they  arise. 

BULBAR   PARALYSIS    FROM    OTHER   CAUSES. 

In  addition  to  the  foregoing  somewhat  definite  type  of  bulbar 
disease,  many  other  causes  of  disturbance  in  the  function  of  this  region 
have  been  described,  some  of  which,  on  acount  of  their  rarity  and  lack 
of  relation  to  the  general  subject-matter  of  this  book,  may  be  cursorily 
considered.  A  few  cases  have  been  described  (Leyden,  Etter)  of  mye- 
litis of  the  bulb,  with  symptoms  of  acute  bulbar  paralysis.  Leyden 
describes  and  pictures  a  case  in  which  inflammatory  changes  and  mul- 
tiple foci  of  hemorrhage  were  found  in  the  region  of  the  olives  and  else- 
where throughout  the  bulb,  showing  markedly  on  the  floor  of  the  ven- 
tricle. This  condition  is  to  be  regarded  as  a  local  manifestation  of  the 
group  of  conditions  usually  described  as  acute  encephalitis,  or  poli- 
encephalitis,  and  corresponds  in  a  measure  to  the  condition  of  superior 
encephalitis,  of  which  special  mention  is  hereafter  made  (page  452).  It 
serves  to  demonstrate  the  fact,  not  infrequently  observed,  that  inflam- 
matory processes,  with  a  certain  predilection  for  the  gray  matter,  may 
occur  anywhere  throughout  the  central  nervous  system,  the  local  symp- 
toms naturally  depending  upon  the  part  involved. 

Trauma  of  the  bulb  occasionally  occurs  through  stabbing  wounds  or 
fracture  and  dislocation  of  the  upper  vertebrae.  Such  injuries  are 
usually  fatal,  but  if  not,  the  familiar  symptoms  are  produced  in  pro- 
portion to  the  extent  and  severity  of  the  lesions. 

Bulbar  symptoms  in  the  course  of  typhoid  fever  and  leukaemia  have 
been  described  (Eisenlohr)  and  also  in  multiple  neuritis.  An  infective 
agent  (streptococci)  w^as  found  in  the  fatal  typhoid  fever  cases,  hemor- 
rhages and  inflammatory  changes  occurred  in  the  sheaths  of  bulbar 
nerves  in  the  cases  of  leukaemia,  and  in  rare  eases  it  has  been  shown  that 
a  neuritis  may  affect  the  bulbar  nerves. 

Tumors  in  the  posterior  cranial  fossa,  meningeal  thickenings  from 
syphilis  or  other  cause,  aneurismal  dilatations  or  actual  aneurisms  of 
the  basilar  artery,  and  more  rarely  of  the  vertebrals  (Oppenheim, 
Seimerling)  may  by  compression  lead  to  bvilbar  symptoms,  often  with 
involvement  of  the  pyramidal  tracts,  and  consequent  spasticity  of  the 
extremities  (Figs.  5  and  6).  The  symptoms  are  naturally  variable; 
they  are  usually  slowly  progressive,  often  with  a  final  severe  exacerba- 
tion. Owing  to  the  tortuous  course  of  the  artery  in  compression  by 
aneurism,  the  motor  s;)Tnptoms  are  irregular  in  distribution,  and  often 


452  THE    EYE    AND    NERVOUS    SYSTEM 

of  the  type  of  an  alternating  hemiplegia.  The  principles  of  diagnosis 
apply  in  general  to  eases  of  this  type  as  to  others,  a  peculiarity  being 
gradual  course,  with  final  increase  of  symptoms,  either  through  soften- 
ing or  rupture  of  the  aneurism. 

Cases  of  a  family,  congenital  type  of  bulbar  paralysis  have  been 
described  by  Remak,  Londe,  and  others,  to  which  allusion  has  previously 
been  made,  in  which  the  upper  branches  of  the  facial  nerves  are  apt  to 
be  involved,  a  fact  possibly  of  diagnostic  significance. 

SUPERIOR   ENCEPHALITIS    (WERNICKE). 

On  a  basis  of  three  cases  Wernicke,^ ^  in  1881,  described  a  new  symp- 
tom-complex, to  which  he  gave  the  name  of  acute  hemorrhagic  superior 
poliencephalitis.  A  previous  case,  reported  by  Gayet  in  1875,  had  not 
served  to  establish  the  identity  of  the  process.  Wernicke  regarded  the 
affection  as  an  independent,  acute  inflammatory  process  in  the  region  of 
the  nuclei  of  the  ocular  nerves,  fatal  in  from  ten  to  fourteen  days.  The 
focal  symptoms  consist  in  associated  paralysis  of  ocular  muscles,  of  quick 
onset  and  rapid  progress,  finally  leading  to  a  practically  total  paral- 
ysis of  the  muscles  involved,  with  the  possible  exception  of  the  sphinc- 
ter iridis  or  the  levator  palpebrarum.  The  gait  of  the  patient  is  ataxic, 
suggesting  alcoholic  intoxication.  The  mental  state  is  markedly  dis- 
turbed ;  somnolence  and  agitation  may  both  occur ;  a  mental  condition 
strongly  suggesting  dilirium  tremens  may  form  part  of  the  clinical 
picture.  In  the  three  cases  described  by  Wernicke  changes  of  an  in- 
flammatory character  were  observed  in  the  optic  disk.  Alcohol  played 
a  prominent  part  in  the  etiology  of  the  affection  in  two  cases,  and  sul- 
phuric acid  poisoning  in  the  other.  The  essential  pathological  altera- 
tion in  each  case  was  multiple  punctiform  hemorrhages  in  the  dorsal 
portion  of  the  third  ventricle  and  the  region  of  the  aqueduct  of  Sylvius, 
including  especially  the  nuclei  of  the  ocular  nerves. 

Subsequent  observations  have  extended  the  knowledge  of  the  acute 
.  poliencephalitis  described  by  Wernicke.  Influenza  is  probably  an  im- 
portant factor  in  etiology  as  well  as  other  infectious  diseases,  and 
poisonings  of  varied  character.  The  prognosis  is  also  not  so  invariably 
bad  as  at  first  supposed,  nor  may  the  pathological  anatomy  be  regarded 
as  uniform. 

In  twenty  well  observed  cases  of  Wernicke's  hemorrhagic  polien- 
cephalitis, fifteen  with  autopsies,  Wilbrand  and  Saenger  ^^  note  the  fol- 


"  Wernicke:    Lehrbuch  der  Gehirnkrankheiten,  1881,  §47,  p.  229. 
"  Wilbrand  and  Saenger :    loc.  cit.,  p.  270. 


BULBAR  AND  PSEUDOBULBAR  DISEASES         453 

lowing  facts  of  importance  with  reference  to  the  ocular  conditions :  In 
nine  cases  there  was  ptosis  of  varying  degree ;  in  all  the  cases,  excepting 
two,  in  which  no  observation  on  the  condition  of  the  muscles  is  made, 
the  ocular  muscles  were  variously  involved,  affecting  both  the  third  and 
the  sixth  nerves,  giving  rise  to  more  or  less  immobility  of  the  eyes,  with 
accompanying  diplegia,  nystagmus,  and  strabismus ;  the  pupils  showed 
varying  reactions  to  light,  from  normal  to  complete  immobility;  prac- 
tically no  observations  were  made  on  the  power  of  accommodation,  for 
obvious  reasons,  in  view  of  the  constant  palsy  of  the  external  muscles ; 
ophthalmoscopic  examination  showed  changes  in  a  few  instances,  of  the 
character  of  hypera^mia,  unusual  pallor  of  parts  of  the  disk,  and  occa- 
sionally papillitis.  Various  nuclear  and  peripheral  degenerations  were 
found  post-mortem.  In  general,  the  ocular  conditions  showed  no  ten- 
dency to  conform  to  any  one  type. 

The  position  definitely  taken  by  Wernicke  that  superior  polien- 
cephalitis  is  a  wholly  analogous  affection  to  poliomyelitis  and  other  in- 
flammatory affections  throughout  the  brain  stem  has  been  amply  borne 
out  by  subsequent  observations.  This  is  shown,  first,  by  the  essential 
identity  of  the  pathological  findings  in  these  variously  localized  pro- 
cesses, and  secondly,  by  the  many  transitional  forms  which  have  been 
described.^ ^  Encephalitis,  on  the  one  hand,  superior  poliencephalitis, 
inferior  poliencephalitis,  giving  the  symptoms  of  an  acute  bulbar  paraly- 
sis, poliomyelitis,  and  finally  poliencephalomyelitis,  no  doubt  represent 
simply  different  expressions  of  a  common  underlying  cause,  the  symp- 
toms naturally  varying  widely  with  the  portion  of  the  nervous  system 
involved. 

The  analogy  may  usefully  be  carried  further  in  considering  the 
more  chronic  forms  of  degeneration  of  the  upper  cranial  nerves,  chronic 
ophthalmoplegia  and  allied  conditions,  as  being  similar  in  character 
to  chronic  progressive  bulbar  paralysis,  already  described  (page 
436),  and  to  the  spinal  form  of  progressive  muscular  atrophy.  The 
transition  between  these  lesions  and  the  degree  of  acuteness  of  onset 
together  make  possible  most  varied  symptomatic  manifestations,  of 
which  a  certain  number  stand  out  conspicuously,  and  have,  therefore, 
been  regarded  as  clinical  entities.  Among  these  chronic  ophthalmo- 
plegia is  important. 

CHRONIC    OPHTHALMOPLEGIA. 

In  the  great  majority  of  cases  chronic,  usually  progressive,  ophthal- 
moplegia, forms  a  part  of  a  more  general  process,  as  for  example,  tabes 

"Taylor:  Poliencephalitis  and  Allied  Conditions,  Boston  Med.  and  Surg.  Jour., 
cxlviii.  634,  1903. 


454  THE    EYE    AND    NERVOUS    SYSTEM 

or  dementia  paralytica,  and  is  frequently  associated  with  progressive 
bulbo-pontine  degenerations  of  motor  nuclei,  and  at  times  with  spinal 
cord  lesions  of  similar  character.  As  an  early  manifestation  of  one  of 
these  more  extensive  affections,  as  in  those  cases  in  which  it  dominates 
the  clinical  picture,  chronic  ophthalmoplegia  is  deserving  of  special 
consideration. 

The  symptom-complex  was  first  described  by  von  Graefe.  Its  de- 
velopment is  slow  and  its  course  long,  in  contrast  to  progressive  bulbar 
paralysis,  due  to  the  fact  that  the  process  is  located  in  a  region  of  the 
brain  not  essential  to  life.  Although  varying  widely  in  detail,  the 
disease  begins  usually  with  diplegia  or  ptosis,  followed  by  a  very  gradual 
extension  of  the  palsy  to  the  external  ocular  muscles  of  both  eyes;  the 
accommodative  mechanism  and  the  levator  palpebrae  superioris  are 
often  spared.  Later  the  internal  muscles  may  be  involved,  or  in  other 
cases  they  may  be  affected  from  the  beginning.  The  progressive  in- 
volvement of  the  muscles  points  always  toward  nuclear  degeneration  as 
a  cause,  easily  explained  by  the  complicated  anatomical  arrangement  of 
the  nucleus  of  the  third  nerve.  As  the  process  continues,  the  eyeball 
may  ultimately  become  wholly  immovable,  and  the  levator  palpebrae 
and  orbicularis  oculi  also  be  involved.  In  rare  cases  the  disease  is 
checked  when  the  ocular  paralysis  is  complete  (Striimpell).  It  is,  how- 
ever, usual  for  the  degeneration  to  extend  to  other  nuclei,  with  an  ulti- 
mately fatal  outcome.  ' 

Pathological  Anatomy. — The  usual  lesion  is  a  progressive  degenera- 
tion of  the  cells  of  the  nuclei  of  the  third,  fourth  and  sixth  nerves,  pre- 
cisely analogous  to  that  described  under  progressive  bulbar  paralysis. 

PSEUDOBULBAR  PARALYSIS. 

Knowledge  of  this  type  of  bulbar  paralysis  dates  from  1872,  when 
Joffroy  first  called  attention  to  the  fact  that  the  symptoms  of  bulbar 
lesion  may  occur  without  disease  of  the  oblongata,  the  essential  altera- 
tions being  in  the  cerebral  hemispheres.  An  almost  simultaneous  pub- 
lication by  Jolly  of  a  case  with  bulbar  symptoms  from  cerebral  multiple 
sclerosis,  and  in  1877  a  report  of  cases  by  Lepine  and  Barlow  estab- 
lished the  essential  identity  of  the  symptom-complex.  Lepine  first  used 
the  term  "  pseudobulbar"  as  applied  to  these  cases.  Since  that  time 
papers  have  appeared  by  Kirchoff,  Fere,  Jolly,  Oppenheim,  and  Siemer- 
ling,  Russell  and  Taylor,  Becker,  Remak,  Colman,  Oppenheim, 
Bouchard,  Karplus,  Weisenburg,  and  others.  From  these  various  ob- 
servations it  is  apparent  that  a  bilateral  destructive  lesion  of  the  cortico- 
bulbar  tracts  is  sufficient  to  produce  the  general  symptoms  of  bulbar 


Fig.  7.-Poliencephalitis  superior  and  inferior.  The  symptoms  of  gradual  onset  were  dysphagia 
paresis  of  soft  palate,  respiratory  and  cardiac  disturbances,  vertigo,  somewhat  exaggerated  tendon 
reflexes  (but  no  weakness  of  limbs),  bilateral  ptosis,  intense  external  and  internal  ophthalmoplegia, 
diplopia,  and  pallor  of  optic  disks.    (Unreported  case  seen  by  Dr.  Spiller  with  Dr  Thorington  ) 


Fig.  8.— Same  patient  as  Fig.  7.    Photograph  shows  the  bilateral  ptosis  and  the  inability  to  look  upward. 


Fir.s.  9, 10.— Intense  muscular  atrophy  otcurring  in  encephalo  myelitis.    (Case  of  Dr.  Spiller.) 


Fig.  11.— a  case  of  myasthenia  gravis,  with  paralysis  confined  to  the  ocular  muscles.  (Reported  by 
Drs.  Spiller  and  Buckman,  Amer.  Jour,  of  the  Med.  Sciences,  April,  1905).  The  ptosis  of  the  right  eye 
is  not  so  great  as  that  of  the  left.  The  photograph  was  taken  one  or  two  minutes  after  glasses,  one  of 
which  was  opaque,  were  removed.    At  times  the  patient  was  able  to  open  both  eyes  fully. 


Fig.  12.— Same  patient  as  shown  in  Fig.  11.    The  eyeballs  are  almost  completely  covered.    The  photo- 
graph was  taken  after  the  glasses  had  been  removed  three  or  four  minutes. 


BULBAE  AND   PSEUDOBULBAR   DISEASES         455 

palsy,  presumably  by  a  cutting  off  of  the  upper  paths  of  voluntary 
control. 

Pathological  Anatomy. — The  usual  post-mortem  findings  are  arterio- 
sclerotic changes,  with  the  resultant  areas  of  softening  and  degeneration. 
The  lesions  are  usually  bilateral,  though  a  few  cases  of  bulbar  palsy 
have  been  observed,  in  which  the  cerebral  lesions  were  confined  to  one 
hemisphere.  The  usual  locations  of  the  softened  areas  are  the  cerebral 
white  matter,  internal  and  external  capsules,  central  ganglia,  particu- 
larly in  the  lenticular  nuclei,  and  more  rarely  in  the  cortex.  That  such 
lesions  are  sufficient  to  produce  the  symptoms  of  bulbar  paralysis  has 
been  shown  by  adequately  studied  cases.  Oppenheim  and  Siemerling,*^ 
however,  warn  against  the  too  hasty  acceptance  of  cerebral  lesions  alone 
as  explanatory  of  the  symptoms.  In  the  investigation  of  five  cases,  in 
which  the  pons  and  oblongata  were  studied  serially,  it  was  found  that 
multiple  small  areas  of  softening  were  present  in  such  a  position  as  to 
require  consideration  in  the  production  of  symptoms.  They  very  rightly 
maintain,  therefore,  that  the  cerebral  origin  of  the  symptoms  should  not 
be  insisted  upon  until  a  careful  microscopic  examination  of  the  pons- 
oblongata  region  is  made.  This  apparently  was  not  done  in  many  of  the 
earlier  cases  reported. 

It  seems  advisable,  therefore,  to  divide  the  cases  on  the  pathological 
side  into  cerebral,  and  cerebrobulbar,  recognizing  the  fact  that  a  purely 
cerebral  form  is  less  usual  than  at  first  supposed.  The  cerebrobulbar 
forms  evidently  may  not  differ  widely  in  their  pathological  anatomy 
from  certain  forms  of  acute  bulbar  paralysis,  due  to  focal  softening  in 
the  pons  or  oblongata,  a  general  arteriosclerosis  being  the  underlying 
condition  in  both  cases.  Oppenheim  and  Siemerling  have  also  called 
attention  to  the  importance  of  pressure  exerted  on  the  oblongata  by  a 
dilated,  aneurismal  or  arteriosclerotic  vertebral  or  basilar  artery,  in  pro- 
ducing symptoms  which  might  otherwise  be  attributed  to  more  distant 
lesions.  Recognizing  these  various  possibilities,  the  following  lesions 
are  types  of  those  which  have  been  described  as  causative  of  the  symp- 
toms :  bilateral  softening  in  lenticular  nucleus,  parietal  lobe,  cortex  of 
island  of  Reil,  tempero-sphenoidal  lobe  (Fere)  ;  lesions  in  both  cap- 
sules (Colman)  ;  cortical  cystic  tumors  (Picot  and  Hobbs)  ;  cortical 
changes  in  an  epileptic  (Bouchard) ;  diffuse  lesions  of  hemispheres 
(Becker)  ;    microgyria  (Oppenheim). 

Symptomatology. — Considering  the  rather  wide-spread  lesions  in  the 
brain  which  are  required  to  produce  disturbance  in  the  bulbar  innerva- 

"  Oppenheim  and  Siemerling:   Versammlung  Deuts.  Naturforscher,  Berlin,  1886. 


456  THE    EYE    AND    NERVOUS    SYSTEM 

tion,  it  follows  that  the  symptoms  are  less  sharply  confined  to  this  region 
than  in  processes  before  discussed.  Various  paralyses  of  the  extremi- 
ties, hemiplegia,  paraplegia,  together  with  aphasia,  hemianopsia,  and 
general  mental  defect,  may  form  a  part  of  the  clinical  picture,  together 
with  distinctly  bulbar  symptoms. 

The  development  of  the  affection  is  characterized  by  repeated  ex- 
acerbations, each  leaving  the  patient  with  a  further  disability,  and  in- 
creased mental  enfeeblement.  Characteristic,  and  found  only  in  this 
type,  is  progressive  dementia,  with  various  supervening  conditions  of 
confusion  and  excitement,  forming  part  of  the  picture  of  a  wide-spread 
arteriosclerosis.  The  paralyses  of  extremities  are  naturally  dependent 
upon  the  position  of  lesions  interrupting  motor  conduction.  The  sig- 
nificant symptoms  for  present  consideration  are  those  dependent  upon 
lesion  of  the  tracts  to  the  bulbar  nerves,  whence  comes  the  simulation  of 
actual  lesion  of  the  oblongata.  Loss  of  the  power  of  speech,  difficulty 
in  deglutition,  paresis  of  lower  branches  of  the  facial  nerve  (lip  mus- 
cles) and  of  the  palate,  occur  in  a  form  superficially  indistinguishable 
from  true  bulbar  paralysis.  The  muscles  involved  are,  however,  not 
atrophied  (disease  of  upper  motor  neurone)  and  the  electrical  reactions 
are  not  affected.  Other  symptoms  of  less  constant  occurrence  are: 
Weakness  of  muscles  of  mastication;  increased  pulse-rate  and  aryth- 
mia ;  optic  atrophy  or  neuritis ;  occasional  impairment  of  bladder  and 
rectal  function ;  rarely  anaesthesia  in  various  localities.  More  frequent 
are  respiratory  disorders:  attacks  of  dyspnoea  and  Cheyne-Stokes 
breathing.  As  in  other  forms  of  bulbar  paralysis,  uncontrolled 
expression  of  the  emotions  is  frequent;  due,  no  doubt,  to  a  diminu- 
tion of  inhibition  through  destruction  of  paths  of  control,  especially, 
it  has  been  suggested,  those  from  the  cortex  to  the  optic  thalamus. 
Certain  observations  seem  to  show  that  a  bilateral  break  in  tracts 
from  the  cortex  to  the  nuclei  of  ocular  nerves  may  lead  to  disorder 
in  movement  of  the  eyes,  of  such  a  character  that  voluntary  control 
is  difficult,  whereas  they  may  still  follow  an  object  (Oppenheim  and 
others). 

The  symptoms  usually  first  manifest  themselves  after  an  apoplectic 
attack,  often  slight  in  itself,  but  succeeded  at  varying  intervals  by 
others,  until  finally  the  clinical  picture  is  fully  developed.  Much  more 
rarely  the  disease  progresses  slowly  after  an  acute  onset.  The  outcome 
is  dependent  upon  the  position,  character  and  extent  of  the  arterio- 
sclerotic process. 

It  is  evident  in  this,  as  in  other  inflammatory  and  degenerative  pro- 
cesses, which  have  been  considered  in  the  foregoing  pages,  that  an  abso- 


BULBAR  AND  PSEUDOBULBAR  DISEASES         457 

lutelj  uncomplicated  clinical  type  is  rarely  found.  The  distinction  be- 
tween cerebral  and  cerebrobulbar  forms  is  often  impossible  to  make 
clinically,  and  again  the  relationship  between  these  forms  and  acute 
softening  of  the  bulb  is  often  confused.  Many  unclassified  transitional 
forms  of  bulbar  disease,  dependent  upon  general  arteriosclerosis,  must 
be  recognized,  which  it  is  not  necessary  to  describe  in  detail,  provided  the 
underlying  principle  of  the  genesis  of  the  symptoms  be  understood. 

Prognosis  and  Treatment. — The  outcome  of  pseudobulbar  paralysis  is 
ultimately  fatal,  through  the  increasingly  disastrous  results  of  the  un- 
checked arteriosclerotic  process.  Remissions  are  frequent,  but  the  gen- 
eral tendency  is  progressive,  and  little  can  be  done  by  way  of  treatment, 
beyond  a  careful  regulation  of  the  patient's  life  in  all  particulars,  and 
attention  to  the  details  of  feeding  and  prophylaxis,  as  stated  in  a 
previous  section. 

MYASTHENIA    GRAVIS. 

This  condition  is  also  described  under  the  following  titles:  Erb's 
disease;  asthenic  bulbar  paralysis  (Striimpell)  ;  asthenic  paralysis; 
myasthenia  gravis  pseudo-paralytica  (Jolly)  ;  bulbar  paralysis  without 
discoverable  anatomical  lesion ;  Erb-Goldflam  or  Hoppe-Goldflam  symp- 
tom-complex. 

Although  an  undoubted  case  was  earlier  described  by  Wilks,  the 
credit  of  first  calling  definite  attention  to  this  affection  belongs  to  Erb, 
who,  in  1879,^^  on  a  basis  of  three  cases  described  the  group  of  symp- 
toms, since  known  as  myasthenia  gravis.  He  drew  special  attention  to 
the  symptoms  of  ptosis,  weakness  of  the  muscles  of  mastication  and  of 
the  neck  muscles.  No  autopsy  was  obtainable  in  his  one  case  which 
died ;  but  a  bulbar  involvement  was  naturally  suspected,  from  the  char- 
acter of  the  objective  signs.  Less  importance  was  attached  to  coincident 
weakness  of  the  tongue,  muscles  of  deglutition  and  extremities.  Mus- 
cular atrophy  suggested  a  chronic  poliencephalitis.  In  1886  Jendras- 
sik  ^"^  published  a  probable  case  of  the  same  affection,  and  in  1887 
Oppenheim  ^^  called  attention  to  a  similar,  if  not  identical,  symptom- 
complex,  which  he  then  regarded  as  unique,  apparently  not  considering 
Erb's  earlier  cases  as  coming  into  the  same  category.  In  Oppenheim's 
case  a  painstaking  post-mortem  examination  showed  no  lesions,  and  the 
affection  was,  therefore,  characterized  as  a  chronic  progressive  neurosis 

'"Erb:    Arch.  f.  Psych.,  ix.  336,  1870. 

"  Jendrassik :    Arch,  f .  Psych.,  xvii.  301,  1886, 

"  Oppenheim :    Virchow's  Arehiv,  eviii.  522,  1887. 


458  THE    EYE    AND    NERVOUS    SYSTEM 

manifesting  itself  essentially  by  the  symptoms  of  a  glossopharyngo- 
labial  paralysis  without  atrophy.  Goldflam,^®  in  1891,  more  clearly  de- 
fined the  condition  by  laying  stress  upon  the  element  of  muscular  fatigue 
as  distinguished  from  actual  paralysis,  a  matter  later  amplified  by 
Jolly  ^®  in  regard  to  electrical  alterations,  which  he  defined  as  the  "  my- 
asthenic reaction."  These  changes  consist  essentially  in  a  rapid  tiring 
of  the  muscles  under  strong  faradic  stimulation,  manifested  by  an 
increasing  diminution  in  contractility. 

An  important  contribution  to  the  general  subject  was  made  by 
Hoppe^'  in  1892,  from  Oppenheim's  laboratory,  in  which  he  described 
a  case  of  general  motor  weakness,  but  especially  of  the  cranial  nerves, 
of  slow  progression,  in  some  regions  going  on  to  paralysis,  with  electrical 
alterations,  but  without  atrophy;  final  fatal  outcome  and  absolutely 
negative  post-mortem  findings.  Hoppe  considers  this  case  not  identical 
with  Erb's,  chiefly  on  the  ground  of  complete  absence  of  muscular 
atrophy.  He  further  questions  the  utility  of  the  term  neurosis,  as  ap- 
plied to  the  condition  by  Oppenheim,  and  regards  it  as  probably  of 
cortical  origin. 

Since  this  preliminary  work,  which  established  the  disease  on  a 
fairly  firm  basis,  a  very  large  number  of  cases  have  been  reported,  with 
several  critical  reviews  of  the  subject  and  ample  bibliographical  lists.^^ 
The  general  conception  of  the  affection  has  not  been  materially  altered 
by  the  work  of  the  past  few  years,  although  a  certain  light  has  been 
thrown  upon  its  possible  pathological  anatomy  by  Weigert,^^  and  very 
recently  by  Hun  and  his  collaborators,  Drs.  George  Blumer  and  G.  L. 
Streeter,  and  by  Drs.  C.  W.  Burr  and  B.  F.  Stahl. 

Pathological  Anatomy. — According  to  Hun's  tabulation,  autopsies  in 
thirty-two  cases  have  shown  negative  findings  in  the  nervous  system  in 
seventeen  cases.  In  the  remaining  cases  lesions  found  in  the  nervous 
system  were  slight  recent  hemorrhages,  and  uncertain  changes  in  motor 
nerves  and  nuclei  of  the  oblongata,  together  with  certain  abnormalities 
of  development,  which  cannot  be  brought  into  causal  relationship  with 
the  clinical  course  of  the  disease.  Findings  outside  the  nervous  system 
are  probably  of  more  significance;    in  fact,  there  is  small  evidence  to 

'•  Goldflam :  Neurolog.  Ctb.,  x.  162,  1891 ;  also  Deuts.  Zeitsch,  f.  Nervenheilk,, 
iv.  312,  1893. 

"Jolly:    Berl.  klin.  Woch.,  xxxii.  1,  1895. 

*"  Hoppe:    Berl.  klin.  Woch.,  xxix.  332,  1892. 

**  The  more  important  of  these  are  Campbell  and  Bramwell :  Brain,  xxiii.  277, 
1900;  Myers:  Jour,  of  Pathol,  and  Bacteriol.,  viii.  306,  1903;  Hun:  Albany 
Medical  Annals,  xxv,  28,  1904   (124  references). 

"Weigert:    Neurolog.  Ctb.,  xx.  597,  1901. 


BULBAE  AND  PSEUDOBULBAK  DISEASES         459 

show  that  a  primary  affection  of  the  nervous  system  is  in  any  way 
responsible  for  the  symptoms  supposed  to  be  characteristic  of  the  affec- 
tion. Weigert's  observation,  made  in  1901,  is  possibly  of  importance 
in  the  light  of  certain  other  similar  findings,  and  especially  of  Hun's, 
to  which  allusion  has  been  made.  In  a  case  of  myasthenia  gravis  Wei- 
^ert,  in  a  routine  post-mortem  examination,  found  an  extensive  infiltra- 
tion of  the  muscles  with  lymphoid  cells,  which  he  regarded  as  metastases 
•of  a  malignant  tumor  of  the  thymus,  of  lympho-sarcomatous  character. 
The  appearances  were  so  striking  in  this  case  that  Weigert  was  inclined 
to  think  that  had  similar  changes  been  present  in  all  previous  cases,  they 
■could  not  have  been  overlooked,  and,  therefore,  that  one  is  not  justified 
in  assuming  that  such  a  lesion  is  the  constant  cause  of  myasthenia. 
Hun  has  recently  described  a  case  identical  with  Weigert's,  and  on  the 
basis  of  this  fact,  taken  in  conjunction  with  observations  of  similar 
•character  made  by  Link,  Oppenheim  and  Goldflam,  strongly  advocates 
the  theory  that  the  secret  of  the  pathological  anatomy  probably  lies  in 
these  or  closely  analogous  alterations.  A  very  similar  condition  has 
recently  been  reported  by  Burr  and  Stahl  before  the  Philadelphia  Neu- 
Tological  Society,  and  Burr  still  further  discusses  the  subject  in  the 
Journal  of  Nervous  and  Mental  Disease  for  March,  1905.  The  final 
■explanation  of  the  muscular  weakness  and  myasthenic  reaction  Hun 
seeks  in  a  disturbance  of  the  function  of  the  muscle  end-plates,  brought 
about  by  interference  with  the  lymph  circulation  in  the  muscles.  To 
•explain  the  cause  of  the  infiltration  of  the  muscles  with  lymphoid  cells 
•carries  one  wholly  into  the  field  of  speculation  and  requires  no  discus- 
sion here. 

Symptomatology. — The  disease  is  ordinarily  insidious  in  onset,  and 
•characterized  essentially  by  weakness  and  rapid  tiring  of  muscles.  In 
nearly  one-half  the  reported  cases,  according  to  Hun,^^  ptosis  is  a  very 
•early  symptom.  In  forty-five  per  cent,  of  the  cases  ocular  palsy,  either 
ptosis,  or  diplegia,  occurred  as  the  earliest  symptom.  In  a  smaller  pro- 
portion, twenty-three  per  cent.,  involvement  of  the  facial,  hypoglossal 
and  accessory  nerves  was  the  first  symptom.  A  somewhat  larger  number 
•of  cases  gave  as  the  earliest  sign  of  the  disease  weakness  of  the  arms  or 
legs  or  both.  As  the  disease  develops  the  usual  characteristic  picture  is 
•double  ptosis,  double  facial  paresis,  paralysis  of  the  bulbar  nerves,  asso- 
ciated with  dyspnoea,  and  general  bodily  weakness.  Among  these  symp- 
toms the  ocular  palsies  occupy  probably  the  most  conspicuous  place,  and 


"Hun's  paper,  loe.  cit.,  contains  a  most  valuable  analysis  of  earlier  cases  in 
their  various  aspects. 


460  THE    EYE    AND    NERVOUS    SYSTEM 

of  individual  symptoms  ptosis  is  the  most  frequent,  occurring  sooner  or 
later  in  upward  of  eighty-five  per  cent,  of  all  cases.  Various  external 
muscles  of  the  eye  are  also  frequently  involved,  but  the  internal  muscles 
governing  the  pupil  are  very  rarely  included  in  the  muscular  weakness. 

AVilbrand  and  Saenger  ^^  in  an  analysis  of  forty-three  cases  find 
diplegia  in  fourteen ;  ophthalmoplegia  exterior  in  nine ;  weakness  of 
the  sphincter  iridis  and  ciliary  muscles  in  one;  unilateral  ptosis  in 
three;  bilateral  ptosis  in  twenty-nine;  disturbance  in  the  distribution 
of  the  facial  nerve,  a  number  involving  the  orbicularis  oculi,  in  twenty- 
five.  Gowers  ^^  has  recently  called  special  attention  to  the  affection  of 
the  eye  muscles  in  connection  with  a  study  of  three  personal  cases.  At 
first  sight  the  ophthalmoplegia  simulates  the  variety  due  to  nuclear  de- 
generation, but  the  differences  are  probably  significant.  In  the  myas- 
thenic form,  the  muscles  moving  the  eyeball  downward  are  less  involved 
than  those  moving  it  upward,  the  lateral  muscles  are  constantly  but 
irregularly  involved,  but  differing  in  degree  in  associated  muscles  and 
also  showing  striking  variations  at  different  times.  There  is  no  corre- 
spondence in  some  of  the  cases  between  the  degree  of  ptosis  and  the  im- 
pairment of  upward  movements  of  the  eyes.  The  orbicularis  palpe- 
brarum is  often  included  in  the  weakness,  and  the  frontalis  may  be. 
Gowers  also  draws  attention  to  a  characteristic  facial  expression,  which 
he  calls  the  "  nasal  smile,"  brought  about  by  a  failure  of  the  normal 
movement  of  the  corners  of  the  mouth,  the  furrow  caused  by  the  attempt 
to  smile  being  wholly  confined  to  the  upper  lip,  and  ceasing  towards  the 
corners  of  the  mouth.  The  cause  of  this  peculiarity  is  a  weakness  of  the 
levator  labii  superioris. 

The  involvement  of  muscles  supplied  by  the  facial  nerve,  in  general, 
is  a  frequent  sign,  giving  rise  to  so-called  facial  diplegia.  The  muscles 
of  mastication,  speech,  deglutition  and  phonation  may  all  be  weak,  and 
these  disabilities,  if  conjoined  with  weakness  of  the  neck,  body,  and 
extremity  muscles,  reduce  the  patient  to  a  condition  of  practical  help- 
lessness. The  face  is  expressionless,  the  eyes  partially  closed,  the  jaws 
are  moved  weakly,  speech  is  impossible,  swallowing  is  difiicult,  the  head 
cannot  be  voluntarily  supported,  dyspnoea  may  come  on,  and  the  skeletal 
muscles  be  too  weak  to  hold  the  body  erect. 

In  spite  of  such  a  wide  distribution  of  symptoms,  the  condition  is 
in  every  instance  due  to  a  rapid  tiring  of  the  muscles,  associated  as  the 

^Wilbrand  and  Saenger:    Neurologie  des  Auges,  1899. 

*•  Gowers:  Clinical  Lectures,  Blakiston,  1904,  Lecture  on  Myasthenia  and  Oph- 
thalmoplegia, p.  221. 


BULBAE  AND  PSEUDOBULBAR  DISEASES         461 

process  develops  with  more  or  less  persistent  weakness.  As  a  rule  those 
muscles  which  are  most  constantly  in  use  show  the  greatest  weakness  as  a 
result  of  tiring,  but  this  is  by  no  means  invariably  the  case.  The 
order  of  involvement  of  the  muscles  is  also  not  always  as  given  above, 
beginning  in  the  eyes,  and  extending  gradually  downward.  The  reverse 
may  occur,  and  not  infrequently  the  distribution  of  the  weakness  is 
wholly  irregular. 

The  association  of  weakness  and  abnormally  rapid  tiring  of  muscles 
on  use  must  be  regarded  as  an  essential  symptom  of  the  disease.  To 
this  must  be  added  the  peculiar  action  of  the  affected  muscles  on  elec- 
trical stimulation,  first  carefully  studied  by  Jolly.  This  so-called  "  my- 
asthenic reaction"  consists  in  a  rapid  diminution  in  contractility  when 
stimulated  by  a  tetanizing  faradic  current.  The  galvanic  current  has  a 
much  less  tiring  effect  according  to  most  observers,  although  opinions  on 
this  point  are  not  altogether  uniform.  Reaction  of  degeneration  does 
not  occur,  and  muscular  atrophy  forms  no  part  of  the  clinical  picture. 
The  relation  between  voluntary  and  electrical  (faradic)  contraction  of 
the  muscles  remains  undetermined.  Jolly  found  that  exhaustion  took 
place  when  the  muscles  were  voluntarily  contracted,  in  the  same  degree 
as  when  electrically  stimulated ;  others  have  had  a  different  experience. 
The  myasthenic  reaction,  though  not  constant,  occurs  in  some  form  in 
a  large  majority  of  the  cases.  In  fatigue  brought  about  by  other  causes, 
and  in  certain  cerebral  and  spinal  diseases,  an  electrical  reaction  similar 
to  that  occurring  in  myasthenia  has  been  described,  but  the  reaction, 
nevertheless,  remains  a  characteristic  and  striking  feature  of  the  disease. 

The  sensibility  is  not  disturbed  in  the  affection,  and  the  mental 
faculties  remain  unimpaired.  The  knee-jerks  at  times  show  a  tendency 
to  exhaustion;  the  sphincters  are  not  involved.  E^aturally  a  great 
variety  in  the  extent,  grouping  and  degree  of  the  muscular  exhaustion 
leads  to  much  diversity  in  the  clinical  picture.  In  general,  the  combina- 
tion of  cranial  nerve  weakness,  and  especially  ptosis  with  accompanying 
weakness  of  the  skeletal  muscles;  absence  of  muscular  atrophy,  with 
unaltered  electrical  reaction,  apart  from  rapid  tiring  on  faradic  stimula- 
tion ;  tendency  to  variations  from  time  to  time ;  unimpaired  sensibility 
and  freedom  from  mental  defect,  form  a  symptom-complex  which  is 
found  in  no  other  condition. 

Etiology  and  Course. — The  disease  ordinarily  occurs  in  young  adults, 
without  reference  to  sex  or  hereditary  influences.  There  is  no  uni- 
formity in  the  various  conditions  which  have  been  described  antecedent 
to  the  actual  appearance  of  the  disease.  Even  if  the  finding  of  a 
lymphoid  infiltration  of  the  muscles  with  disordered  lymphatic  circula- 
tion be  regarded  as  etiologically  significant,  the  ultimate  cause  still 


462  THE    EYE    AND    NERVOUS    SYSTEM 

remains  obscure.  To  say  that  the  aifection  is  due  to  a  toxaemia  is  rather 
to  restate  the  question  than  offer  a  solution. 

The  onset  of  the  disease  is  usually  insidious,  and  its  course  slow, 
often  with  marked  remissions  followed  by  exacerbations.  Successive 
groups  of  muscles  are  usually  involved,  often  irregularly.  Temporary 
improvement  may  take  place  for  months  or  even  years,  with  final  relapse 
to  the  former  or  to  a  worse  condition.  Variability  in  the  development 
of  symptoms  is  so  constant  a  feature  of  the  disease  as  to  be  of  great 
importance  in  the  diagnosis.  In  ninety-eight  of  the  tabulated  cases  the 
intensity  of  the  symptoms  varied  from  day  to  day  and  in  sixteen  only 
was  the  progression  steady.  A  few  cases  have  been  described  in  which 
the  onset  was  acute,  and  the  disease  fully  developed  in  the  course  of  a 
few  weeks;  these  are,  however,  wholly  exceptional.  Death  has  ter- 
minated the  affection  in  something  less  than  fifty  per  cent,  of  reported 
cases;  a  small  number,  seven,  are  regarded  as  cured,  and  the  others, 
fifty-seven  out  of  one  hundred  and  fourteen,  are  still  living. 

Treatment. — The  essential  of  treatment  is  the  avoidance  of  muscular 
fatigue.  In  serious  cases  the  patient  should  be  kept  in  bed,  and  warned 
against  exertion  of  any  sort.  Electricity  should  be  applied  with  great 
care.  Central  galvanization  is  advisable.  Oppenheim  quotes  a  case 
in  which  a  fatal  outcome  followed  an  attempt  at  artificial  feeding. 
The  food  should  be  of  such  a  character  that  the  effort  of  swallowing  may 
be  reduced  to  a  minimum ;  the  possibility  of  sudden  death  from  suffoca- 
tion should  be  borne  in  mind.  In  milder  cases  less  care  is  required,  but 
the  results  hitherto  attained  are  not  of  such  a  character  as  to  warrant  the 
hope  that  the  course  of  the  disease  can  be  essentially  modified.  Appro- 
priate tonic  and  general  hygienic  and  symptomatic  treatment  will  ac- 
complish something  toward  the  amelioration  of  the  general  condition. 

PARALYZING  VERTIGO    (GERLIER'S   DISEASE). 

The  disorder  known  as  Paralyzing  Vertigo,  or  Gerlier's  Disease,  was 
first  described  by  a  Swiss  physician,  Gerlier,^^  in  1887.  Since  that  time 
he  has  contributed  a  series  of  articles,  amplifying  his  first  detailed 
description  especially  on  the  clinical  side.  David,^^  Haltenhoff,^® 
Eperon,'*"  Ladame,^'  and  Sulzer  ^^  have  made  communications  of  less 


"Gerlier:    Rev.  M6d.  de  la  Suisse  Roinande,  vii.  1,  260,  1887;    idem,  viii.  22,  86, 
1888;    idem,  xi.  201,  260,  1891. 

"David:    Rev.  Med.  de  la  Suisse  Romande,  vii.  65,  1887. 
"Haltenhoff:    Prog.  Med.,  xv.  515,  1887. 
"Eperon:    Rev.  MC'd.  de  la  Suisse  Romande,  ix.  38,  1889. 
"Ladame:    Critical  Digest.     Brain,  xii.  534,  1890. 
''Sulzer:    Rev.  Med.  de  la  Suisse  Romande,  xiii.  695,  1893. 


BULBAK  AND  PSEUDOBULBAR  DISEASES         463 

importance  on  the  clinical  aspect  of  the  subject  or  in  criticism  of  Ger- 
lier's  views. 

The  affection  has  been  observed  in  Europe  only  in  CoUex,  a  canton 
of  Geneva,  and  in  seven  surrounding  hamlets.  Its  victims  are  workers 
in  the  fields,  and  particularly  cowherds.  Vigorous  men  in  the  early 
period  of  life  are  attacked,  during  the  summer  months  only,  and  those 
exposed  to  the  worst  hygienic  conditions  are  usually  the  first  and  most 
seriously  afflicted. 

Symptomatology. — Three  symptoms  are  characteristic;  pain  in  the 
region  of  the  neck,  muscular  weakness  and  ocular  disorders.  These 
may  be  variously  combined,  and  occur  with  varying  degrees  of  intensity 
in  the  same  person  at  different  times  or  in  different  persons.  Vertigo, 
as  ordinarily  understood,  is  a  less  conspicuous  symptom. 

The  neck  pain  is  described  as  a  sense  of  constriction,  with  a  ten- 
dency to  torticollis,  the  pain  often  radiating  to  the  back.  The  muscular 
weakness  is  confined  to  those  muscles  normally  under  control  of  the 
will,  and  the  extensors  are  more  commonly  affected  than  the  flexors. 
The  neck  muscles  are  usually  involved,  but  unsymmetrically  on  the  two 
sides.  The  disturbance  of  the  eyes  is  characterized  by  ptosis,  usually 
unequal  in  the  two  eyes  and  of  varying  degree.  This  is  not  accompanied 
by  strabismus,  or  palsy  either  of  the  external  or  internal  ocular  muscles, 
and  there  is  at  times  a  diminution  in  the  visual  fields.  The  fundus  has 
apparently  not  been  extensively  examined,  but  so  far  as  they  have  gone 
the  ophthalmoscopic  findings  are  negative,  with  the  exception  of  an 
observation  of  Eperon,  in  which  he  found  papillary  hyperemia  and 
retinal  hemorrhage,  and  a  similar  finding  by  Sulzer.  A  subjective  sen- 
sation of  clouding  of  vision,  preceding  the  onset  of  ptosis,  accompanied 
by  diplegia,  and  vertigo,  rarely  circular  in  character,  often  occurs. 

A  typical  attack  may  be  described  as  follows:  The  patient,  pre- 
viously well,  is  suddenly  attacked  by  pain  in  the  neck  and  back;  his 
sight  is  clouded  to  the  point  of  temporary  blindness;  ptosis  develops 
rapidly  along  with  general  weakness  of  neck  and  body  muscles ;  stand- 
ing erect  becomes  difficult ;  he  reels  and  has  all  the  appearance  of  drunk- 
enness. After  a  period  not  exceeding  ten  minutes,  complete  recovery 
takes  place.  During  an  attack  the  tendon  reflexes  are  preserved,  at 
times  increased;  the  skin  sensibility  is  intact,  false  trismus  occurs, 
swallowing  is  often  impossible,  and  general  motor  weakness  dominates 
the  clinical  picture.  The  head  often  falls  forward  on  the  chest,  due  to 
weakness  of  the  neck  muscles,  which,  with  the  ptosis  cutting  off  vision, 
renders  the  patient  for  the  time  being  wholly  helpless.  Nausea  does  not 
occur.    Many  of  the  attacks  are  very  slight  and  merely  momentarily  in- 


464  THE    EYE    AND    NERVOUS    SYSTEM 

capacitate  the  patient,  but  the  foregoing  symptoms  invariably  are  de- 
veloped in  some  degree. 

Etiology. — Nothing  definite  is  known  of  the  cause  of  the  attacks. 
Gerlier  was  of  the  opinion,  though  it  was  not  experimentally  verified, 
that  inasmuch  as  peasants  working  and  sleeping  in  stables  were  usually 
affected,  the  cause  was  to  be  sought  in  an  infection  or  "miasm,"  only 
active  in  the  warm  months  of  summer,  derived  from  the  soil  of  the 
stables.  The  malady  has  been  shown  to  be  seasonal,  always  checked  by 
the  onset  of  cold  weather ;  the  attacks,  furthermore,  occur  usually  with 
striking  regularity  in  the  afternoon,  ceasing  about  an  hour  after  sunset. 
They  are,  however,  unaffected  by  the  administration  of  quinine.  Attacks 
are  iftduced  by  excitement,  brilliant  light,  by  gazing  fixedly  at  an  object, 
or  by  the  contemplation  of  a  wide  space.  Ladame  regards  the  affection 
as  hysterical,  and  wholly  due  to  suggestion.  This  Gerlier  rather  in- 
dignantly denies,  giving  as  a  conclusive  argument  the  fact  that  he  has 
observed  the  same  attacks  in  cats,  which  presumably  are  not  suggestible. 
Eperon  is  inclined  to  attribute  the  attacks  to  a  meningeal  hypersemia, 
possibly  allied  to  sunstroke ;  Sulzer  considers  the  possibility  of  lesions  of 
the  occipital  lobes,  accompanied  by  descending  neuritis  of  the  optic 
nerves. 

Miura  in  1897  described  a  similar  affection  occurring  in  the  north- 
ern provinces  of  Japan,  to  which  he  gave  the  name  "  Kubisagari."  The 
clouding  of  vision  he  regarded  as  a  nervous  asthenopia,  and  the  diplegia 
present  in  his  cases  as  an  insufficiency  or  paresis  of  the  internal  recti. 
The  possible  relationship  of  these  conditions  with  asthenic  bulbar  paral- 
ysis, and  also  with  the  rare  family  periodic  paralysis,  should  be  borne 
in  mind. 

Treatment. — Iodide  of  potash,  cold  bathing,  and  generally  improved 
hygiene  have  been  recommended.  The  disease  is  never  fatal,  and  is 
invariably  terminated  by  the  onset  of  cold  weather. 


CHAPTEK    XL 

ARTERIOSCLEROSIS;  MULTIPLE  SCLEROSIS;  PSEUDOSCLE- 
ROSIS; DIFFUSE  SCLEROSIS;  FRIEDREICH'S  DISEASE; 
PARALYSIS   AGITANS. 

By  WILIAM  HIRSCH,  M.D. 

ARTERIOSCLEROSIS. 

AccoBDiNG  to  our  present  views,  arteriosclerosis  forms  merely  a 
part  of  a  general  angiosclerosis, — i.e.,  an  affection  of  the  entire  vascular 
system,  which  may  be  due  either  to  a  general  regressive  metamorpho- 
sis, to  the  influence  of  toxic  agents,  or  to  congenital  abnormalities  in  the 
structure  of  the  vessels.  Inasmuch  as  the  integrity  of  every  organ 
depends  on  its  proper  nutrition  and  blood  supply,  and  this  again  is 
dependent  on  an  unimpaired  vascular  system,  it  is  evident  that  arterio- 
sclerosis is  apt  to  produce  morbid  changes  in  any  and  every  part  of 
the  body. 

During  the  earlier  stages  of  arteriosclerosis  the  affections  of  the 
various  organs  consist  merely  of  functional  disturbances,  such  as  irreg- 
ularities in  circulation  are  apt  to  produce.  But  as  soon  as  the  morbid 
changes  in  the  blood-vessels  become  more  marked,  and  consequently  the 
circulatory  disturbances  more  severe,  the  various  organs  are  apt  to 
become  subject  to  organic  diseases. 

The  functional  disturbances  which  a  general  arteriosclerosis  is  apt 
to  produce  in  the  eye  correspond  to  analogous  symptoms  in  other  organs. 
The  muscles  are  easily  fatigued  and  are  lacking  in  endurance  and 
power  of  resistance.  Patients  complain  of  difficulty  in  reading  or  of 
headaches  after  using  the  eyes,  in  spite  of  normal  or  by  glasses  cor- 
rected lenses.  Besides  these  disturbances  in  accommodation  and  con- 
vergence, the  diminution  of  muscular  tonus  may  manifest  itself  by 
slight  difficulties  in  moving  the  eyeballs,  by  incomplete  and  transitory 
ptosis,  or  by  slight  nystagmus,  especially  in  horizontal  movements  of 
the  eyes. 

Arteries  which  are  undergoing  sclerotic  changes  are  subject  to  such 
functional  disturbances  as  spasm  and  temporary  paralysis.  The  well- 
known  symptom  of  a  temporary  scintillating  scotoma  is  in  all  proba- 
bility produced  by  a  spasm  of  the  blood-vessels  supplying  the  cortical 
lobe.     Such  spasms  may  occur  also  in  the  blood-vessels  of  the  retina, 

465 
30 


466  THE    EYE    AND    NERVOUS    SYSTEM 

and  then  produce  specks  before  the  eyes,  scintillation,  and  peculiar 
color-sensations.  Another  symptom  which  is  evidently  produced  by 
vascular  spasm,  and  therefore  may  occur  in  arteriosclerosis,  is  that  of 
so-called  "  paroxysmal"  or  "  epileptiform"  amaurosis.  The  symptom 
consists  of  a  sudden  attack  of  blindness  in  one  or  both  eyes,  lasting  from 
a  few  seconds  to  several  hours,  without  any  ophthalmoscopic  changes. 

In  some  cases  of  general  arteriosclerosis  (according  to  some  writers 
in  50  per  cent.)  a  sclerotic  process  of  the  retinal  arteries  can  be  recog- 
nized with  the  ophthalmoscope.  While  under  normal  conditions  the 
wall  of  a  retinal  vessel  is  invisible,  a  sclerotic  artery  may  show  a  gray- 
ish shadow  along  its  course.  At  some  places  the  lumen  of  the  vessel  may 
have  become  narrower  on  account  of  the  thickening  of  the  wall.  Such 
places  appear  in  the  ophthalmoscopic  picture  as  fusiform  yellowish- 
gray  patches.  In  extreme  cases  the  well-known  red  lines  on  the  retina 
seem  to  be  changed  into  thin  white  threads. 

Another  objective  symptom  of  sclerosis  of  the  retinal  vessels  may  be 
the  presence  of  an  arterial  pulse,  which,  of  course,  must  be  distinguished 
from  the  pulse  of  pressure  or  the  intermittent  entrance  of  blood.  Ac- 
cording to  Thoma,  this  phenomenon  occurs  during  the  early  stage  of 
arteriosclerosis,  and  differs  from  the  pulsation  in  aortic  insufficiency  by 
its  lateral  motions  and  flexion  of  the  vessels,  so  that  it  is  a  locomotion 
rather  than  a  true  change  of  calibre.  Thoma  explains  this  phenomenon 
by  the  fact  that  in  the  beginning  of  arteriosclerosis  the  walls  of  the  ves- 
sels are  more  elastic  than  under  normal  conditions;  as  soon,  however, 
as  the  thicker  layer  of  connective  tissue  in  the  intima  between  endothe- 
lium and  elastica  interna  has  been  formed,  liie  wall  becomes  firmer  again 
and  gradually  loses  its  elasticity. 

Sometimes  the  shape  of  the  pupil  may  become  slightly  changed. 
It  may  be  oval  instead  of  circular  or  show  some  other  small  irregulari- 
ties of  its  circumference. 

Occasionally  an  (edematous  swelling  of  the  lids  may  occur  or  slight 
hemorrhages  may  take  place  in  the  conjunctiva  or  retina.  These  hem- 
orrhages into  the  substance  of  the  eye,  although  mostly  harmless  in 
themselves,  may  be  of  great  prognostic  value,  inasmuch  as  similar  proc- 
esses in  any  other  part  of  the  body  would  necessarily  escape  observa- 
tion. Hasket  Derby  found  that  of  31  patients  who  consulted  him 
for  retinal  hemorrhages  nearly  50  per  cent,  died  within  two  years, 
either  from  heart  disease  or  from  apoplexy. 

In  cases  of  cerebral  arteriosclerosis  the  optic  nerve  may  be 
injured  by  direct  pressure  of  an  atheromatous  vessel.  The  anatomical 
conditions  for  such  an  occurrence  are  especially  favorable  in  the  optic 


ARTEKIOSCLEROSIS  467 

canal.  Even  under  normal  conditions  the  external  shape  of  the  optic 
nerve  may  undergo  various  changes.  By  its  lower  surface  the  nerve 
rests  on  the  internal  carotid  artery,  which,  of  course,  performs  most 
vigorous  pulsations.  The  ophthalmic  artery,  which  branches  off  in  the 
form  of  an  upwardly  convex  arch,  also  pulsates  very  strongly,  espe- 
cially at  its  origin.  Every  pulsation  of  these  two  vessels  presses  the 
nerve  against  the  fibrous  roof  of  the  intracranial  part  of  the  optic  canal, 
and  thus  exerts  an  important  influence  on  the  shape  of  the  nerve  in  this 
region.  It  is  evident  that  this  influence  must  be  greatly  increased  if 
the  diameter  of  these  vessels  becomes  enlarged,  their  walls  hardened, 
and  their  course  more  curved  by  a  sclerotic  process. 

The  pressure  which  a  sclerotic  ophthalmic  artery  is  apt  to  exert  on 
the  optic  nerve  may  show  itself  in  various  ways.  As  a  rule,  the  artery 
produces  a  groove  at  the  central  part  of  the  lower  surface  of  the  nerve, 
so  that  its  substance  is  displaced  to  both  sides,  assuming  the  shape  of  a 
kidney.  If  the  process  is  very  slow  and  gradual  and  the  anatomical 
conditions  are  such  that  the  nerve  has  sufficient  space  to  expand  on 
both  sides,  this  transfiguration  may  reach  a  considerable  degree  without 
impairing  the  function  of  the  nerve.  The  optic  nerve  behaves  in  this 
respect  less  like  a  nerve  than — what  it  really  is — a  part  of  the  brain. 
It  is  well  known  what  great  endurance  the  substance  of  the  brain  is 
apt  to  offer  to  a  slowly  growing  tumor,  that  sometimes  a  growth  may 
reach  an  enormous  size  without  destroying  the  brain-substance,  merely 
displacing  and  deforming  it. 

Inasmuch,  however,  as  there  is  a  bony  wall  at  the  opposite  side  of 
the  pressure,  and  as  the  space  within  the  canal  is  extremely  limited, 
the  optic  nerve  has  only  a  small  chance  of  escaping  injury,  and,  espe- 
cially if  the  changes  in  the  artery  occur  a  little  more  rapidly,  a  pro- 
gressive atrophy  of  the  nerve  is  unavoidable. 

At  first  there  may  be  only  a  slight  atrophy  in  the  centre,  corre- 
sponding to  the  impression  of  the  artery.  In  cases  of  this  kind  the 
nerve  appears  to  be  divided  into  two  halves,  connected  by  a  white 
bridge,  which  is  formed  by  compressed  septa  and  atrophic  fi^bres.  But 
gradually  the  atrophy  may  progress  until  complete  blindness  occurs. 

According  to  the  position  of  the  ophthalmic  artery,  the  compres- 
sion of  the  nerve,  instead  of  taking  place  in  the  centre,  may  occur  more 
laterally,  so  that  only  a  small  portion  of  the  nerve  is  cut  off,  the  larger 
part  being  displaced  to  the  other  side,  filling  the  empty  space  between 
the  arteries  and  the  margin  of  the  canal  in  a  triangular  shape. 

The  ophthalmoscopic  picture  of  the  atrophic  nerve  may,  therefore, 
vary  greatly,  according  to  the  relation  between  the  artery  and  the  nerve 


468  THE    EYE    AND    XERVOUS    SYSTEM 

and  the  intensity  and  rapidity  of  the  increasing  pressure.  The  atrophy 
may  be  confined  to  one  group  of  fibres,  like  the  "crossed,"  the  "  non- 
crossed,"  or  the  "  papillo-macular"  fibres,  and  in  some  cases  it  might 
be  possible  to  recognize  approximately  the  seat  of  the  pressure  from  the 
fibres  involved  in  the  atrophic  process. 

The  prolongation  of  the  bony  part  of  the  optic  canal  within  the 
cranial  cavity  is  formed  by  a  ring  of  thick,  fibrous  connective  tissue. 
This  ring  is  covered  by  the  dura  mater,  which  near  the  optic  foramen 
forms  a  tensely  stretched  free  margin.  The  base  of  the  intracranial 
portion  of  the  canal  is  formed  by  the  internal  carotid  artery.  If, 
therefore,  this  artery  becomes  hardened  and  considerably  enlarged  in 
diameter,  it  may  push  both  optic  nerves  upward  and  forward  against 
the  hard  margin  of  the  dura  mater,  crushing  and  destroying  both 
nerves. 

A  sclerotic  process  in  the  carotid  and  in  the  vessels  of  the  circle  of 
Willis  is,  of  course,  apt  to  produce  atrophy  of  one  or  both  optic  nerves 
by  exerting  pressure  in  the  region  of  the  chiasma.  It  is  evident  that 
eventually  under  suitable  conditions  the  various  forms  of  hemianopsia 
may  be  produced  by  a  corresponding  pressure. 

So,  then,  we  see  that  sclerosis  of  the  cerebral  arteries  may  affect 
the  optic  nerves  in  numerous  ways.  We  may  have  partial  or  com- 
plete blindness  in  one  or  both  eyes;  we  may  have  a  narrowing  of  the 
visual  field  or  central  scotoma;  we  may  have  hemianopsia  or  ambly- 
opia. Any  of  these  conditions  may  occur  during  the  course  of  a  general 
arteriosclerosis,  and  most  cases  of  so-called  "  spontaneous  optic  atrophy 
of  old  age"  are  evidently  due  to  a  sclerotic  process  in  the  cerebral 
arteries. 

Arteriosclerosis  has  been  regarded  as  a  cause  of  quite  a  number  of 
diseases  of  the  eye.  We  will  only  mention  these  conditions  here. 
Michel  and  his  pupils  have  held  that  cataract  can  be  produced  by 
sclerosis  of  the  carotid  artery.  According  to  some  authors,  glaucoma 
is  produced  by  a  general  arteriosclerosis  and  the  consequent  disturbances 
in  the  circulation.  Embolism  of  the  central  artery  may  be  due  to  a 
sclerosis  of  vessels  belonging  to  quite  a  different  region  of  the  body. 
A  disease  which  only  recently  has  been  described  more  accurately,  and 
which  is  supposed  to  stand  in  close  relation  to  arteriosclerosis,  is  "  reti- 
nitis circinata." 

There  can  be  no  doubt  that  arteriosclerosis  forms  one  of  the  most 
important  etiological  factors  in  the  production  of  diseases  in  general, 
and  the  more  our  knowledge  of  this  subject  advances  the  more  we  will 
appreciate  this  important  fact. 


MULTIPLE   SCLEROSIS  469 

MULTIPLE  SCLEROSIS;    PSEUDOSCLEROSIS; 
DIFFUSE  SCLEROSIS. 

The  optic  nerve  is  affected  in  nearly  50  per  cent,  of  all  cases  of 
multiple  sclerosis,  and,  if  we  consider  that  the  changes  in  the  optic 
disk  may  constitute  one  of  the  earliest  manifestations  of  the  disease,  or 
may  even  precede  all  other  symptoms  by  several  years,  we  will  readily 
appreciate  the  great  diagnostic  value  of  the  ophthalmoscopic  findings 
in  this  disorder. 

In  order  fully  to  understand  the  nature  of  the  various  affections 
of  the  optic  nerve  in  multiple  sclerosis,  we  must  always  bear  in  mind 
the  fact  that  the  optic  nerve  is  not  a  nerve  in  the  same  sense  as  the 


Fig.  1.— Multiple  sclerosis,  showing  a  sclerotic  area  in  the  oculomotor  nucleus.    (Case  of  Dr.  Spiller.) 

other  peripheral  nerves,  but  forms  a  part  of  the  brain,  so  that  it  would 
be  much  more  appropriate  to  speak  of  the  optic  tract  rather  than  of  the 
optic  nerve,  even  in  its  most  peripheral  parts. 

The  optic  nerve  represents  a  system  of  peurones,  and  corresponds 
in  every  respect  to  other  systems,  such  as  the  posterior  columns  or  the 
pyramidal  tracts,  and  its  affections  in  the  various  diseases  of  the  cen- 
tral nervous  system  are  entirely  analogous  to  the  affections  of  the  other 
systems.  In  locomotor  ataxia,  for  instance,  the  system  becomes  affected 
as  such,  as  a  unit.  The  posterior  columns  degenerate  as  a  system,  and, 
if  the  optic  nerve  is  affected  at  all,  it  degenerates  also  as  a  system,  under- 


470  THE    EYE    AND    NERVOUS    SYSTEM 

going  complete  atrophy.  Multiple  sclerosis,  however,  is  not  a  systemic 
disease.  No  system  is  affected  as  such.  The  multiple  plaques  are  dis- 
tributed indiscriminately  through  the  entire  brain  and  cord,  regardless 
of  any  order  or  system.  One  plaque  may  reach  from  one  system  to  the 
other,  destroying  a  little  here  and  a  little  there.  The  posterior  col- 
umns may  be  affected  at  various  levels,  but  they  are  not  apt  to  be  either 
more  or  less  affected  than  any  other  system.  According  to  this  character 
of  the  disease,  every  system  is  more  or  less  affected  in  multiple  sclerosis, 
but  no  system  is  affected  entirely  throughout  its  course  as  is  the  case  in 
the  systemic  diseases. 

The  optic  atrophy  in  multiple  sclerosis  is,  therefore,  very  different 
from  that  in  locomotor  ataxia.  In  the  latter  disease  the  affection  is 
symmetrical,  it  progresses  slowly  but  surely,  and  ends  invariably  in 
complete  atrophy  of  both  nerves.  The  atrophy  is  from  the  start 
equally  distributed  over  the  entire  disk.  In  multiple  sclerosis,  on  the 
other  hand,  the  atrophy  is  hardly  ever  complete.  There  is  either  a 
partial  atrophy  distributed  equally  over  the  entire  disk  or  a  partial 
atrophy  confined  to  its  temporal  half.  The  affection  in  a  large  per- 
centage of  cases  remains  one-sided.  The  morbid  process  may  reach  a 
certain  stage  and  then  remain  stationary  for  a  long  time.  The  atrophic 
process  never  produces  complete  blindness  in  multiple  sclerosis.  On 
the  contrary,  an  improvement  ha"s  been  reported  in  50  per  cent,  of  the 
cases.     Occasionally  even  a  restitutio  ad  integrum  has  been  observed. 

In  some  cases  there  was  an  optic  neuritis  either  one-sided  or  bilat- 
eral. Sometimes  the  optic  atrophy  shows  traces  of  a  previous  neuritis, 
so  that  it  can  be  assumed  that  in  a  certain  percentage  the  atrophy  is  a 
secondary  condition  to  a  primary  neuritis. 

The  ophthalmoscopic  finding  often  seems  not  to  be  in  accordance 
with  the  real  condition  of  the  optic  nerve.  On  the  one  hand  there  are 
cases  in  which  vision  seems  to  be  considerably  impaired  in  spite  of 
an  absolutely  negative  ophthalmoscopic  picture,  and  on  the  other  hand 
the  disk  may  appear  considerably  atrophied  and  still  the  power  of 
vision  not  be  diminished  in  a  degree  which  one  would  expect. 

This  peculiarity,  as  well  as  the  other  irregularities  described  above, 
can  easily  be  explained  by  the  anatomical  nature  of  the  disease.  A 
plaque  may  destroy  considerable  substance  of  the  nerve  without  affect- 
ing the  parts  in  the  immediate  neighborhood  of  the  retina,  thus  offering 
the  clinical  picture  of  a  retrobulbar  neuritis.  On  the  other  hand,  an 
affection  of  the  retinal  part  of  the  optic  nerve  does  not  .indicate  its 
entire  condition,  as  it  does  in  other  cases  of  atrophy. 

Of  clinical  significance  in  optic  atrophy  of  multiple  sclerosis  is  the 


MULTIPLE   SCLEROSIS 


471 


irregular,  atypical  behavior  of  the  visual  field.  There  is  no  homo- 
geneous narrowing,  as  in  cases  of  tabes,  but  a  most  irregular  constric- 
tion of  the  peripheral  line.  There  may  be  a  central  scotoma  or  a  com- 
bination of  this  with  an  irregular  narrowing  of  the  field.  In  a  few 
cases  an  annular-shaped  scotoma  has  been  found,  and  in  other  rare 
instances  small  parts  of  the  periphery  were  alone  preserved  while  the 
function  of  all  the  rest  of  the  nerve  had  been  lost.  Sometimes  there  is 
a  central  scotoma  only  for  colors,  or  in  some  cases  a  general  achroma- 
topsia. 


Fig.  2.— Multiple  sclerosis  of  the  optic  nerve.    (Case  of  Dr.  Spiller.) 


Paralysis  of  the  muscles  of  the  eye  is  a  comparatively  frequent 
symptom  in  multiple  sclerosis,  the  various  statistics  showing  an  aver- 
age of  about  20  per  cent.  The  nature  of  the  paralysis  is  always  that  of 
a  central  lesion,  characterized  by  a  conjugate  paralysis,  paralysis  of 
convergence,  and  the  impairment  of  isolated  muscles. 

The  paralysis  itself  is  mostly  an  incomplete  one,  and  its  clinical 
course  is  characterized  by  manifold  changes,  such  as  improvements, 
exacerbations,  intermissions,  and  relapses.  The  irregularity  and  the 
atypical  course,  therefore,  correspond  exactly  to  the  pathological  condi- 
tion of  the  optic  nerve.  The  latter  differs,  however,  from  the  nerves  of 
the  eye-muscles  by  its  frequent  peripheral  lesions,  the  other  nerve- 
trunks  being  affected  only  very  rarely  in  multiple  sclerosis.  Here  again 
the  optic  nerve  behaves  like  a  part  of  the  brain,  differing  also  in  this 
respect  from  all  the  true  peripheral  nerves. 

Paralysis  of  the  abducens  seems  to  be  the  most  frequent  affection. 
Then  follows  the  oculomotorius,  which,  however,  is  always  affected  only 


472  THE    EYE    AND    NERVOUS    SYSTEM 

partially  and  incompletely;  a  complete  paralysis  of  all  branches  of  the 
oculomotorius,  such  as  is  seen  in  affections  of  the  trunk  of  this  nerve, 
hardly  ever  occurs  in  multiple  sclerosis.  Of  the  conjugate  movements 
those  to  right  and  left  are  more  frequently  affected  than  the  vertical 
movements  of  the  eyeball. 

There  may  be  disturbances  in  the  movements  of  convergence.  In 
cases  of  this  kind  the  recti  externi  appear  impaired  in  this  function, 
while  in  other  functions,  such  as  the  conjugate  lateral  movements, 
they  seem  to  be  entirely  intact.  This  shows  very  plainly  the  central 
nature  of  the  lesion. 

Ptosis  has  been  observed  in  quite  a  number  of  cases,  but,  like  the 
other  affections,  offers  the  character  of  incompleteness.  It  is  more  fre- 
quently unilateral  than  bilateral,  and  is  often  associated  with  double 
vision  or  other  disturbances  of  sight. 

Partial  paralysis  and  disturbances  in  the  function  of  the  eye  mus- 
cles may  be  produced  by  cortical,  subcortical,  nuclear,  and  fascicular 
lesions,  according  to  the  localization  of  the  sclerotic  plaques,  and  we 
must  admit  that  in  a  great  many  instances  we  are  still  unable  to  localize 
the  lesion  accurately  from  its  clinical  manifestations. 

A  symptom  to  which  a  great  diagnostic  importance  is  generally 
attached  in  multiple  sclerosis  is  nystagmus.  Here  we  have  to  distin- 
guish between  the  nystagmus  proper,  a  condition  in  which  the  eyeballs 
are  in  constant  motion,  and  the  so-called  "  intentional  nystagmus,"  or 
"nystagmus  of  motion,"  in  which  the  oscillatory  movements  do  not 
take  place  when  the  eyeballs  are  at  rest,  but  only  when  they  are  turned 
towards  an  object,  especially  at  the  end  of  the  horizontal  movements. 
It  is  the  latter  form  which  we  find  most  frequently  in  multiple 
sclerosis.  Inasmucli,  however,  as  slight  oscillatory  horizontal  motions 
of  the  eyeballs  after  a  sudden  extreme  lateral  movement  may  occur 
also  in  hysterical  or  even  normal  individuals,  we  have  to  be  very  careful 
not  to  attach  too  much  importance  to  this  symptom,  or  at  least  confine 
its  diagnostic  value  to  the  more  marked  cases.  Sometimes  the  twitch- 
ing of  the  eyeballs  takes  place  only  with  the  horizontal  motions,  while 
in  other  cases  every  motion,  even  vertical  ones,  may  be  accompanied  by 
this  phenomenon. 

Quite  a  number  of  theories  have  been  offered  to  explain  this  peculiar 
symptom  of  the  disease,  of  which  we  will  mention  only  the  most 
important. 

Some  authors  regard  the  nystagmus  of  multiple  sclerosis  analogous 
to  the  intentional  tremor  of  the  extremities,  due  to  a  paralysis  of  the 
centres  of  association  or  to  an  affection  of  the  peripheral  nerves.   Others 


MULTIPLE  SCLEKOSIS 


473 


explain  the  nystagmus  by  a  disturbance  in  the  equilibrium,  due  to  the 
insufficiency  of  certain  groups  of  muscles.  According  to  an  other  theory 
the  nystagmus  is  produced  by  a  diminution  of  the  tonus  of  the  muscles, 
the  innervations  from  the  centres  being  interrupted  and  impaired  by 
the  various  sclerotic  lesions. 

Whatever  the  true  cause  of  the  nystagmus  may  be,  so  much  is  cer- 
tain, that  it  is  located  in  the  central  nervous  system,  in  the  brain  or  in 
the  medulla  oblongata.  The  various  territories,  which  have  been 
claimed  to  be  responsible  for  this  phenomenon,  are  still  very  numerous, 
and  in  all  probability  we  have  to  deal  here  with  a  condition  which  can 
be  brought  about  by  manifold  lesions  in  the  brain. 


Fig.  3.— Multiple  sclerosis  of  the  optic  chiasm  and  one  optic  nerve.    (Case  of  Dr.  Spiller.) 

The  function  of  the  pupils  remains  normal,  as  a  rule,  throughout 
the  entire  course  of  the  disease.  If,  however,  we  bear  in  mind  the  ana- 
tomical picture  of  multiple  sclerosis,  if  we  consider  that  sclerotic 
plaques  are  apt  to  occur  in  any  part  of  the  central  nervous  system,  we 
will  readily  understand  that  there  is  really  no  symptom  in  neuropath- 
ology which  may  not  occasionally  occur  in  this  disease.  Abnormal  con- 
ditions of  pupils,  therefore,  have  been  reported,  but  they  really  do  not 
belong  to  the  characteristics  of  the  disease,  and  if  they  do  occur,  they 
mislead  rather  than  assist  in  diagnosis.       The  pupillary  symptoms. 


474  THE    EYE    AND    NERVOUS    SYSTEM 

Avhich  were  observed  in  very  rare  cases,  were:  myosis,  difference  of 
pupils,  diminution  and  exaggeration  of  light-reflex,  loss  of  light-reflex, 
loss  of  reflex  on  accommodation  and  paradoxic  reaction. 

There  is  hardly  any  other  disease  of  the  central  nervous  system 
in  which  the  clinical  phenomena  of  the  eye  are  so  characteristic  of  the 
pathological  process,  and  in  which,  therefore,  the  symptoms  are  of  the 
same  significance  as  in  multiple  sclerosis. 

There  is  a  disease,  the  clinical  manifestations  of  which  resemble 
multiple  sclerosis,  but  wherein  the  anatomical  changes  do  not  corre- 
spond to  the  clinical  symptoms.  This  affection  was  first  described  by 
Westphal  under  the  name  of  pseudo-sclerosis,  and  recently  Struempell 
has  thrown  more  light  on  this  peculiar  subject.  It  is  of  special  im- 
portance to  us,  inasmuch  as  the  condition  of  the  eye  forms  one  of  the 
main  factors  in  the  differential  diagnosis  between  this  affection  and 
genuine  multiple  sclerosis.  While  the  other  symptoms  offer  a  great 
resemblance  to  the  clinical  features  of  multiple  sclerosis,  the  eye  symp- 
toms always  remain  absent  in  pseudo-sclerosis.  There  is  no  optic 
atrophy,  no  muscular  palsy,  no  nystagmus. 

Another  affection,  which  offers  many  clinical  resemblances  to  mul- 
tiple sclerosis,  pseudosclerosis  and  general  paresis,  is  called  diffuse 
cerebral  sclerosis.  This  is  a  disease  which  may  occur  at  all  ages,  in 
children  as  well  as  in  adults.  The  main  symptoms  consist  of  spasticity 
of  lower  and  upper  extremities,  increased  reflexes,  mental  apathy  and 
dementia  and  various  palsies  which  may  increase  to  complete  paralysis. 
This  disease  also  is  characterized  by  the  absence  of  any  ocular  symp- 
toms, optic  neuritis  having  been  observed  only  in  a  single  case 
(Heubner). 

FRIEDREICH'S  DISEASE. 

In  spite  of  the  general  interest  which  was  created  by  Friedreich's 
first  publications  and  which  has  prevailed  ever  since,  in  spite  of  the 
numerous  excellent  clinical  observations  and  anatomical  investigations 
of  this  disease,  we  are  still  far  from  having  reached  a  generally  accepted 
view  regarding  the  nature  and  pathology  of  this  affection.  Even  with 
reference  to  the  clinical  features  of  the  disease,  there  still  exist  great 
differences  of  opinion,  especially  as  to  the  grouping  into  various  types. 

As  far  as  ocular  symptoms  were  concerned,  it  was  emphasized  by 
Friedreich  himself,  that  the  disease  is  characterized  by  a  complete 
absence  of  any  abnormalities  or  disturbances  in  the  eyes,  with  the 
exception  of  one  symptom,  namely,  a  certain  form  of  nystagmus. 

This  nystagmus,  which  is  sometimes  called  pseudo-nystagmus,  or 


FRIEDREICH'S  DISEASE  475 

atactic  nystagmus,  does  uot  consist  of  the  rhythmical  oscillations  of 
the  eyeball  like  the  ordinary  nystagmus,  regardless  whether  the  eyes  are 
at  rest  or  whether  movements  of  some  kind  are  performed ;  it  consists 
of  irregular  twitchings,  which  occur  only  when  the  eyes  are  fixed  on  a 
moving  object,  especially  in  the  horizontal  direction.  When  the  eyes 
are  at  rest,  or  when  they  are  fixed  at  a  point  in  the  sagittal  plane,  they 
are  as  a  rule  perfectly  quiet.  This  form  of  nystagmus,  which  resem- 
bles that  of  multiple  sclerosis,  only  being  more  intense  than  the  latter, 
forms  one  of  the  most  constant  phenomena  of  Friedreich's  disease. 
Only  very  rarely  is  this  symptom  absent ;  but  then  it  can  sometimes 
be  produced  artificially  by  turning  the  patient  several  times  around 
his  axis.  In  all  probability  this  phenomenon  has  to  be  considered  as  a 
disturbance  in  co-ordination,  as  an  atactic  condition  of  the  muscles  of 
the  eye. 

As  said  before,  this  was  claimed  by  Friedreich  to  be  the  only  ocular 
s}Tnptom  of  the  disease,  and  up  to  the  present  day  a  great  many  authors 
regard  all  other  abnormalities  of  the  eyes  as  not  belonging  to  this 
affection,  and  therefore  as  a  contraindication  to  the  diagnosis.  Inas- 
much, however,  as  soon  afterwards  a  considerable  number  of  cases  were 
reported,  which  did  offer  ocular  symptoms  and  differed  also  in  other 
resj)ects  from  the  cases  described  by  Friedreich,  Pierre  Marie  described 
another  clinical  picture  under  the  name  of  heredoataxie  cerebelleuse 
(hereditary  cerebellar  ataxia).  This  disease,  which  had  a  great  many 
traits  in  common  with  Friedreich's  disease,  such  as  the  familiar  char- 
acter, the  ataxia  and  progressive  nature,  differed  from  it  by  the  condi- 
tion of  the  patellar  reflexes,  which  were  increased  here  and  absent  in 
Friedreich's  disease,  and  also  in  the  symptoms  of  the  eye,  muscular 
palsies,  loss  of  pupillar  reflex,  disturbances  of  vision,  various  affections 
of  the  optic  nerve  being  comparatively  frequent  in  this  disease. 

A  theory  had  been  offered  according  to  which  Friedreich's  disease 
consisted  of  a  maldevelopment  of  certain  parts  of  the  spinal  cord,  while 
the  affection  described  by  Marie  was  due  to  an  abnormal  cerebellum. 

The  attempt  to  keep  these  two  types  of  the  disease  apart  has  not 
been  successful.  The  more  observations  were  put  on  record,  and  the 
more  attention  was  given  to  this  interesting  disease,  the  more  it  was 
realized  that  such  a  division  is  more  or  less  arbitrary.  It  soon  became 
necessary  to  regard  some  cases  as  a  "  mixed  type,"  as  they  offered 
features  of  each  group  in  equal  proportion.  Some  cases  were  diagnosed 
as  Friedreich's  disease  by  one  observer  and  as  cerebellar  ataxia  by 
another.  In  a  few  instances  both  diagnoses  had  to  be  made  among 
brothers,  one  resembling  the  one  and  the  other  the  other  type. 


476  THE    EYE    AND    NERVOUS    SYSTEM 

Even  anatomically  it  was  not  possible  to  keep  the  two  types  apart. 
In  some  cases  both  the  cerebellum  and  the  spinal  cord  were  affected. 
In  others  the  anatomical  findings  seemed  not  to  correspond  to  the 
clinical  features.  According  to  one  theory  the  disease  always  consists 
of  a  spinocerebellar  affection,  the  tracts  communicating  between  the 
cord  and  the  cerebellum  in  both  directions  being  affected. 

The  division  of  the  disease  into  these  various  types  will  in  all  prob- 
ability not  withstand  future  investigations.  All  that  we  can  say  to-day 
is,  that  clinically  as  well  as  anatomically  the  features  of  the  disease  may 
vary,  and  that  we  must  not  draw  the  line  too  closely  in  making  the  diag- 
nosis, but  rather  allow  a  certain  latitude,  even  if  the  clinical  picture 
differs  from  the  original  description  of  Friedreich. 

As  far  as  the  eye-symptoms  are  concerned  we  may  say  that  the 
special  form  of  nystagmus,  which  was  described  above,  is  by  far  the 
most  frequent  and  constant  symptom  of  the  disease,  but  that,  in  some 
cases  it  may  be  absent.  Ocular  palsies,  optic  neuritis,  and  even  the 
Arg;^'ll-Robertson  pupil  may  occur  in  the  disease,  although  they  are 
comparatively  rare. 

PARALYSIS  AGITANS. 

Ocular  symptoms  do  not  form  an  essential  feature  of  paralysis  agi- 
tans.  There  are  a  few  cases  on  record  in  which  paralysis  agitans  was 
combined  with  other  diseases  of  the  central  nervous  system,  as,  for 
instance,  locomotor  ataxia.  The  l6ss  of  pupillar  reflexes  or  affections 
of  the  optic  nerve  in  cases  of  this  kind  would,  of  course,  not  form  a 
feature  of  paralysis  agitans,  but  of  the  complicating  disease. 

Inasmuch,  however,  as  in  paralysis  agitans  the  muscular  apparatus 
of  the  entire  body  is  apt  to  be  affected  in  the  characteristic  manner,  it 
is  evident  that  the  muscles  of  the  eye  may  once  in  a  while  show  the 
same  morbid  condition,  although  as  a  rule  they  escape  even  this  affection. 

The  characteristic  condition  of  the  muscles  consists  of  the  typical 
rhythmical  tremor,  and  of  a  Very  marked  tension,  which  interferes  with 
normal  motion,  so  that  in  the  later  stages  the  individual  may  become 
nearly  unable  to  perform  any  movements  at  all,  although  there  is  no 
true  paralysis  in  the  ordinary  sense.  The  main  difficulty  in  performing 
motions  adequate  to  their  purpose  seems  to  arise  from  the  inability  of 
the  patient  to  contract  a  group  of  muscles  suddenly  after  they  are  at 
rest,  or  to  relax  them  quickly  when  they  are  contracted.  Both  these 
functions  are  indispensable  to  normal  motion.  This  condition  would 
also  explain  the  inability  of  the  patient  to  arrest  the  body  suddenly 
when  in  locomotion,  a  symptom  known  by  the  names  of  propulsion, 


PAEALYSIS   AGITAI^S  477 

retropulsion  and  lateropulsion.  If  a  person,  who  is  walking,  wants 
to  arrest  this  locomotion  suddenly,  he  must  quickly  contract  the  antag- 
onistic muscles,  must  throw  the  trunk  in  the  opposite  direction,  a  func- 
tion which  an  individual  afflicted  with  paralysis  agitans  is  unable  to 
perform. 

If  now  we  apply  this  condition  of  the  muscular  apparatus  to  the 
muscles  of  the  eye,  we  would  expect  quite  a  number  of  interesting  phe- 
nomena. To  the  tremor  would  apparently  correspond  a  nystagmus. 
But  this  symptom  has  never  been  observed  in  paralysis  agitans.  This 
fact  furnishes  another  proof  for  the  assumption  that  nystagmus  is  pro- 
duced by  some  central  lesion  and  not  by  a  disturbance  in  the  general 
muscular  tonus,  because  if  the  latter  view  were  correct  we  would  be 
bound  to  observe  this  symptom  at  least  occasionally  in  paralysis  agitans. 

The  tension  and  rigidity  do  occur  in  the  ocular  muscles,  although 
far  less  frequently  than  one  would  expect.  The  sphincter  is  never 
affected;  the  pupils  have  always  been  found  normal  in  this  disease. 
Likewise  the  optic  nerve  always  remains  unimpaired.  The  rigidity  is 
very  apt  to  show  itself  in  the  orbicularis,  thus  interfering  with  the 
mimical  motions,  the  expression  of  the  features,  and  therefore  contribu- 
ting to  the  mask-like  appearance  of  the  face.  The  closing  and  opening  of 
the  eye  is  sometimes  very  slow  and  sluggish,  and  in  a  few  cases  there 
was  a  partial  ptosis.  In  order  to  assist  in  the  opening  of  the  eyes,  the 
musculi  frontales  are  contracted,  and  on  account  of  the  difficulty  in 
relaxing  the  muscles,  patients  often  have  marked  wrinkles  in  the  fore- 
head. 

The  movements  of  the  eyeballs  are  sometimes  somewhat  retarded. 
A  few  cases  have  been  observed  where  there  was  great  difficulty  in  con- 
vergence; also  a  spasm  of  accommodation  has  been  described.  In  one 
case  Graefe's  symptom  occurred  on  one  side  in  the  hemilateral  form 
of  the  disease. 

There  is  only  a  single  case  (Minkowski)  on  record  in  which  there 
was  a  complete  ophthalmoplegia  externa.  Both  eyes  were  entirely 
closed  and  could  not  be  opened  by  the  patient.  When  one  raised  the 
lids  with  the  finger,  the  eyeballs  stood  in  a  parallel  axis,  directed  straight 
forward,  without  ability  on  the  part  of  the  patient  to  move  them  in 
any  direction. 


CHAPTER    XII. 

PARASYPHILITIC    AFFECTIONS,     INSANITIES,    AND    TOXIC 
ENCEPHALOPATHIES. 

By  F.  X.   DERCUM,  M.D. 

PARESIS. 

The  ocular  phenomena  of  paresis  consist  in  disturbances  which  may 
involve  all  parts  of  the  visual  apparatus, — namely,  the  muscles,  internal 
and  external,  the  optic  nerve,  and  the  fundus.  These  disturbances  must 
be  studied  in  detail,  but,  in  order  that  we  should  do  so  intelligently,  it 
will  be  necessary,  first,  to  enter  upon  certain  general  considerations. 
Paresis  is  an  affection  which  begins,  as  a  rule,  insidiously  and  extremely 
gradually.  Its  earliest  symptoms  are  usually  those  of  a  purely  func- 
tional disturbance,  and  occasionally  this  disturbance  not  only  precedes 
the  appearance  of  physical  signs,  but  antedates  the  latter  by  a  consider- 
able period  of  time.  During  this  period  the  symptoms  may,  roughly 
speaking,  resemble  those  of  neurasthenia,  and  the  differential  diagnosis 
between  the  two  affections  is  attended  with  considerable  difficulty  and 
may  indeed  be  open  to  serious  doubt.  Paresis,  we  may  here  remark, 
is  readily  divided,  as  regards  its  course,  into  three  stages, — first,  the 
initial  or  prodromal  period ;  second,  the  period  of  the  fully  developed 
disease,  and,  third,  the  period  of  profound  dementia — the  terminal 
period.  During  the  second  or  third  stages  no  difficulty  with  regard  to 
diagnosis  can  possibly  arise  as  each  of  these  stages  is  attended  by  physical 
signs,  both  positive  and  numerous.  It  is  in  the  first  stage  only,  as  just 
stated,  that  difficulty  may  arise.  If,  therefore,  the  existence  of  an  early 
physical  sign  can  be  determined,  a  vast  assistance  is  given  to  the  prob- 
lem. Indeed,  the  appearance  of  such  a  symptom  assumes  a  decisive- 
importance. 

Paresis,  we  should  remember,  is  a  degenerative  disease  attended  by 
a  constantly  increasing  loss  of  function.  One  of  the  cardinal  points 
to  be  borne  in  mind  in  its  early  diagnosis  is  that  the  changes  in  func- 
tion are  quantitative  and  not  merely  qualitative.  In  its  very  begin- 
nings are  these  fundamental  features  to  be  noted.  There  is  a  general 
though  slight  mental  enfeeblement,  an  inability  to  apprehend  readily, 
a  faint  but  unmistakable  loss  of  memory,  slight  lapses  in  conduct,  and 
a  neglect  of  the  proprieties.  There  is  especially  an  evident  inability 
478 


PARESIS  479 

of  the  patient  to  properly  appreciate  his  surroundings ;  his  close  con- 
tact Avitli  his  environment  is  lost,  and  this  is  true  not  only  as  regards 
his  relation  to  matters  of  business  and  to  his  family,  but  also  as  regards 
his  own  illness.  This  blunting  or  obtusion  of  the  mental  faculties  is 
the  early  and  often  faint  expression  of  the  more  profound  dementia 
to  supervene  in  the  future.  In  neurasthenia,  on  the  other  hand,  the 
character  of  the  mental  operations  never  changes ;  but  one  cardinal 
symptom  is  noted, — namely,  the  diminution  in  the  capacity  for  sustained 
mental  effort.  There  are  never,  however,  any  losses  of  function.  There 
is  no  loss  of  memory,  no  blunting  of  judgment,  no  obtusion  of  the  moral 
and  a3sthetic  faculties — none  of  those  countless  changes  in  conduct  and 
habits  to  indicate  a  progressive  psychic  disintegration. 

Xotwithstanding  the  fact  that  such  fundamental  differences  obtain 
in  the  symptomatology  of  neurasthenia  and  of  early  paresis,  it  is  at 
times  a  matter  of  extreme  difficulty  in  practice  to  arrive  at  a  decision, 
so  faint  and  shadowy  may  the  beginning  symptoms  be.  It  is  obviously 
of  vast  importance  to  discover  actual  physical  signs  at  the  earliest 
moment.  Unfortunately,  it  happens  that  at  this  stage  the  pupils  and 
eye-grounds  often  fail  to  yield  us  any  information.  One  sign,  however, 
that  may  be  present  and  one  which  I  personally  regard  as  of  great 
significance,  is  the  existence  of  a  slight  degree  of  amblyopia.  With  or 
without  a  diminution  of  the  color  sense,  it  is.  an  invaluable  symptom. 
It  may  antedate  distinct  and  demonstrable  anomalies  of  the  pupils  or 
changes  in  the  eye-grounds.  Visual  hypaisthesia  is  apparently  in 
keeping  with  the  general  psychic  obtusion  that  is  setting  in,  as  well  as 
with  the  lessening  of  general  cutaneous  sensibility  noted  sooner  or  later. 
It  is  a  sign  whose  importance  is  not  sufficiently  recognized,  nor  is  it  sought 
for  in  individual  cases  as  it  should  be.  The  difficulty  with  regard  to 
light  perception  is  not  always  slight  in  the  beginning,  but  may  amount 
to  an  actual  loss  of  vision,  though  this  early  loss  is  frequently  transient 
in  duration.  Further,  it  is  admitted  by  numerous  writers — Mendel, 
Mickle,  Kraepelin,  Ziehen,  Ballet,  and  Blocq — that  optic  atrophy  may 
be  the  first  noticeable  symptom  of  paresis. 

The  most  common  of  the  ocular  phenomena  of  paresis  are  those 
which  relate  to  the  pupil.  One  of  the  earliest  phenomena  usually  ob- 
served is  that  of  inequality  of  pupils.  Difference  in  the  size  of  the 
pupils,  we  should  remember,  may  occur  physiologically,  but  in  such 
case  this  difference  is  usually  slight.  If,  however,  a  difference  in  the 
size  of  the  pupils  be  noted,  and  this  difference  be  associated  with  a  slug- 
gishness to  light  reaction  or  if  the  pupils,  being  equal,  react  slightly 
differently  to  light,  the  finding  Is  suggestive  with  regard  to  paresis  in 


480  THE    EYE    AND    NERVOUS    SYSTEM 

its  incipiency.  In  the  early  stages  of  paresis,  indeed,  a  difference  in 
the  promptness  of  the  response  to  light  reaction  between  the  right  and 
left  pupils  is  almost  the  rule.  Likewise  an  impairment  of  the  consen- 
sual light  reaction  may  be  the  first  symptom  of  a  pupillary  disorder 
in  paresis,  and  it  is  of  the  same  value  as  a  difference  of  reaction  of  the 
two  pupils. 

The  above  facts  are  established  by  numerous  observations  and  admit 
of  no  question.  How  valuable  slight  changes  of  the  pupils  are  in  enabling 
us  to  make  a  diagnosis  is  revealed  by  a  case  reported  by  Lucas  in  which 
a  diagnosis  of  melancholia  had  been  made,  but  in  which  Joffroy  after- 
wards noted  inequality  of  the  pupils  and  suspected  a  paresis,  which  later 
matured. 

Narrowing  or  small  size  of  the  pupils,  even  if  slight,  taken  together 
with  other  symptoms,  is  very  significant.  Distinct  myosis  is,  of  course, 
a  symptom  of  unquestioned  value.  Exceptionally,  it  occurs  in  the 
very  early  stages  of  paresis.  Changes  in  the  fundus  of  the  eye  may 
also  be  noted  early,  though  such  changes  are  exceptional.  However, 
they  have  been  noted  early  by  Walker  and  more  especially  by  Knapp. 
Knapp,  indeed,  has  reported  three  cases  of  paresis  in  which  optic 
atrophy  preceded  the  mental  symptoms.  Abolition  of  the  light  reflex 
with  preservation  of  accommodation — the  Argyll-Robertson  pupil — 
may  also  infrequently  be  observed  in  the  early  stage  of  paresis,  though 
this  is  quite  unusual.  Commonly,  sluggishness  of  the  light  reaction 
alone  is  noted  in  the  early  stage. 

The  mental  changes  of  paresis  may  long  antedate  the  appearance 
of  physical  signs.  However,  this  is  not  by  any  means  the  rule.  Physi- 
cal signs  may  indeed  make  their  appearance  either  simultaneously  with 
the  mental  changes  or  may  sometimes  even  precede  the  latter.  Notably 
is  this  the  case  with  the  visual  phenomena.  In  other  words,  it  is  un- 
usual, in  practice,  to  meet  with  early  cases  of  paresis  in  which  some 
ocular  changes,  even  though  slight,  cannot  be  found. 

The  relative  frequency  of  ocular  phenomena  in  the  early  part  of 
the  disease  may  be  gathered  from  the  statistics  of  Joffroy.  Joffroy 
examined  two  hundred  and  twenty-seven  cases  of  paresis,  of  which  the 
majority  were  in  the  first  period  of  the  disease.  Two  hundred  and 
twelve  of  these  cases  presented  ocular  symptoms.  Joffroy  believes  that 
among  the  fifteen  who  did  not  have  ocular  symptoms  a  later  examina- 
tion, at  a  more  advanced  stage,  would  certainly  have  revealed  them. 

If  a  systematic  study  be  made  of  the  eyes  in  paresis  in  the  stage 
of  the  well  established  disease,  the  following  phenomena  are  noted. 
First,  there  is  very  frequently  observed  an  inequality  of  the  pupils. 


PARESIS  481 

Some  idea  of  the  relative  frequency  of  this  symptom  can  be  gained 
by  the  following  statistics  which  have  been  taken  in  part  from  the 
groupings  of  Mignot  and  of  Bumke.  Siemerling  noted  inequality  in 
26  per  cent,  of  the  cases  examined  by  him,  Lasegue  33  per  cent,  Fran- 
cotte  51  per  cent.,  Moreau  58  per  cent.,  Mendel  60  per  cent.,  Mobeche 
61  per  cent,  Kaes  62.7  per  cent,  Marie  63  per  cent,  Oebecke  64  per 
cent.,  Renaud  65  per  cent.,  Seifert  QQ  per  cent,  Boy  QQ  per  cent. 
Regis  66  per  cent.,  Doutrebente  77  per  cent,  and  Vincent  80  per  cent. 
Mignot  found  64  per  cent. ;  Cololian,  who  studied  fifty-seven  cases, 
found  unequal  pupils  in  almost  all  of  them.  Dawson  found  92.3  per 
cent,  in  the  forty  cases  studied  by  him.  It  was  the  most  frequent  of 
all  the  ocular  phenomena  in  his  cases.  The  right  pupil  was  the  larger 
in  twenty-five,  the  left  in  eleven.  Kornfeld  and  Bikeles  found  unequal 
pupils  in  70  per  cent,  Raehlmann  in  60  per  cent.,  Gudden  in  57.5  per 
cent,  Wallenberg  in  53  per  cent.,  A.  Westphal  in  45  per  cent.,  Hillenberg 
in  45  per  cent,  and  Siemerling  in  27.5  per  cent.  Joffroy  found  inequal- 
ity of  the  pupils  in  one  hundred  and  forty-four  of  his  two  hundred  and 
twenty-seven  cases — that  is,  63.4  per  cent.  If  to  these  there  are  added 
his  cases  of  extreme  double  mydriasis  and  extreme  double  myosis,  he  ob- 
tains 87  per  cent,  of  cases  of  modifications  of  the  dimensions  of  the  iris. 

Variations  in  the  frequency  with  which  unequal  pupils  are  observed 
is,  in  part,  to  be  attributed  to  the  stages  at  which  the  cases  have  been 
examined  and  to  a  less  degree  to  the  element  of  personal  equation :  some 
observers  noting  minute  differences  and  others  only  inequalities  that 
are  striking.  Notwithstanding  the  divergence  in  the  percentage  ob- 
tained by  different  observers,  it  cannot  but  be  admitted  that  inequality 
of  pupils  is  an  exceedingly  common  symptom  of  paresis.  However,  of 
itself  inequality  of  the  pupils  has  no  more  than  a  confirmatory  value 
in  the  diagnosis.  As  is  well  known,  it  may  occur  in  gross  organic  dis- 
ease, brain  syphilis,  and  the  insanities ;  indeed,  it  may  in  moderate 
degree  be  occasionally  noted  in  perfectly  normal  individuals.  It  is 
important  to  add  that  the  degree  of  inequality  in  paresis  may  vary 
decidedly  in  a  given  case,  and  sometimes  during  a  comparatively  short 
period  of  observation.     Rapid  fluctuations  are  by  no  means  infrequent 

The  pupil  in  paresis  is  not  infrequently  irregular  in  shape, — that 
is,  it  may  be  slightly  oval  or  ovoid  or  its  circumference  may  be  made 
irregular  by  various  modifications  of  shape.  These  modifications  do 
not,  however,  lead  to  gross  deformities;  notwithstanding,  they  can  be 
very  readily  observed.  They  are  undoubtedly  present  in  a  percentage 
of  cases.  Kaes  found  them  in  3.1  per  cent.,  Mobeche  in  61  per  cent., 
and  Mignot  in  18  per  cent.  Dawson  noted  irregularity  in  outline  in 
31 


482  THE    EYE    AND    NERVOUS    SYSTEM 

one  or  both  pupils  in  thirty-seven  out  of  thirty-nine  cases  (94.8  per 
cent.).  Joffroy  noted  in  one  hundred  and  twenty-five  cases,  in  which 
the  symptom  was  studied,  deformity  of  both  pupils  in  ninety-three, — 
that  is,  75.2  per  cent.  In  eight  cases  one  pupil  only  was  deformed. 
Mignot  observed  deformities  in  fifteen  cases  out  of  twenty-two.  In 
seven  of  his  cases  Mignot  observed  the  gradual  diminution  and  final 
loss  of  the  pupillary  reflexes.  Joffroy  observed  irregular  pupils  in  two 
cases  in  which  the  symptom  was  not  accompanied  by  impairment  of 
the  light  reflex.  However,  in  these  two  cases  he  later  observed,  first, 
paresis  and  then  paralysis  of  the  light  reflex.  Beginning  deformity 
or  irregularity  of  the  pupil  is  to  be  regarded  as  a  precursor  of  the 
Argyll-Robertson  sign.  In  one  case  inequality  made  its  appearance 
while  the  patient  was  under  observation  at  the  hospital.  In  another, 
pupillary  disturbances  made  their  appearance  fifteen  days  after 
admission. 

Myosis  is  also  a  symptom  very  frequently  observed  in  the  well- 
established  period  of  the  disease.  The  opposite  condition,  mydriasis, 
likewise  occurs,  and  would  appear  from  the  following  figures  to  be 
decidedly  less  frequent  than  myosis. 

Kaes  found  myosis  in  7.2  per  cent,  Mobeche  in  12  per  cent.,  Doutre- 
bente  in  12  per  cent.,  Moreau  in  18  per  cent,  Vincent  in  38  per  cent, 
and  Marie  in  39  per  cent.  Mignot  found,  out  of  twenty-two  cases, 
myosis  in  six.  Dawson  found  myosis  on  both  sides  in  six  cases  out 
of  twenty-three ;  in  three  cases  he  noted  myosis  in  only  one  eye. 

As  regards  mydriasis,  Kaes  found  it  in  3.6  per  cent,  Doutrebente 
in  4  per  cent,  Vincent  in  14  per  cent,  Mobeche  in  15  per  cent,  Moreau 
in  26  per  cent,  and  Marie  in  44  per  cent.  Out  of  twenty-two  cases 
Mignot  found  mydriasis  in  only  one.  Out  of  twenty-three  cases  Daw- 
son found  mydriasis  in  eight.  In  one  case  he  observed  mydriasis  of 
the  right  eye  and  myosis  of  the  left  Mydriasis  is  a  paralytic  phe- 
nomenon which  Fournier  regards  rather  as  a  symptom  of  syphilis  than 
of  paresis.  That  it  should,  at  times  at  least,  be  regarded  as  an  expres- 
sion of  ojjhthalmoplegia  interna  and  indicative  of  nervous  syphilis 
rather  than  of  paresis  seems  plausible,  but  involvement  of  the  intrinsic 
muscles  of  the  eyes  is  so  frequent  in  cases  of  undoubted  paresis  that 
this  explanation  can  apply  only  exceptionally — perhaps  in  cases  in 
which  true  syphilitic  processes  are  going  on  at  the  same  time  as  the 
degenerative  changes  of  paresis. 

Next  most  important  are  changes  in  the  light  reflex.  This  is  im- 
paired or  lost  upon  one  side  or  both  in  a  very  large  number  of  cases. 
In  statistical  studies  of  the  loss  of  the  reflex,  different  observers  have 


PARESIS  483 

likewise  arrived  at  different  percentages  of  results,  as  the  follo\ving 
figures  (grouped  by  Mignot)  will  show:  Oebecke  26  per  cent.,  Thom- 
sen  47  per  cent.,  Mendel  47  per  cent.,  Siemerling  54  per  cent.,  Magnan 
and  Serieux  60  per  cent.,  Moeli  61  per  cent.,  Siemerling  (second  sta- 
tistic study)  64  per  cent.,  Francotte  88  per  cent.,  Siemerling  (third 
statistic  study)  68  per  cent.,  and  Vincent  90  per  cent.  To  these  should 
be  added  Dillenberg  47.5  per  cent,  Wollenberg  50  per  cent.,  A.  West- 
phal  50  per  cent,  Gudden  41.2  per  cent.,  Kornfeld  and  Bikeles  62 
per  cent.,  and  Hirschl  50  per  cent.  Joffroy  noted  loss  or  impairment 
of  the  light  reflex  in  one  hundred  and  seventy-one  cases  out  of  the  two 
hundred  and  twenty-seven  cases  studied  by  him, — that  is,  in  75.3  per 
cent.  It  was  abolished  on  both  sides  in  one  hundred  and  three  cases, 
abolished  upon  one  side  and  diminished  upon  the  other  in  fourteen 
cases,  diminished  upon  both  sides  in  thirty-five  cases,  abolished  upon 
one  side  and  normal  upon  the  other  in  nine  cases,  and  diminished  upon 
one  side  and  normal  upon  the  other  in  ten  cases. 

M.  de  Montyel  has  made  an  exhaustive  analysis  of  the  light  reflex. 
He  found  it  normal  in  30.8  per  cent.,  abnormal  in  69.2  per  cent.  Exag- 
geration was  present  in  5.3  per  cent.,  diminution  in  43.1  per  cent, 
loss  in  47.6  per  cent.  Analyzing  his  findings  with  regard  to  the  period 
of  the  disease,  he  found  the  reflex  exaggerated  in  17.4  per  cent,  during 
the  first  period,  and  2.3  per  cent  in  the  second  period,  and  never 
during  the  third  period.  He  found  it  diminished  in  44.6  per  cent,  in 
the  first  period,  47.2  per  cent,  in  the  second  period,  and  34  per  cent 
in  the  third  period.  He  found  it  lost  in  32.5  per  cent,  in  the  first 
period,  45  per  cent,  in  the  second  period,  and  66  per  cent,  in  the  third 
period.  He  found  it  normal  upon  one  side  and  lost  on  the  other  in 
5.5  per  cent,  in  the  first  period,  5.5  per  cent  in  the  second,  and  never 
in  the  third  period.  Analyzing  his  results  with  regard  to  the  form  of 
paresis,  he  found  it  abnormal  in  66.1  per  cent,  in  the  expansive  form, 
in  100  per  cent,  in  the  depressive  form,  in  45.4  per  cent,  in  the  mixed 
form,  in  80.6  per  cent,  in  the  simple  demented  forms,  and  in  25  per 
cent,  of  cases  with  remission. 

Dividing  his  cases  into  those  which  were  agitated  and  those  which 
were  quiet,  he  found  it  abnormal  in  69.2  per  cent,  in  the  agitated  forms 
and  75.8  per  cent,  in  the  quiet  forms.  Analyzing  his  results  again  as 
to  ages,  he  found  the  light  reflex  abnormal  in  93.8  per  cent,  in  the 
ages  from  twenty  to  thirty,  in  63.9  per  cent,  in  the  ages  from  thirty 
to  forty,  in  67.2  per  cent  in  the  ages  from  forty  to  fifty,  and  70  per 
cent,  in  the  ages  of  fifty  to  sixty.  De  Montyel's  conclusions  from  these 
studies  were  as  follows:    First,  the  light  reflex  was  more  frequently 


484  THE    EYE    AND    NERVOUS    SYSTEM 

abnormal  than  normal.  Second,  exaggeration  of  the  reflex  was  excep- 
tional. Third,  there  was  only  a  slight  difference  in  the  frequency  of 
the  occurrence  of  the  diminished  and  the  lost  reflex.  Fourth,  the 
changes  in  the  reflex  were  identical  on  the  two  sides ;  only  exceptionally 
was  the  reflex  lost  upon  one  side  and  normal  upon  the  other.  Fifth, 
in  the  first  period  the  normal  reflex  was  more  frequent  than  the  abnor- 
mal, while  the  reflex  is  more  frequently  abnormal  in  the  second  and 
third  periods.  Sixth,  exaggeration  and  unequal  changes  on  the  two 
sides  were  always  present  in  the  initial  stages  of  the  disease;  the 
frequency  of  loss  was  proportionate  to  the  progress  of  the  disease. 
Diminution  was  more  frequent  than  loss  during  the  first  period;  the 
proportion  was  about  equal  in  the  second  and  reversed  in  the  third 
period.  Seventh,  the  reflex  was  abnormal  in  one-fourth  of  the  remis- 
sions. Eighth,  exaggeration  was  seen  only  in  the  expansive  and  mixed 
forms.  Loss  of  the  reflex  prevailed  over  diminution  in  the  demented 
form;  only  in  the  demented  form  were  both  eyes  unequally  affected. 
Ninth,  an  abnormal  reflex  was  always  noted  in  the  traumatic  cases. 
Tenth,  at  all  ages,  the  abnormal  reflex  was  more  frequent  than  the 
normal ;  however,  abnormality  of  the  light  reflex  was  especially  noted 
in  the  extreme  ages,  especially  in  the  younger  cases.  Eleventh,  in  the 
first  and  second  periods  the  reflex  was  more  affected  when  the  motor 
symptoms  were  more  pronounced.     (See  Chapter  VI,  p.  308). 

The  consensual  light  reflex  should  also  be  studied.  Its  importance 
is  not  fully  appreciated.  Dawson  found  absence  of  the  consensual  light 
reflex  in  one  or  both  eyes  in  twenty-seven  out  of  forty  cases  (67.5  per 
cent.).  Further,  it  was  involved  without  impairment  of  the  direct 
light  reflex  in  eleven  cases,  though  in  only  two  of  these  cases  was  it 
actually  absent.  Impairment  or  loss  of  the  consensual  light  reflex  must 
be  regarded  as  of  great  significance  whenever  met  with.  Dawson  con- 
cludes that  it  is  more  often  affected  than  the  direct  light  reflex. 

Next,  it  is  of  importance  to  study  the  reaction  to  accommodation. 
This  is  preserved,  as  in  tabes,  long  after  the  light  reflex  is  lost.  How- 
ever, it  is  sooner  or  later  involved.  The  more  advanced  the  case,  other 
things  equal,  the  more  likely  is  loss  or  impairment  of  accommodation 
to  be  present,  Mignot,  in  twenty-two  cases,  found  accommodation  to 
be  normal  in  eleven,  lost  in  five,  and  diminished  in  six.  De  Montyel 
made  an  elaborate  study  of  the  reaction  in  thirty  cases.  These  cases  were 
followed  from  the  outset  to  the  termination  of  the  disease,  the  reflexes 
being  examined,  in  all,  some  six  hundred  and  eighty  times.  It  was 
found  normal  in  two  hundred  and  eighty-one  examinations,  abnormal 
in  three  hundred  and  ninety-nine.    In  fifteen  examinations,  diminution 


PAEESIS  485 

was  present  in  one  hundred  and  sixty-eight  and  loss  in  two  hundred 
and  ten.  It  was  normal  on  one  side  and  abolished  on  the  other  in  six 
examinations. 

In  almost  all  the  cases  changes  were  present  in  both  eyes  simul- 
taneously. Only  in  1,5  per  cent,  was  the  reflex  normal  upon  one  side 
and  lost  upon  the  other.  The  majority  of  cases  presented  a  diminution 
of  the  reflex.  Wlien  exaggeration  was  present  it  was  always  moderate. 
Only  six  times  did  the  reflex  show  changes  in  one  eye.  Six  times 
the  loss  of  of  the  reflex  of  one  eye  coincided  with  abnormality  of  the 
other.  Analyzing  his  results  as  to  the  various  periods  of  the  disease, 
de  Montyel  found  that  in  the  first  j)eriod  the  accommodation  reflex  was 
normal  in  63.2  per  cent.,  abnormal  in  36.7  per  cent.  During  the  second 
period  these  figures  were  reversed,  for  now  he  found  them  normal  in 
only  33.7  per  cent,  and  abnormal  in  66.3  per  cent.  In  the  third  period 
a  still  greater  reversal  of  percentage  took  place.  It  was  normal  in  16.7 
per  cent,  and  abnormal  in  83.2  per  cent.  He  found  it  exaggerated  in 
the  first  period  15.3  per  cent.  He  at  no  time  found  it  exaggerated  in 
the  second  or  third  periods.  He  found  it  diminished  in  64.2  per  cent, 
in  the  first  period,  in  41.9  per  cent,  in  the  second  period,  and  26.1  per 
cent,  in  the  third  period.  He  found  it  lost  in  20.4  per  cent,  in  the  first 
period,  54.4  per  cent,  in  the  second  period,  73.9  per  cent,  in  the  third 
period.  In  3.5  per  cent,  he  found  it  normal  upon  one  side  and  abolished 
on  the  other  in  the  second  period  of  the  disease.  Analyzing  those  cases 
in  which  the  reflex  was  found  to  be  merely  diminished  and  not  lost,  he 
found  that  it  was  somewhat  diminished  in  76.2  per  cent,  and  very 
much  diminished  in  23.8  per  cent,  in  the  first  period.  During  the 
second  period  he  found  that  it  was  diminished  in  88.6  per  cent,  and 
much  diminished  in  11.4  per  cent.  In  the  third  period  he  found  it 
diminished  in  82.9  per  cent,  and  much  diminished  in  17.1  per  cent. 
Analyzing  his  results  according  to  the  depressive,  the  expansive,  or  the 
mixed  forms,  or  forms  with  the  remissions,  he  found  the  reflex  normal 
in  53  per  cent,  and  abnormal  in  46.9  per  cent,  in  the  expansive  form, 
normal  in  51.7  per  cent,  and  abnormal  in  48.3  per  cent,  in  the  de- 
pressive form,  normal  in  53  per  cent,  and  abnormal  in  46  per  cent,  in 
the  mixed  form,  and  in  the  demented  form  normal  in  25.3  per  cent, 
and  abnormal  in  74.7  per  cent.  ^Vllile  in  the  cases  with  remissions 
he  found  this  reflex  normal  in  62.5  per  cent  and  abnormal  in  37.5 
per  cent.  He  also  found  that  it  was  exaggerated  in  15.2  per  cent,  in 
the  expansive  form  and  never  exaggerated  in  the  depressive,  mixed, 
simple  demented  forms,  and  forms  with  remissions.  It  was  diminished 
in  42.4  per  cent,  in  the  expansive  form,  in  100  per  cent,  in  the  depres- 


486  THE    EYE    AND    NERVOUS    SYSTEM 

sive  form,  in  41  per  cent  in  the  mixed  form,  and  33.3  per  cent  in 
the  simple  demented  form,  and  in  100  per  cent,  in  the  forms  with 
remissions.  It  was  lost  in  42.4  per  cent,  in  the  expansive  form,  never 
entirely  lost  in  the  depressive  form,  lost  in  58.8  per  cent,  in  the  mixed 
form,  in  64  per  cent,  in  the  demented  form,  and  never  entirely  lost 
in  the  form  with  remissions.  It  was  normal  upon  one  side  and  abolished 
on  the  other  in  no  case  of  the  expansive,  the  depressive,  or  the  mixed 
form.  This,  however,  occurred  in  2.5  per  cent,  in  the  simple  demented 
form,  and  in  none  of  tlie  cases  with  remissions. 

Analyzing  his  findings  as  to  age,  de  Montyel  found  that  the  reflex 
was  abnormal  in  88.9  per  cent  in  the  ages  from  twenty  to  thirty,  in 
57  per  cent,  in  the  ages  from  thirty  to  forty,  in  21  per  cent,  in  the 
ages  from  forty  to  fifty,  in  82.9  per  cent,  in  the  ages  from  fifty  to 
sixty.  His  conclusions  are  as  follows:  First,  that  the  accommodation 
reflex  is  more  frequently  normal  than  abnormal;  second,  tliat  the 
exaggeration  of  the  reflex  is  exceptional;  third,  that  loss  is  more  fre- 
quent than  diminution ;  fourth,  in  one-half  per  cent,  it  is  abnormal  upon 
one  side  and  normal  on  the  other;  fifth,  exaggeration  or  diminution 
are  usually  moderate ;  sixth,  only  during  the  first  period  of  the  disease 
is  the  reflex  more  frequently  normal  than  abnormal,  and  especially 
is  this  the  case  in  the  third  stage;  seventh,  exaggeration  and  unequal 
changes  on  both  sides  were  noticed  exclusively  in  the  initial  stages, 
while  the  loss  of  the  reflex  was  in  direct  proportion  and  diminution 
of  the  reflex  in  indirect  proportion  to  the  progress  of  the  disease ;  eighth, 
an  abnormal  reflex  is  noticed  in  more  than  one-third  of  the  cases  in 
the  period  during  the  remission,  and  it  was  only  in  the  demented  form 
that  the  abnormality  of  the  reflex  prevailed  over  the  normal  reflex.  In 
all  of  the  other  varieties — expansive,  depressive,  and  mixed — the  nor- 
mal was  more  frequent  than  the  abnormal ;  ninth,  the  reflex  was  more 
frequently  and  more  profoundly  changed  in  the  period  of  excitement; 
tenth,  changes  in  the  reflex  were  most  frequently  noted  in  the  extreme 
ages — before  thirty  and  after  fifty ;  eleven,  exaggeration  of  the  reflex 
was  present,  while  the  motor  disturbances  were  slight  During  the 
first  and  second  periods  of  the  disease  the  reflex  was  the  more  pro- 
foundly changed  the  more  the  motor  disturbances  were  marked. 

Changes  in  the  eye-ground,  with  progressive  amblyopia  and  amau- 
rosis, may  be  noted  in  paresis.  However,  as  compared  with  tabes, 
such  changes  are  relatively  infrequent  Perhaps,  as  Klippel  points 
out,  this  difference  is  due  to  the  fact  that  in  tabes  the  peripheral  sen- 
sory neurone  is  primarily  attacked,  while  in  paresis  the  peripheral 
sensory  neurone  is  but  rarely  and  only  indirectly  attacked, — that  is,  by 


PAEESIS  487 

the  propagation  of  degeneration  from  neurone  to  neurone.  The  change 
in  the  optic  nerve,  according  to  Keravel  and  Raviart,  is  more  insular 
in  tabes  and  more  annular  in  paresis.  However,  lesions  of  the  fundus 
occur  in  paresis  earlier  and  more  frequently  than  the  older  observers 
thought.  That  they  sometimes  occur  at  a  very  early  period  is  proven 
by  the  three  cases  placed  on  record  by  Knapp.  Thus,  a  man  of  thirty- 
seven  presented  a  history  of  sudden  loss  of  vision  in  1889.  This  loss 
proved  temporary,  and  vision  soon  returned.  Five  months  later,  how- 
ever, failure  of  vision  set  in,  was  progressive,  and  finally  became  com- 
plete. Optic  atrophy  was  present  in  both  eyes.  In  another  case,  a  man 
of  thirty-six,  there  was  a  gradual  loss  of  vision  with  gray  atrophy  of 
both  nerves.  In  a  third  case,  a  man  of  forty-five  presented  marked 
impairment  of  vision  of  both  eyes,  while  both  discs  were  bluish-gray. 
In  all  of  these  cases  the  eye-ground  changes,  according  to  the  author, 
preceded  the  paresis. 

That  fundus  lesions  occur  more  frequently  than  was  formerly  be- 
lieved is  shown  by  the  studies  of  Keraval.  The  latter  has  made  a  study 
of  the  fundus  in  paresis  and  in  its  initial  lesions  in  fifty-one  cases.  He 
found  that  forty-two  cases  presented  lesions  of  the  fundi.  In  seven 
patients  in  an  advanced  stage  of  the  disease  there  were  five  with  white 
atrophy,  one  with  gray  atrophy,  and  one  with  sclerochoroiditis.  In 
thirteen  cases  the  papilla  was  pale.  In  twenty-two  there  was  involve- 
ment only  of  segments  of  the  papilla?  (preliminary  stage  of  optic 
atrophy).  In  nine  cases  in  the  period  of  remission  there  were  no  fun- 
dus lesions.  Raviart  made  a  study  of  the  fundus  in  forty-four  cases 
of  paresis.  In  one  there  was  bilateral  white  optic  atrophy.  In  ten 
both  papilla?  were  pale;  in  two  a  white  papilla  on  the  right  side;  in 
four  there  were  gray  papilla? ;  in  five  the  papilla?  were  pale  (or  washed 
•out)  ;  in  six  only  were  the  fundi  normal.  Changes  occurred  therefore 
in  73.33  per  cent.  The  majority  of  the  cases  in  which  these  changes 
were  observed  were  in  the  second  stage  of  the  disease.  Visual  acuity 
was  slightly  diminished  in  the  majority  of  cases. 

Individual  statistics  differ  considerably  regarding  the  frequency  of 
optic  atrophy.  Thus,  in  contrast  to  the  above  results  are  the  findings  of 
Hans  Gudden.  The  latter  found  primary  optic  atrophy  only  sixty-five 
times  in  one  thousand  three  hundred  and  twenty-six  cases  of  paresis. 
Morselli  again,  on  the  other  hand,  thinks  that  optic  atrophy  is  very 
€ommon  in  paresis.  Clifford  Allbutt  also  found  forty-one  cases  of 
atrophy  in  fifty-three  of  paresis,  while  Tebaldi  found  the  fundus  healthy 
in  only  one  case  out  of  twenty.  Boy  found  optic  atrophy  in  ten  per 
•cent.,  Mendel  in  five  per  cent.,  Joffroy  in  twelve  per  cent.   Optic  atrophy 


488  THE    EYE    AND    NERVOUS    SYSTEM 

sufficiently  marked  to  cause  blindness  is  relatively  rare  in  paresis. 
Varying  degrees  of  amblyopia  are  more  common.  Finally,  optic 
atrophy,  as  we  have  already  seen  (page  480),  may  be  the  first  physical 
sign  of  paresis. 

Disturbances  of  the  visual  fields  may  occur  in  paresis,  but  they  are 
not  common.  This  is  especially  true  of  marked  disturbances.  Con- 
tracture, however,  may  be  noted,  as,  for  instance,  in  a  case  reported  by 
Deutsch  of  concentric  narrowing  of  the  fields.  More  rarely  gross  de- 
formities are  observed,  as  in  a  case  of  Storch  in  which,  after  an  epilepti- 
form seizure,  a  left  hemianopsia  was  noted  which  persisted  for  a  year. 

Among  the  physical  signs  of  paresis  are  various  palsies  of  the 
cranial  nerves,  more  especially  of  the  third,  fourth,  and  the  sixth  pair. 
These  may  give  rise  variously  to  strabismus,  diplopia  and  ptosis.  They 
may  make  their  appearance  at  the  very  beginning  of  paresis,  or  may 
occur  during  the  period  of  the  fully  developed  disease.  Usually  these 
palsies  are  not  very  pronounced  or  accentuated.  Slight  degrees  of 
ptosis,  weakness  of  adduction  or  abduction  may  be  noted,  though  as  a 
rule  careful  study  is  required  to  detect  these  anomalies.  In  this  respect 
they  differ  markedly  from  those  observed  in  tabes  and  syphilis.  Quite 
commonly  also  they  are  transitory  and  fugacious.  Dawson,  in  forty 
cases,  noted  palsies  of  external  ocular  muscles  in  only  five ;  Raekes  in 
18.2  per  cent. ;  Joffroy  in  16.7  per  cent.  Out  of  his  two  hundred  and 
twenty-seven  cases,  Joffroy  noted  ocular  palsies  thirty-eight  times.  In 
twelve  cases  a  simple  ptosis  was  noted ;  fourteen  times  paralysis  or  pare- 
sis of  muscles  supplied  by  the  third  nerve  and  five  times  of  the  muscle 
supplied  by  the  fourth  nerve,  five  times  nystagmus,  and  twice  spasmodic 
contraction  of  the  orbicularis  were  noted.  On  the  whole,  palsies  and 
other  motor  disturbances  are  quite  secondary  in  importance  to  the 
other  ocular  phenomena  of  paresis;  are  relatively  infrequent  and  in- 
constant. At  times  slight  atactic  movements  and  rarely  a  well  marked 
nystagmus  is  noted,  as  witness  the  cases  reported  by  Deutsch,  Hepburn, 
Ballet,  and  others.  These  movements  are  not  to  be  regarded  as  truly 
nystagmic,  but  merely  as  a  local  incoordination  comparable  to  the 
ataxia  and  uncertainty  of  movement  noted  in  the  limbs. 

SUMMARY. 

Among  the  earliest  signs  of  paresis  are  inequality  and  irregularity  of 
the  pupil,  and  at  times  impairment  of  the  visual  acuity  and  even 
atrophic  changes  in  the  optic  nerve.  We  observe  also  that  the  involve- 
ment of  the  visual  apparatus  is  always  gradual  and  progressive.  Fur- 
ther we  note  that  it  is  always  asymmetrical,  or  nearly  so. 


PARESIS  489 

In  keeping  with  this  fact  of  asymmetry,  we  find,  as  the  most  frequent 
ocular  symptom  in  paresis,  inequality  of  the  pupils.  Inasmuch,  how- 
ever, as  the  inequality  of  the  pupils  is  very  frequent  in  affections  other 
than  paresis,  it  is  obvious  that  the  association  of  other  phenomena  is 
necessary  to  give  to  this  symptom  a  specific  value.  Of  itself  inequality 
cannot  even  serve  to  differentiate  between  an  organic  and  a  functional 
disease.  As  is  well  known,  it  may  occur,  on  the  one  hand,  in  tabes, 
paresis,  syphilis,  or  other  gross  organic  disease ;  or,  on  the  other,  in 
purely  functional  conditions,  such  as  hysteria  and  the  psychoses.  It  is, 
therefore,  necessary  to  search  for  other  signs  such  as  impairment  or 
loss  of  the  light  reaction  and  anomalies  of  accommodation  and  con- 
vergence. In  paresis  such  additional  symptoms  sooner  or  later  make 
their  appearance. 

Irregularity  or  deformity  of  the  pupils  is  almost  as  common  as 
inequality.  Myosis,  on  the  other  hand,  is  less  frequent  though  it  occurs, 
roughly  speaking,  in  about  one-third  of  the  cases. 

The  usual  sequence  of  the  phenomena  observed  is  about  as  follows: 
at  first  inequality,  irregularity,  and  impairment  or  loss  of  the  light 
reflex,  consensual  or  direct,  upon  one  or  both  sides,  with  at  the  same 
time  preservation  of  and  persistence  for  a  time  of  the  reaction  to  ac- 
commodation ;  later  only  do  we  find  impairment  and  finally  loss  of  the 
reaction  to  accommodation,  the  pupil  being  then  inert  to  all  forms  of 
excitation.  The  Argyll-Robertson  pupil  is,  therefore,  present  during 
a  part  of  the  course  of  paresis.  However,  we  should  bear  in  mind  that 
while  this  is  so  in  many  cases,  in  others  the  loss  of  the  light  reflex  may 
be  somewhat  retarded  and  the  loss  of  accommodation  may,  on  the  other 
hand,  occur  relatively  early,  so  that  the  duration  of  a  typical  Argyll- 
Robertson  pupil  in  a  given  case  may  be  short.  In  this  respect  paresis 
presents  a  marked  contrast  to  tabes,  in  which  the  Argyll-Robertson 
pupil  is  a  prominent  and  characteristic  feature  during  almost  the 
entire  course  of  the  disease.  Observers  are  not  wanting  who  find  the 
Argyll-Robertson  pupil  present  in  but  a  small  number  of  paretics. 
Thus,  Dawson  out  of  forty  cases  found  it  present  upon  both  sides  in 
only  five  cases,  and  on  one  side  in  only  three  cases.  Intermediate  con- 
ditions also  are  found  in  which  the  light  reflex  is  not  wholly  lost,  but 
in  which  at  the  same  time  the  reaction  to  accommodation  is  reduced. 
Dawson  found  this  to  be  the  case  in  30.76  per  cent.  Sluggish  or  slow 
reaction  of  the  pupil  to  light  with  diminished  movement  is  not  in- 
frequently observed.  It  appears  to  constitute  a  period  between  the 
normal  pupil  and  the  typical  Argyll-Robertson  pupil.  Similarly,  the 
accommodation  reaction  is  not  infrequently  found  to  be  sluggish  and 


490  THE    P:YE    AND    NERVOUS    SYSTEM 

slow  instead  of  being  lost.  Its  occurrence  means  the  beginning  of  a 
total  iridoplegia.  Finally  we  should  remember  that  inequality,  irregu- 
larity, deformity,  myosis,  and  mydriasis  are  either  forerunners  of  or  are 
closely  associated  with  anomalies  of  pupillary  action. 

The  pupillary  changes  of  paresis  are,  it  need  hardly  be  stated,  not 
peculiar  to  paresis.  They  are  met  with  in  varying  degrees  in  both 
syphilis  and  tabes,  and  some  of  them,  such  as  inequality,  are  also  found 
in  purely  functional  nervous  and  mental  affections,  as  already  stated. 
Not  only  are  all  of  the  pupillary  disturbances  of  paresis  observed  in 
tabes,  but  Babinski  and  Charpentier  have  shown  that  the  Argyll-Robert- 
son sign  is  very  frequently  met  with  in  old  cases  of  syphilis,  irrespec- 
tive of  signs  suggestive  either  of  tabes  or  paresis.  Whether  the  appear- 
ance of  such  symptoms  justifies  a  prognosis  of  tabes  or  paresis  is  still 
an  open  question.  That  it  does  so,  with  or  without  other  symptoms  and 
in  spite  of  the  presence  of  the  active  signs  of  true  nervous  syphilis,  is  an 
opinion  to  which  the  author  strongly  inclines. 

Finally  it  should  be  borne  in  mind  that  some  of  the  pupillary  phe- 
nomena observed  in  paresis — e.g.,  inequality  and  irregularity — may 
vary  considerably  and  may  ev^en  be  intermittent ;  especially  may  this 
be  noted  in  the  early  stages.  The  light  reflex  also  may  in  rare  instances 
vary ;  for  example,  if  greatly  impaired  though  not  entirely  lost,  it  maj' 
be  re-established  during  a  remission  as  in  the  case  observed  by  Bumke. 
It  is  even  asserted  that  in  very  rare  cases  (Fuerstner,  Gaup)  the  reflex 
to  light  may  be  again  established  after  having  been  entirely  lost.  On 
the  whole,  all  of  the  ocular  phenomena  of  paresis  are  less  fixed  and 
definite  than  they  are  in  tabes.  Again,  irregularity  of  the  pupil,  so 
frequently  an  early  sign,  is  to  be  looked  upon  as  the  beginning  of  the 
progressive-iridoplegia  of  which  the  Argj^'ll-Robertson  is  a  more  ad- 
vanced stage  and  the  inert  pupils  of  the  terminal  period  the  final 
stage. 

Eyes  which  are  normal,  or  at  any  rate  in  which  no  symptom  can  be 
detected,  are  also  met  with  in  paresis,  but  only  in  the  early  stages  of  the 
affection.  Thus,  in  two  cases  reported  by  James,  the  pupils  were  equal 
and  reacted  properly  to  light  and  accommodation.  In  a  third  case, 
however,  while  the  pupils  reacted  normally,  one  was  smaller  in  size 
than  the  other.  In  a  series  of  nine  cases,  studied  by  Keraval,  eye 
symptoms  were  absent  in  four  cases,  an  experience  which  must  be  con- 
sidered, however,  as  quite  unusual. 

Amblyopia,  amaurosis,  optic  atrophy  are  all  physical  signs,  the 
detection  of  which  as  we  have  already  seen  may  be  of  great  moment  to 
the  diagnosis.     That  amblyopia,  transient  amaurosis,  and  even  optic 


PAKESIS  491 

atrophy  may  be  the  first  noticeable  physical  sign  of  paresis  should  be 
especially  borne  in  mind. 

The  extra-ocular  palsies  and  anomalies  of  movement,  though  not 
striking  in  paresis,  should  be  remembered  especially  because  of  their 
value  in  differentiating  the  affection  from  syphilis,  gross  organic  disease, 
and  perhaps  tabes. 

The  eyes  in  juvenile  paresis  present  a  symptom  group  similar  to 
that  found  in  older  subjects.  In  keeping  with  the  fact  that  pupillary 
changes  are  apt  to  be  observed  at  early  ages  in  paresis,  Marchand  noted 
sluggish  light  reaction  in  a  boy  of  18  together  with  inequality  of  the 
pupils.  Jeffrey  likewise  noted  Argyll-Robertson  pupils,  together  with 
inequality  in  a  juvenile  case.  Nonne,  in  a  boy  of  twelve,  noted  loss  of 
the  light  reflex  in  the  right  pupil  and  sluggishness  of  the  reflex  in  the 
left  pupil.  The  pupils  were  mydriatic  and  unequal,  while  the  con- 
vergence reflex  also  was  very  slight.  Raymond,  in  a  girl  of  twelve, 
noted  diminution  of  the  light  reflex,  likewise  with  dilated  and  unequal 
pupils.  Regis  made  a  similar  observation  of  a  patient  of  twenty-three. 
There  was  loss  of  the  light  reflex  especially  upon  the  left  side,  together 
with  unequal  pupils ;  there  was  marked  diminution  also  of  the  reaction 
•of  accommodation.  In  a  boy  of  eighteen,  Marchand  also  noted  sluggish 
pupillary  reactions,  the  right  pupil  being  larger  than  the  left.  Hirschl 
noted,  in  a  girl  of  twenty-two,  that  the  right  pupil  was  large  and  slug- 
gish to  light,  while  the  left  did  not  react  to  accommodation.  In  a  boy 
of  sixteen  he  noted  unequal  pupils,  loss  of  the  light  reflex  upon  both 
sides.  In  a  boy  of  nineteen  he  again  noted  that  the  right  pupil  was 
larger  than  the  left  and  also  that  there  was  present  a  loss  of  the  light 
reflex.  In  a  man  of  twenty-four,  the  same  observer  noted  that  the  right 
pupil  was  larger  than  the  left,  with  sluggish  reaction  to  light;  in  the 
left  pupil  the  light  reaction  was  lost ;  both  pupils  presented  sluggish 
reaction  to  accommodation. 

In  senile  paresis,  upon  the  other  hand,  Toulouse  noted  inequality  of 
pupils  in  a  man  of  seventy-two ;  Marchand,  inequality  of  the  pupils  in 
a  man  of  sixty,  and  Doutrebente,  inequality  of  pupils  in  a  man  of  sixty- 
five,  together  with  sluggishness  of  the  light  and  consensual  reflexes, 
especially  upon  the  left  side. 

A  subject  but  little  studied  and  of  relatively  slight  importance  is 
the  sympathetic  pupillary  reaction.  This  consists  of  a  dilatation  of 
the  pupil  which  ensues  upon  irritation  of  sensory  nerves  of  the  skin  of 
the  face,  neck,  or  other  contiguous  areas.  It  is  best  elicited  by  pricking 
or  pinching  the  skin.  Hirschl  found  that  in  all  the  cases  studied  by  him, 
the  sympathetic  reflex  was  absent  when  the  Argyll-Robertson  pupil  was 


492  THE    EYE    AND    :N^ERV0US    SYSTEM 

present,  except  in  two.  It  was  also  frequently  absent  when  the  light  reac- 
tion was  sluggish,  and  this  was  also  frequently  the  case  even  when  the 
light  reflex  was  still  preserved.  When  an  Argj'll-Robertson  pupil  was 
present  upon  the  one  side  and  absent  upon  the  other,  the  sympathetic  re- 
flex was  absent  upon  both  sides.  The  loss  of  the  sympathetic  reflex, 
however,  was  also  noted  by  Hirschl  in  senility  and  alcoholism,  and  he 
believes  that  in  cases  in  which  these  two  factors  can  be  excluded,  this 
symptom  offers  some  diagnostic  value.  Hirschl  also  observed  that  in 
cases  in  w^hich  this  reflex  was  absent,  there  were  atrophic  changes  in  the 
sympathetic  nerve,  sympathetic  ganglia,  and  in  the  anterior  horns  of  tjie 
lower  and  upper  dorsal  cord.  Moeli  found  that  the  reaction  was  much 
more  frequently  lost  in  cases  in  which  the  light  reflex  had  suffered. 
Hillenberg  found  similar  results. 

Among  the  pupillary  phenomena  still  to  be  mentioned  is  that  known 
as  the  paradoxical  pupillary  reaction.  Here  there  is  an  apparent  re- 
versal of  the  light  reflex, — that  is,  an  increase  of  light  is  followed  by 
dilatation  of  the  pupil  and  diminution  of  light  by  contraction  of  the 
pupil.  The  occurrence  of  this  reaction  is  an  exceedingly  rare  one  in 
paresis.  In  fact,  but  one  case,  that  of  Morselli,  is  known  to  the 
writer;  in  this  instance  dilatation  of  the  pupil  followed  the  light  im- 
pression. It  is  important  to  bear  in  mind  that  a  paradoxical  pupillary 
light  reaction  may  be  apparent  only  and  not  genuine, — for  instance,  a 
weakness  or  paresis  of  accommodation  occurring  at  the  moment  that 
the  light  test  is  made,  may  bring  about  a  dilatation  of  the  pupil. 
Similarly  a  paresis  of  the  convergence  reaction,  accompanied  it  may 
be  by  divergence  of  the  eyeballs,  may  also  occur  at  the  moment  that 
the  light  test  is  made,  and  thus  give  rise  to  a  dilatation  of  the  pupil.  A 
high  degree  of  amblyopia  or  amaurosis  may  be  associated  with  an  im- 
pairment of  accommodation  and  convergence,  and  may  further  account 
for  the  occurrence  of  such  a  phenomenon.  Shock,  fright,  nervousness,  or 
hysteria  may  also  play  a  role.  In  practice,  as  far  as  paresis  is  con- 
cerned, the  paradoxical  reaction  of  the  pupil  being  so  rare,  may  be 
ignored. 

The  synergic  narrowing  of  the  pupil  that  accompanies  contraction 
of  the  orbicularis  palpebrarum,  and  which  has  been  in  recent  years 
studied  by  A.  Westphal  and  Piltz,  and  w^liich  had  been  earlier  noted  by 
von  Graefe,  by  Galassi,  and  by  Moeli,  is  another  pupillary  reaction 
which  may  be  mentioned.  It  has,  however,  been  studied  so  little  in 
paresis,  that  it  cannot  be  said  to  possess  any  symptomatic  value.  It 
may  be  added  that  another  pupillary  phenomenon,  hippus,  likewise 
plays  no  role  in  the  syndrome  of  paresis. 


TABES  493 

I  TABES. 

The  ocular  phenomena  of  tabes  constitute  a  group  of  symptoms  of 
great  diagnostic  value.  As  in  paresis,  they  may  involve  varied  struc- 
tures and  functions, — e.g.,  the  iris,  its  movements  and  reflexes;  the 
various  extrinsic  muscles,  the  optic  nerve  and  vision.  Tabes  is  an  affec- 
tion with  widely  diffused  lesions  and  it  is  but  natural  that  the  early 
clinical  manifestations  should  vary  somewhat  in  different  cases.  Ac- 
cordingly the  eye  symptoms  make  their  appearance  somewhat  earlier  in 
some  cases  than  in  others. 

In  general  terms,  the  symptoms  of  tabes  consist  of  a  triad  made  up 
of  incoordination,  loss  of  tendon  reflexes,  and  the  Argyll-Robertson  pupil, 
— the  pupil  which  no  longer  reacts  to  light  but  in  which  the  associated 
movements  of  the  iris  in  accommodation  and  convergence  are  preserved. 
The  presence  of  this  pupil  is  not  infrequently  noted  as  a  very  early  symp. 
torn.  It  may  be  noted  long  before  the  knee-jerk  is  lost  and  before  inco- 
ordination makes  its  appearance.  Sometimes  it  antedates  all  other  symp- 
toms by  a  number  of  years.  On  the  other  hand,  its  appearance  may  be 
much  delayed  and  in  rare  cases  it  may  not  be  observed  at  all  even  after 
other  symptoms  of  tabes  have  existed  for  many  years.  While  present 
in  the  great  majority  of  cases  of  tabes,  it  cannot  therefore  be  claimed 
as  a  necessary  symptom. 

It  is  important  in  this  connection  to  bear  in  mind  that  the  loss  of 
the  light  reflex  is  a  symptom  which  in  all  probability  makes  its  appear- 
ance gradually.  In  examining  the  eye,  a  difference  in  the  promptness 
and  general  character  of  the  light  reaction  and  the  reaction  to  accommo- 
dation should  always  be  noted.  A  striking  contrast  between  the  two 
reactions,  such  as  a  slight,  slow  or  sluggish  light  reflex  and  a  prompt 
reaction  to  accommodation  possesses  an  unmistakable  significance.  As 
a  matter  of  fact  there  are  many  cases  in  which,  in  the  incipient  period,  a 
fully  developed  Argyll-Robertson  pupil  is  not  present  and  in  which  a 
sluggish  or  other  change  in  the  light  reaction  is  the  only  symptom  noted. 
In  a  few  cases  also  it  has  been  observed  that  the  Argyll-Robertson  pupil 
is  not  definitely  or  permanently  established  and  exists  only  as  an  inter- 
mittent symptom.  Thus  Eichhorst  reports  two  cases  of  intermittent 
Argyll-Robertson  pupils,  both  occurring  in  women.  Treupel  reports 
another  occurring  in  a  man.  Camus  and  Chiray  report  a  most  interest- 
ing case  in  which  the  Argyll-Robertson  pupil  was  present,  accompanied 
by  gastric  crises.  It  occurred  only  during  the  crises  and  lasted  only  as 
long  as  the  crises. 

In  estimating  the  frequency  of  the  Argyll-Robertson  pupil  in  tabes. 


494  THE    EYE    AND    NERVOUS    SYSTEM 

several  factors  should  be  considered.  First,  while  it  is  true  that  this 
pupil  is  frequently  an  early  symptom,  it  is  not  rarely  a  late  symptom ; 
therefore  cases  of  the  fully  developed  disease  would,  other  things  equal, 
present  the  symptoms  in  a  larger  proportion.  Secondly,  the  proportion 
varies  according  as  the  observer  excludes  or  includes  pupils  in  which 
the  light  reflex  has  not  been  entirely  or  absolutely  lost  but  in  which  it 
has  been  unmistakably  impaired.  It  is  not  surprising  therefore  that 
there  should  be  some  differences  in  the  results  of  various  observers  and 
yet  these  differences  are  not  as  great  as  one  would  suppose.  Dillmanu, 
for  instance,  found  loss  of  the  light  reflex  in  76  per  cent,  of  cases  in 
the  fully  developed  stage  of  the  disease.  In  the  early  stage  he  found 
only  23.7  per  cent.  Leimbach  found  the  symptom  in  75.6  per  cent,  in 
the  fully  developed  disease  and  47  i^er  cent,  in  the  early  stage.  The 
chief  variation  is  found  in  the  determination  of  the  symptom  in  the 
early  stage  and  here  the  element  of  the  personal  judgment — the  per- 
sonal equation  of  the  observer — doubtless  plays  a  role.  On  the  other 
hand  it  is  difficult  to  reconcile  the  findings  of  other  observers  such  as 
Mann,  who  found  the  symptom  in  79  per  cent,  of  early  cases, — i.e.,  cases 
in  which  no  pronounced  ataxia  had  yet  sho^\^l  itself.  Statistical  studies 
have  been  made  by  a  large  number  of  observers.  Uhthoff,  for  instance, 
in  three  hundred  cases  noted  rigidity  of  the  pupil  in  71  per  cent. ;  ex- 
cluding cases  in  which  a  slight  reaction  of  the  pupil  was  still  preserved, 
the  percentage  became  62.  In  averaging  the  results  of  other  observers, 
for  instance,  Gowers,  Bernhardt,  Voigt,  Thomsen,  Siemerling,  Vincent, 
Dillmann,  Berger,  Mobius,  Marina,  Forster  and  Mann,  Uhthoff  ob- 
tained a  percentage  of  67,  which  nearly  corresponded  to  his  own  results. 
Among  other  studies  there  should  be  mentioned  the  following:  Harris, 
82.6  per  cent. ;  Leimbach,  84  per  cent. ;  Bramwell,  86.1  per  cent.,  and 
also  72.2  per  cent. ;  Mott,  80  per  cent. ;  H.  M.  Thomas,  63.6  per  cent. ; 
Thoenes,  75  per  cent ;  Collins,  80  per  cent. ;  Fulton,  67  per  cent.  The 
writer  found  among  thirty  rather  advanced  cases  in  the  wards  of  the 
Philadelphia  Hospital  63.3  per  cent.  These  figures  do  not,  it  will  be 
admitted,  vary  very  greatly.  However,  such  results  as  were  obtained  by 
F.  S.  Pearce, — namely,  one  hundred  and  fifty  cases  of  Argyll-Robertson 
pupil  in  one  hundred  and  fifty-six  cases  of  tabes,  that  is,  96  per  cent., 
— are  difficult  to  explain.  The  percentage  found  by  Riley,  who  foimd 
Argyll-Robertson  pupil  in  only  twenty-eight  cases  out  of  sixty-one, — 
namely,  46  per  cent., — must  impress  one  as  unusually  small.  This  is 
also  true  of  Vaughan's  results,  who  found  pupillary  rigidity  in  only  40 
per  cent. 

Very  interesting  and  important  are  the  results  obtained  by  Rochon- 


TABES  495 

Duvigneaud  and  Jean  Heitz,  inasmuch  as  their  studies  were  made  by  a 
special  method.  They  examined  seventy-seven  patients  who  for  the  most 
part  were  from  the  service  of  Professor  Dejerine.  The  patients  belonged 
to  all  of  the  periods  and  the  most  diverse  forms  of  the  disease.  The 
examinations  of  the  pupillary  reactions  were  made  in  an  entirely  dark 
chamber  under  special  precautions  in  order  to  determine  as  far  as  pos- 
sible the  exact  condition  of  the  light  reaction.  A  gas-lamp  furnished  the 
illumination.  The  patient  was  separated  from  the  latter  by  an  opaque 
screen.  After  lowering  the  gas,  without  absolutely  extinguishing  it,  the 
eye  to  be  examined  was  placed  in  the  most  complete  darkness  for  several 
minutes.  Then  the  operator  suddenly  raised  the  gas  flame  and  the  light 
was  projected  on  the  eye  by  means  of  an  ophthalmoscopic  mirror.  The 
eye  not  to  be  examined  remained  covered. 

Of  forty-eight  patients  examined  by  these  observers,  twenty-three 
had  lost  all  trace  of  light  reflex,  fifteen  presented  a  slight  reaction  to 
light,  and  two  reacted  almost  normally  but  not  entirely  so.  From  this  it 
appears  that  in  47.9  per  cent,  the  light  reflex  was  lost  entirely  and  that 
in  about  35  per  cent,  it  was  lost  only  incompletely;  that  is,  a  total  of 
about  83  per  cent,  presented  lost  or  impaired  light  reflex,  which  accords 
fairly  well  with  the  results  of  other  observers.  The  observations  of  these 
writers  would  go  to  show  that  the  methods  pursued  by  them  reveal  a 
partial  preservation  of  the  light  reflex  when  to  ordinary  tests  such 
preservation  would  escape  notice.  Thus  they  examined  twenty-nine 
cases  by  the  ordinary  methods, — i.e.,  with  partial  obscurity  and  moving 
light.  Of  these  only  four  presented  an  incomplete  loss  of  the  light  reflex, 
a  percentage  of  13,  as  compared  with  35.  The  authors  also  lay  especial 
stress  on  the  importance  of  studying  the  dilatation  of  the  pupil  in  the 
dark.  This  they  maintain  is  an  active  act,  an  impairment  of  which  is 
of  profound  significance.  • 

Statistical  studies  for  reasons  already  pointed  out  can  hardly  be 
expected  to  yield  precise  results.  The  conclusion,  however,  is  unques- 
tionably justified,  and  this  indeed  is  the  sum  total  of  individual  clinical 
experience — that  the  Argyll-Robertson  pupil  is  present  in  a  large  ma- 
jority of  cases. 

An  unilateral  Argyll-Robertson  pupil  is  relatively  infrequent.  Thus, 
Berger  found  an  unilateral  Argyll-Robertson  pupil  only  three  times  in 
one  hundred  and  nine  tabetics,  and  Gowers  once  in  seventy-two.  H.  M. 
Thomas,  on  the  other  hand,  states  that  he  found  the  Argyll-Robertson 
pupil  in  seventy  out  of  one  hundred  and  eleven  cases  and  upon  both 
sides  in  fifty-seven;  that  is,  it  was  unilateral  in  thirteen.  Probably 
pupils  with  but  slightly  impaired  reaction  were  here  excluded.     Out  of 


496  THE    EYE    AND    NERVOUS    SYSTEM 

thirty  of  the  writer's  cases,  it  was  unilateral  in  four.  It  remains  an 
unquestionable  fact  that  in  tabes  an  unilateral  Argyll-Robertson  pupil 
is  the  exception,  and  there  is  in  this  respect  a  decided  departure  from 
the  symptomatology  of  paresis,  in  which  affection  this  symptom  is 
observed  much  more  frequently. 

Finally  it  should  be  stated  that  the  Argyll-Robertson  pupil  may  be 
found  when  the  pupil  is  of  average  or  normal  size,  when  it  is  myotic  or 
when  it  is  dilated. 

Irregularity  or  deformity  of  the  pupil  is  found  quite  commonly  in 
tabes,  as  in  paresis  and  syphilis.  Joffroy  states  that  he  has  never  seen 
a  case  of  Argj'll-Robertson  pupil  without  some  deformity  in  the  latter. 
This  statement,  while  rather  sweeping,  accords  in  the  main  with  the 
experience  of  other  observers.  Deformity  is  at  times  the  initial  pupillary 
symptom  observed  and  it  may  be  regarded  as  a  precursor  of  the  changes 
in  the  pupillary  reflexes  later  to  supervene.  Joffroy,  indeed,  regards  it  as 
of  the  same  semeiologic  value  as  the  Argyll-Robertson  pupil.  Bumke  also 
insists  upon  the  importance  of  the  loss  of  the  circular  shape  of  the  pupil. 

Next  to  the  Argyll-Robertson  pupil  in  importance,  is  the  size  of  the 
pupil.  The  pupil  may  be  normal,  unusually  small,  or  dilated.  Very 
frequently  it  is  decidedly  contracted  and  this  diminution  in  size  may 
be  moderate  in  degree  or  excessive.  Pin-point  pupils  are  occasionally 
met  with.  Myosis  frequently  accompanies  the  Argj^ll-Robertson  pupil, 
though  it  is  by  no  means  a  necessary  concomitant,  for  the  latter  may  be 
associated  with  pupils  of  normal  size  or  pupils  in  a  state  of  mydriasis. 
Thus,  Erb  found  it  in  44  per  cent. ;  Uhthoff,  Dillmann,  and  Voigt  found 
it  in  a  somewhat  smaller  number.  Their  findings  averaged  by  Bumke 
amount  to  about  24  per  cent.  Collins  found  the  pupils  small  in  45  per 
cent.;  the  writer  in  36.6  per  cent.  Rochon-Duvigneaud  and  Ileitz  found 
40  per  cent,  of  bilateral  myosis  and  9  per  cent,  of  unilateral  myosis. 
In  every  case  of  myosis  there  was  a  pure  Argyll-Robertson  pupil,  ex- 
cept in  three.  In  the  latter  contraction  to  convergence  was  slow  and 
incomplete.  External  ophthalmoplegias  were  not  found  associated  with 
the  myosis.  These  authors  also  point  out  that  optic  atrophy  is  more 
frequently  found  associated  with  mydriatic  eyes  than  with  myotic  eyes. 
In  forty  myotic  patients,  they  found  only  three  with  total  atrophy 
and  blindness  and  three  with  beginning  atrophy. 

Myosis,  it  would  appear,  is  accompanied  quite  regularly  by  the 
Arg}'ll-Robertson  sign.  In  a  number  of  cases,  however,  a  slight  reaction 
to  light  is  preserved.  It  is  very  exceptional,  further,  to  find  a  myotic 
patient  with  optic  atrophy  and  more  rarely  still  with  internal  or  ex- 
ternal ophthalmoplegia. 


TABES  497 

From  an  analysis  of  eleven  hundred  cases,  Dufour  comes  to  the 
conclusion  that  myosis,  associated  with  other  disturbances  of  the  pupils 
or  of  the  nervous  system,  confirms  the  diagnosis  of  syphilis  or  organic 
nervous  disease. 

Mydriasis  is  met  with  much  less  frequently  than  myosis.  Thus 
Riley  in  sixty-one  cases  found  myosis  in  twenty-eight  and  mydriasis  in 
eight.  Bramwell  in  one  hundred  and  fifty-five  cases  of  tabes  found  the 
pupils  contracted  in  sixty-nine  and  dilated  in  eight.  In  twenty-seven 
they  were  of  medium  size.  Collins  found  mydriasis  in  only  5  per  cent, 
and  when  found  it  was  nearly  always  associated  with  irregularity  of  the 
pupils.  Rochon-Duvigneaud  and  Heitz  found  mydriasis  in  20  per  cent, 
bilateral  and  11  per  cent,  unilateral  of  their  seventy-seven  cases.  My- 
driasis was  accompanied  by  a  complicated  Argyll-Robertson  pupil, — that 
is  to  say,  a  pupil  in  which  the  reaction  to  accommodation  was  also  im- 
paired. These  writers  point  out  that  when  blindness  is  present,  it  is  as 
a  rule,  accompanied  by  mydriasis.  They  found  seven  blind  tabetics 
with  bilateral  mydriasis  and  two  with  unilateral  mydriasis.  In  all  of 
the  cases  the  reflex  to  convergence  was  absent  or  diminished.  In  twelve 
cases  of  mydriasis,  they  found  two  with  bilateral  internal  ophthalmo- 
plegia, ten  with  unilateral  ophthalmoplegia,  and  in  two  cases  there  was 
a  nuclear  palsy  of  the  third  pair,  incomplete  for  the  external  muscles 
but  complete  for  the  ciliary  muscles.  In  the  remaining  cases,  ten  in 
number,  all  unilateral,  there  were  five  times  found  besides  the  internal 
ophthalmoplegia,  also  various  palsies  of  the  external  muscles  of  the  third 
nerve  supply ;  especially  was  ptosis  found.  Once  internal  ophthalmo- 
plegia with  palsy  of  the  sixth  nerve  was  found ;  four  times  internal 
ophthalmoplegia  only  was  present.  In  five  cases  of  mydriasis  there  was 
no  blindness  or  paralysis  of  accommodation. 

The  mydriatic  pupil  is  accompanied  by  various  other  disturbances, 
a  fact  which  is  directly  opposed  to  the  findings  in  the  myotic  pupil. 
Thus  the  light  reflex  is  totally  lost,  the  convergence  reflex  is  always  in- 
complete or  lost,  while  optic  atrophy  and  ophthalmoplegias  are  fre- 
quent. Finally  it  is  mostly  in  cases  with  mydriasis  that  inequality 
of  the  pupils  is  found. 

There  is  a  group  of  tabetic  pupils  in  which  the  sphincter  iridis  pre- 
sents a  reflex  paralysis,  while  the  ciliary  muscle  remains  intact  and 
the  accommodation  is  preserved.  This  paralysis  of  the  sphincter  could 
perhaps  be  attributed  to  a  limited  nuclear  lesion,  for  the  nucleus  of 
the  circular  fibres  of  the  iris  is  entirely  distinct  from  that  of  the  ciliary 
muscle.  Again,  while  paralysis  of  accommodation  is  often  accompanied 
by  paralysis  of  the  external  muscles,  in  not  one  case  did  Rochon-Duvig- 
32 


498  THE    EYE    AND    NERVOUS    SYSTEM 

neaud  and  Heitz  find  such  a  paralysis  associated  with  the  isolated  palsy 
of  the  sphincter  iridis. 

It  has  been  observed  that  during  a  gastric  crisis,  pupils  previously 
noted  as  normal  may  dilate,  but  that  in  such  case  they  return  to  their 
former  size  after  the  crisis  has  subsided.  Such  an  observation  has  been 
made  by  Rochon-Duvigneaud  and  Heitz,  dilatation  and  subsequent  re- 
turn to  normal  occurring  in  one  of  their  patients  at  every  gastric  crisis. 
Similar  observations  have  been  made  by  Trousseau  and  Raymond. 

Schmeichler  states  that  he  has  never  seen  unusually  large  pupils  in 
the  early  stages  of  tabes.  This  is  an  important  observation  and  in  keep- 
ing with  general  experience. 

It  is  interesting  to  note  also  that  atropine  gives  an  incomplete  or 
imperfect  dilatation  of  myotic  pupils,  and  that  pilocarpine  in  mydriasis 
yields  a  contraction  which  is  not  very  pronounced. 

Mydriasis,  according  to  Rochon-Duvigneaud  and  Heitz,  always  ac- 
companies the  complicated  Argyll-Robertson  pupil, — that  is,  the  pupil 
in  which  the  light  reaction  is  totally  abolished,  but  in  which  the  reac- 
tion to  convergence  is  also  lost  or  is  poor.  In  a  certain  number  of  cases, 
mydriasis  depends  upon  blindness.  When  the  mydriasis  is  accompanied 
by  a  preservation  of  vision  the  state  of  the  accommodation  should  be 
investigated,  taking  into  account,  of  course,  the  age  and  refraction  of 
the  patient.  Usually  under  these  circumstances  the  accommodation  is 
paralyzed.  There  is  then  a  total  internal  ophthalmoplegia,  involving  the 
sphincter  iridis  and  the  ciliary  muscle.  Very  rarely  temporary  dila- 
tation of  the  pupils,  that  is  "  alternating  mydriasis,"  is  met  with.  It 
is  much  less  frequent  in  tabes  than  in  general  paralysis. 

Inequality  of  the  pupils  should,  even  if  slight,  be  regarded  with  sus- 
picion, especially  if  one  of  the  pupils  reacts  sluggishly  to  light  or  shows 
impairment  of  the  light  reflex,  no  matter  how  slight.  Collins  noted 
inequality  in  23  per  cent,  of  his  cases.  He  adds  that  it  was  probably 
not  noted  in  cases  in  which  the  inequality  was  very  slight.  Bramwell 
found  the  pupils  unequal  in  34.8  per  cent.  Vaughan  found  60  per  cent, 
of  inequality  of  the  pupils,  a  percentage  which  it  cannot  be  denied  is 
out  of  keeping  with  general  experience.  The  writer  found  46.6  per 
cent,  of  decided  inequality  and  13.3  per  cent,  of  very  slight  inequality. 
It  will  be  noted  that  the  percentage  of  inequality  in  tabes  is  decidedly 
less  than  in  paresis.  Among  other  statistics  are  those  grouped  by 
Rumke, — namely,  Bernhardt,  who  saw  inequality  of  the  pupils  in 
43  per  cent.,  Voigt  in  40  per  cent.,  Dillmann  in  34  per  cent.,  Forster 
in  33  per  cent.,  Uhthoff  in  28  per  cent.,  and  Berger  in  27  per  cent. 
Inequality,  if  observed  as  an  isolated  sign  and  without  other  ocular 


TABES  499 

or  nervous  symptoms,  has  no  special  significance.  In  other  words,  in- 
equality may  be  perfectly  physiological.  It  must,  however,  be  borne  in 
mind  that  a  physiological  inequality  is  usually  slight.  (See  Chapter 
VI,  p.  314). 

The  optic  nerve  is  frequently  involved  in  tabes.  There  is  present  in 
such  cases  a  simple  and  progressive  atrophy  of  both  optic  nerves, — an 
atrophy  which  may  and  frequently  does  lead  to  complete  blindness.  It 
is  a  symptom  which  is  frequently  associated  with  an  Argyll-Kobertson 
pupil,  but  it  may  exist  without  the  latter  and  it  may  be  the  first  and 
only  symptom.  Indeed,  simple  optic  atrophy,  in  the  absence  of  other 
signs,  is  very  suggestive  of  tabes.  Usually  it  makes  its  appearance  early ; 
it  is  only  in  exceptional  cases  that  optic  atrophy  is  a  late  symptom.  Col- 
lins believes  that  the  discoloration  of  the  optic  nerve  begins  within  five 
years  after  the  onset  of  the  disease.  He  regards  it  as  an  early  symptom 
and  believes  that  the  changes  probably  start  peripherally  in  the  retina. 
So  frequently  is  early  optic  atrophy  noted  that  it  is  justly  counted  along 
with  the  Argyll-Robertson  pupil  as  one  of  the  first  symptoms  of  tabes. 
Mott  also  regards  limitation  of  the  visual  field  and  optic  atrophy  as  the 
early  signs  of  tabes.  Gowers  has  seen  optic  atrophy  fifteen  or  twenty 
years  before  other  tabetic  signs  made  their  appearance.  Xot  infre- 
quently one  eye  is  involved  earlier  and  to  a  larger  extent  than  its  fel- 
low, but  the  optic  atrophy  is  never  really  unilateral.  Defective  vision 
is  the  eventual  result.  The  first  indication  is  a  narrowing  of  the  visual 
field.  The  impairment  usually  consists  of  an  irregular  concentric  con- 
traction; at  times  the  field  is  irregularly  indented.  There  may  be  lit- 
tle or  no  diminution  of  the  acuity  of  the  central  vision  and  no  visible 
pallor  of  the  disc.  Central  scotomata  are  vetj  exceptional ;  when  pres- 
ent they  are  frequently  explicable  on  the  ground  of  a  complicating  alco- 
holism. Occasionally  the  visual  field  is  limited  on  one  side,  as,  for 
instance,  in  the  case  reported  by  Halbau,  in  which  the  visual  field  in 
the  right  eye,  especially  for  red,  was  limited  on  the  temporal  side. 
Hemianopsia,  incomplete  in  character,  is  a  rare  anomaly  of  the  field  and 
is  apparently  due  to  an  asymmetrical  degeneration.  It  has  been  noted 
by  Gowers,  Berger  and  Gabrielides.  In  Gowers's  and  Gabrielides's 
cases,  there  was  a  double  temporal  hemianopsia  referable  to  a  change 
in  the  chiasm.  In  Berger's  case,  there  w^as  a  right  homonymous  hemian- 
opsia. There  were  also  present  Argyll-Robertson  pupil,  double  optic 
atrophy,  palsy  of  the  right  fourth  nerve  and  other  symptoms  of  tabes. 
The  loss  may  also  involve  early  the  color  perception,  the  vision  for  green, 
red,  and  blue  being  affected  in  the  order  named.  Dysehromatopsia  was 
first  noted  in  tabes  by  Benedict.     Generally  speaking  dysehromatopsia 


500  THE    EYE    AND    NERVOUS    SYSTEM 

is  partial  and  concerns  mostly  red  and  green,  the  red  being  taken  for 
black  and  the  green  for  gray.  A  curious  symptom  occasionally  noted  in 
tabes  is  green  vision.  Such  a  case,  for  instance,  has  been  placed  upon 
record  by  W.  Dodd,  in  which  the  patient  saw  everything  a  bright  emerald 
green  in  color.  Occasionally  he  saw  rose-colored  spots  in  the  green. 
These  colors  increased  in  intensity  when  he  was  fatigued.  He  oc- 
casionally also  saw  a  brilliant  light.  In  1891  diplopia  had  been  noted 
from  which  the  patient  recovered.  Ocular  vision  remained  good  until 
1897.  Examined  in  September,  1898,  green  vision  was  noted.  The 
vision  of  the  right  eye  was  2-60,  of  the  left  1-60. 

Rarely  optic  atrophy  is  accompanied  by  photophobia  and  blepharo- 
spasm. 

Statistics  as  to  the  relative  frequency  of  optic  atrophy  in  tabes  vary 
very  widely  and  are  on  the  whole  unsatisfactory.  Collins  found  optic 
atrophy  in  14  per  cent.,  Bonar  8  per  cent.,  Thomas  10  per  cent.,  Riley 
11.4  per  cent.,  Bramwell  22  per  cent.,  Sarbo  30.4  per  cent.  Eulton 
found  only  2  per  cent.,  Vaughan  noted  50  per  cent.,  Dillmann  42  per 
cent.,  Berger  44  per  cent.,  Leimbach  16.75  per  cent.,  and  Grosz  in 
88  per  cent.  Mott  places  the  percentage  of  cases  of  optic  atrophy  in 
tabes  at  80.  Pearce,  on  the  other  hand,  found  only  thirty  cases  of 
optic  atrophy  in  one  hundred  and  nineteen  cases.  Marina  observed 
optic  atrophy  in  9  per  cent.,  Grosz  in  88  per  cent.,  UhthofF  in  20  per 
cent.,  Bernhardt  in  10  per  cent.,  Berger  in  44  per  cent,  and  Silex 
15  per  cent,  Oliver  believes  that  optic  atrophy  is  met  with  in  10  to 
15  per  cent. 

It  has  long  been  held  that  when  tabes  begins  with  optic  atrophy 
that  the  affection  soon  becotnes  arrested.  The  very  exliaustive  investiga- 
tions, however,  of  Andre  Leri  fail  to  substantiate  this  position.  Numer- 
ous observations  fail  to  prove  that  the  occurrence  of  optic  atrophy  is 
attended  by  a  cessation  of  the  progress  of  the  disease.  A  case  reported 
by  Bramwell  will  serve  as  an  illustration.  In  a  man  of  thirty,  two 
years  previous  to  all  signs  of  tabes,  optic  atrophy  with  blindness  and 
dilatation  of  the  pupils  were  observed.  Shortly  after,  however,  other 
important  signs  of  tabes  made  their  appearance.  Collins  also  noted  that 
in  no  case  did  the  superimposition  of  blindness  seem  to  have  any  effect 
upon  the  course  of  the  disease.  Gowers  maintains  that  optic  atrophy 
is  generally  an  early  symptom  beginning  before  incoordination  is  de- 
veloped and  that  in  a  large  number  of  cases  ataxia  does  not  supervene. 
Benedict  believes  that  tabetic  motor  symptoms,  even  after  they  have 
attained  a  high  degree,  retrograde  after  the  optic  atrophy  appears.  In 
eight  out  of  thirty-three  cases  observed  by  Bramwell,  the  development 


TABES  501 

of  the  optic  atrophy  seemed  to  be  attended  by  an  amelioration  of  the 
symptoms  and  a  retardation  of  the  tabetic  process.  In  twelve  cases,  how- 
ever, the  development  of  the  optic  atrophy  was  not  followed  by  an 
amelioration  of  the  symptoms  and  had  no  retarding  effect  upon  the 
course  of  the  disease.  In  nine  out  of  twenty-six  cases  in  which  optic 
atrophy  was  very  marked,  the  development  of  the  optic  atrophy  coin- 
cided wath  a  rather  apparent  arrest  in  the  tabetic  process. 

The  details  obtained  by  some  observers  are  interesting;  thus  Bram- 
well,  who  studied  one  hundred  and  fifty-five  cases  of  tabes,  noted  that 
in  seven  of  his  cases,  in  which  optic  atrophy  was  present,  the  symptom 
was  much  more  marked  in  one  eye  than  in  the  other  and  that  in  three 
cases  it  was  limited  to  one  eye.  In  seven  cases  it  was  slight,  in  seven 
marked,  and  in  twenty-one  very  marked  or  complete,  the  patient  being 
almost  blind,  quite  blind,  or  only  able  to  distinguish  light  from  dark- 
ness. In  one  case,  double  optic  neuritis,  marked  in  the  left  eye  and 
slight  in  the  right,  preceded  the  optic  atrophy.  Such  an  occurrence 
suggests,  however,  an  active  syphilitic  process  rather  than  tabes.  In 
nine  patients  dimness  of  vision,  due  to  optic  atrophy,  was  the  initial 
symptom  of  the  disease.  In  twenty-five  of  the  cases  in  which  optic 
atrophy  was  present,  there  was  ataxia. 

Ocular  palsies  are  extremely  common  in  tabes  and  in  quite  a  large 
number  of  cases  they  occur  as  very  early  symptoms.  They  involve 
variously  the  third,  fourth  and  sixth  nerves.  They  usually  appear  sud- 
denly and  may  disappear  with  or  without  treatment.  Their  duration  is 
usually  short,  lasting  a  few  weeks,  a  few  days,  or  indeed  only  a  few 
hours.  They  may,  however,  be  permanent,  though  this  is  rare  and 
points  to  a  syphilitic  rather  than  to  a  tabetic  origin.  However,  a  per- 
sistent ocular  palsy  may  be  present  in  the  very  beginning  of  the  affec- 
tion but  is  more  frequently  observed  in  the  later  stages  of  the  disease. 

The  ocular  palsies  of  tabes  present  nothing  that  is  characteristic. 
Though  they  develop  rapidly,  they  usually  disappear  gradually  and 
sometimes  slowly.  The  muscular  involvement  is  seldom  complete  and 
is  usually  limited  to  one  eye.  Further,  the  palsy  is  never  a  palsy  of 
associated  muscles  or  movements,  but  always  involves,  irregularly,  dif- 
ferent muscles  of  one  or  both  eyes.  Occasionally  a  complete  ophthalmo- 
plegia appears.  This  again  is  to  be  regarded  as  pointing  to  a  syphilitic 
rather  than  a  tabetic  origin.  It  is  noteworthy  that  in  syphilitic  ocular 
palsy,  a  large  number  of  muscles  are  affected  at  the  same  time  while 
in  tabes  involvement  of  a  single  muscle  is  the  rule.  It  is  further  in- 
teresting and  in  practice  important  to  note  that  it  is  extremely  rare  in 
tabes  to  find  ocular  palsies  without  a  loss  of  the  light  reaction.     Opin- 


502  THE    EYE    AND    NERVOUS    SYSTEM 

ions  differ  as  to  the  muscles  most  frequently  involved.  Thus  Swanzy 
and  others  believe  that  the  external  rectus  is  most  frequently  affected, 
while  Charcot  believed  that  the  oculomotor  nerve  supply  suffered  first. 
Javal  found  that  the  levator  was  most  frequently  involved,  then  the  in- 
temus,  the  externus  and  the  superior  oblique.  Certain  it  is  that  ptosis 
is  very  frequent.  It  may  be  slight  or  pronounced  and  may  be  single  or 
double.  It  does  not  always  depend  upon  paralysis,  especially  when 
slight;  it  is  then  due  to  atony  of  the  levator  and  under  such  circum- 
stances may  be  inconstant.  Finally  it  rarely  exists  alone.  Very  fre- 
quently other  muscles  are  involved,  giving  rise  to  a  diplopia.  This 
diplopia  is  usually  due  to  a  palsy  in  one  eye,  is  very  common,  and  is 
usually  transitory.  Transitory  diplopia  is  frequently  an  important 
point  in  the  history  of  the  early  period  of  a  case  of  tabes.  At  times  the 
diplopia  depends  upon  palsy  of  the  external  rectus,  at  times  it  depends 
upon  palsy  of  the  internus  or  other  muscles  of  the  oculomotor  supply, — 
e.g.,  the  superior  rectus.  Involvement  of  the  fourth  nerve, — especially 
isolated  involvement, — is  infrequent,  if  not  rare.  The  diplopia,  though 
transitory  for  the  most  part,  is  in  rare  cases  permanent.  Further,  hav- 
ing once  disappeared,  it  may  recur.  It  is  sometimes  an  exceedingly 
early  symptom;  thus,  in  a  case  reported  by  Harris,  it  existed  for  six 
years  before  other  symptoms  of  tabes  made  their  appearance. 

Rarely  muscles  other  than  the  ocular  muscles  may  be  palsied  at  the 
same  time.  Thus  in  a  case  reported  by  Turner,  a  man  of  forty,  there 
was  some  ptosis  and  also  some  bilateral  palsy  of  the  soft  palate  and 
paralysis  of  the  vocal  cords.  Rarely  also  instead  of  an  ocular  palsy,  a 
blepharospasm  may  be  noted  or  a  blepharospasm  may  precede  the  de- 
velopment of  ocular  palsies. 

A  most  interesting  form  of  ocular  palsy  has  been  reported  by  Egger. 
In  one  of  his  clinics,  Dejerine  presented  a  case  of  bulbar  tabes  in  which 
of  the  twelve  pairs  of  cranial  nerves,  the  affection  had  only  spared  the 
IV  pair,  the  III,  V,  VI,  VII,  VIII,  IX,  X,  XI,  and  XII  pairs  were  all 
more  or  less  strongly  involved.  A  bilateral  and  absolute  deafness  char- 
acterized the  lesion  of  the  VIII  pair,  whilst  a  slight  ptosis  of  the  left 
upper  eyelid  and  a  paresis  of  the  left  internal  rectus  were  the  sole 
manifestations  of  involvement  of  the  III  pair.  Although  there  was  no 
paralysis  of  any  one  muscle  of  the  eye,  there  was  notwithstanding  a 
marked  reduction  in  the  motility  of  the  eyes.  When  in  the  normal 
individual  the  head  is  turned  to  one  side — for  example,  to  the  left 
on  its  vertical  axis — the  eyes  in  order  to  preserve  their  primary  po- 
sition move  slowly  in  the  opposite  direction — i.e.,  to  the  right — 
and  compensate  by  movement  equivalent  to  the   degree  of  rotation 


TABES  503 

effected  by  the  head,  but  at  a  given  moment  the  eyes  abandon  this 
primary  position  and  a  return  movement  ensues.  This  go  and 
come  repeats  itself  about  a  dozen  times  for  one  complete  rotation  of 
the  body  around  its  vertical  axis  and  gives,  if  the  rotatory  movements 
acquire  a  certain  speed,  the  aspect  of  a  nystagmus  which  has  been  called 
the  transverse  nystagmus  of  rotation.  These  movements  are  regulated 
by  the  pressure  of  the  liquid  in  the  semicircular  canals,  varying  for 
each  position  of  the  head.  Each  canal  is  sensitive  for  the  movements 
affecting  its  plane.  A  rotation  in  a  horizontal  plane  to  the  left,  how- 
ever, affects  only  the  left  horizontal  canal,  in  the  interior  of  which  the 
liquid,  thanks  to  the  law  of  inertia  of  matter,  concentrates  toward  the 
ampulla  and  there  produces  an  excitation  which  informs  the  centre  as 
to  the  direction  of  the  change  of  position.  The  movements  in  the  sagittal 
or  vertical  plane  are  only  registered  by  the  sagittal  or  superior-semi- 
circular canals  and  the  movements  in  the  frontal  and  transverse  planes 
by  their  respective  canals,  and  as  each  canal  perceives  only  one  movement 
— that  is  to  say,  the  change  of  position  in  its  own  plane — it  can  only  ex- 
cite a  compensatory  movement  which  the  direction  of  the  change  of  posi- 
tion calls  forth.  It  is  thus  that  the  horizontal  canals  preside  over  the  copi- 
pensations  of  lateral  movements  in  innervating  the  external  and  internal 
recti.  The  sagittal  canals  regulate  the  vertical  oscillations  and  the 
frontal  canals  the  muscular  apparatus  affecting  compensatory  rotation 
around  the  visual  axis.  If  the  tips  of  the  middle  and  ring  fingers  of 
each  hand  are  lightly  placed  against  the  eyeballs  in  the  healthy  man, 
these  movements  can  readily  be  felt.  In  Egger's  case  not  the  least  trace 
of  movement  was  transmitted  to  the  fingers  for  any  change  of  position. 
The  phenomena  of  nystagmus  of  rotation  and  of  post-rotatory  nystagmus 
were  lost.  In  semidarkness,  where  the  retinal  reflex  did  not  impede  by 
the  fixation  of  objects  the  influence  of  the  labyrinth,  he  observed  in  the 
two  open  eyes  that  the  latter,  instead  of  making  a  movement  opposite  to 
that  of  the  direction  of  the  head  in  order  to  preserve  their  primary  posi- 
tion, remained  absolutely  immobile  in  their  orbits.  They  did  not  par- 
ticipate in  the  movements  of  the  head.  This  absence  of  compensatory 
movement  is  the  rule  with  deaf-mutes  and  is  observed  in  some  cases  of 
internal  otitis.  Among  tabetics  it  had  up  to  the  case  reported  by  Egger 
not  been  observed.  The  name  labyrinthic  ophthalmoplegia  has  been 
proposed  by  Egger  for  this  symptom. 

The  cause  of  the  diplopia  or  ptosis  of  tabes  appears,  according  to 
Harris,  to  be  some  vascular  derangement  or  slight  ependymitis  in  the 
nuclear  region.  Pierret  believes  that  it  begins  as  a  nuclear  affection  and 
extends  gradually  downward  toward  the  periphery   as   a   descending 


504  THE    EYE    AND    NERVOUS    SYSTEM 

neuritis  and  here  continues  after  the  nuclear  involvement  has  disap- 
peared. Dejerine  says  that  not  all  ocular  palsies  in  tabes  are  of  nu- 
clear origin,  and  Swanzy  claims  that  a  complete  and  permanent  paralysis 
of  an  isolated  muscle  is  due  to  a  peripheral  lesion  and  not  nuclear. 
Complete  oculomotor  palsy,  rarely  noted  as  an  early  symptom,  is 
probably  always  due  in  part  at  least  to  an  involvement  of  the  oculo- 
motor nucleus.  This  was  the  finding  in  a  case  of  Marina  in  which  a 
third-nerve  palsy  disclosed  at  autopsy  a  degeneration  of  the  oculomotor 
nucleus  besides  a  neuritis  of  the  trunk.  It  is  probable  also  that  in  cases 
where  an  ocular  palsy  becomes  permanent,  there  is  also  involvement 
of  the  nucleus. 

Some  idea  of  the  frequency  of  ocular  palsies  may  be  gained  from 
the  following  figures :  Thvis,  H.  M.  Thomas  found  affections  of  the  ex- 
ternal nmscles  in  thirty-three  out  of  one  hundred  and  eleven  cases. 
G.  Oram  Ring  states  that  palsies  of  the  ocular  muscles  occur  in  from 
twenty  to  twenty-five  per  cent.,  and  that  they  are  usually  found  in  the 
preataxic  stage  and  not  infrequently  exist  as  initial  symptoms.  Harris 
noted  diplopia  and  other  ocular  palsies  in  11.3  per  cent,  of  his  cases. 
Diplopia  due  to  paralysis  of  the  external  rectus  of  one  side,  Riley  found 
in  fourteen  of  sixty-one  cases.  Bramwell  noted  some  form  of  ocular 
palsy  in  16.1  per  cent.,  ptosis  in  7  per  cent.  In  seven  of  his  cases,  the 
ptosis  was  unilateral,  in  four  bilateral.  Paralysis  of  the  external  rectus 
occurred  in  6.4  per  cent.,  unilateral  in  six,  bilateral  in  four.  More  ex- 
tensive paralysis  of  the  external  muscles  occurred  in  2.5  per  cent.  Ley- 
den  and  Goldscheider  found  palsy  of  external  ocular  muscles  in  from 
40  to  50  per  cent,  of  their  cases.     (See  Chapter  V,  p.  209). 

Next,  perhaps,  in  clinical  importance  to  the  phenomena  thus  far 
considered  are  loss  or  impairment  of  the  reactions  of  the  pupil  to  accom- 
modation and  to  convergence.  Loss  or  impairment  of  these  functions 
may  exist  together  with  loss  of  the  light  reflex,  though  such  an  asso- 
ciation is  infrequent.  Further,  when  accommodation  and  convergence 
are  lost,  it  is  usually  in  connection  with  a  large  and  dilated  pupil. 
Ophthalmoplegia  interna  is  very  rare  in  tabes  and,  as  may  be  inferred, 
is  indicative  of  an  active  syphilitic  process  rather  than  tabes.  Uhthoff, 
however,  found  ophthalmoplegia  interna  in  5  per  cent,  of  his  cases  and, 
it  should  be  added,  always  unilateral.  The  latter  fact  alone  suggests 
the  special  character  of  the  lesion  at  work,  a  lesion  not  common  to 
tabetics  as  a  whole. 

It  appears  that  paralysis  of  convergence  is  somewhat  more  rare  than 
paralysis  of  accommodation.  Thus  of  thirty  cases  studied  by  the  writer, 
paralysis  of  accommodation  was  found  twice,  though  six  cases  revealed 


TABES  505 

an  impairment  of  accommodation.  The  convergence  reaction  was  absent 
but  once  and  impaired  but  twice.  Similar  results  were  obtained  by 
Rochon-Duvigneaud  and  Ileitz.  These  investigators  found  only  three 
cases  with  loss  of  convergence  in  seventy-seven.  Collins  again  found  in 
one  hundred  and  forty  cases  loss  of  accommodation  in  nine. 

Paradoxical  pupil  is  an  exceedingly  rare  symptom  in  tabes,  if  in- 
deed it  ever  really  occurs.  In  such  a  pupil,  dilatation  follows  exposure 
to  light,  while  contraction  ensues  upon  darkness.  This  phenomenon  is 
open,  however,  to  gross  misinterpretation.  Very  frequently  the  dilata- 
tion observed  is  in  reality  due  to  an  impairment  of  accommodation,  or 
more  rarely  to  a  weakness  of  the  convergence  reaction.  The  sphinc- 
ter of  the  iris  being  weak  makes,  perhaps,  an  ineffectual  attempt  to  shut 
down  and  then  suddenly  gives  way  and  dilates.  In  the  only  case  ever 
observed  by  the  writer,  involvement  of  accommodation  and  convergence 
was  clearly  present.  Piltz  (cited  by  Bumke)  reports  a  case  of  double 
optic  atrophy  (tabetic?),  in  which  upon  sudden  shading  of  one  eye, 
both  pupils  became  distinctly  narrower.  Upon  sudden  illumination,  the 
left  pupil  did  not  react  at  all,  while  the  right  contracted  slightly,  both 
directly  and  consensually.  It  would  appear  that  the  literature  fails  to 
offer  a  case  entirely  free  from  objection.     (See  also  page  493.) 

Hippus,  though  very  rare  in  paresis,  has  been  noted  in  tabes,  as,  for 
example,  in  the  case  noted  by  Hartman.  The  case  was  that  of  a  girl  of 
twenty.  The  pupils  were  otherwise  normal  and  with  normal  reactions. 
There  was  present,  however,  a  high  degree  of  optic  atrophy  with  marked 
impairment  of  vision  and  concentric  contraction  of  the  visual  fields. 

Atactic  movements  of  the  eyeballs  are  not  infrequently  observed, 
though  they  rarely  approximate  a  true  nystagmus.  It  is  probable  that 
this  symptom  when  present  in  the  lesser  degree  is  expressive  merely  of 
the  general  factor  of  incoordination,  though  when  true  nystagmus  is 
present  a  special  cause  seems  indicated.  Harris  suggests  that  it  is 
probably  due  to  nuclear  disease.  Bramwell  noted  jerking  on  extreme 
lateral  deviation  in  2.5  per  cent,  of  his  cases.  Fulton  noted  it  in  2  per 
cent,  of  his  cases.  In  two  out  of  thirty  cases  examined  by  the  writer 
nystagmus  was  present ;  in  two  atactic  movements,  moderate  in  degree, 
and  in  eleven  very  faint  atactic  movements  were  noted. 

Pain  in  or  about  the  eyeball  is  sometimes  observed,  especially  early 
in  the  history.  It  not  infrequently  assumes  the  form  of  a  periorbital 
neuralgia.  Such  a  neuralgia  may  prove  a  precursor  of  optic  atrophy,  as 
in  a  case  observed  by  Galezowski.  Areas  of  anaesthesia  may  be  found 
also  in  the  course  of  the  fifth  nerve.  Very  rarely  the  pain  assumes  the 
form  of  ocular  crises,  as  in  the  case  reported  by  Pel.     In  the  latter  the 


506  THE    EYE    AND    NERVOUS    SYSTEM 

patient  suddenly  experienced  violent  burning  and  knife-like  pain  in 
and  about  both  eyes.  There  were  present  also  cramp-like  contractions 
of  both  orbiculares,  intense  lachrymation  and  redness  and  injection 
of  the  conjunctiva?.  The  neighboring  structures  were  hyperaesthetic. 
The  crises  lasted  from  two  to  three  hours.  They  were  attributed  by 
Pel  to  a  neuritis  of  the  ciliary  nerves.  They  call  to  mind  very  forcibly 
the  attacks  termed  ophthalmic  migraine  every  now  and  then  noted  in 
paresis.     (See  Chapter  XVIL) 

Lachrymation  is  not  infrequently  observed  in  tabes  and  is  regarded 
by  some  writers  as  an  early  symptom.  It  is  not  infrequently  associated 
with  myosis.    Bramwell  found  it  in  1.2  per  cent,  of  his  cases. 

Other  pupillary  phenomena  than  those  mentioned  above  may  be  ob- 
served in  tabetics,  but  they  have  very  little  clinical  value.  Such,  for 
instance,  is  the  psychic  pupillary  reflex  of  Piltz  which  is  independent 
of  the  presence  of  a  direct  light,  the  patient's  pupil  reacting  or  con- 
tracting in  accordance  to  his  mental  conception  of  the  presence  of  a 
bright  light  or  darkness.  Similarly  in  blind  tabetics,  one  can  oc- 
casionally secure  movements  of  the  pupil  by  asking  the  patient  to  fasten 
his  mind  upon  some  object  supposed  to  be  in  the  distance  and  then 
again  upon  some  object  which  is  close  at  hand.  In  such  case  a  psychic 
reaction  to  accommodation  may  be  observed.  The  writer  has  demon- 
strated such  a  reflex  in  the  case  of  a  blind  tabetic  sailor,  who,  when  asked 
to  think  of  a  ship  barely  discernible  upon  the  horizon,  would  have  a  dila- 
tation of  both  pupils,  and  when  asked  to  think  of  reading  a  newspaper 
in  his  hand,  the  pupils  would  distinctly  contract.  The  synergic  pupillary 
reaction  of  Piltz  and  Westphal  may  also  be  observed  in  tabetics,  though 
it  has  no  special  clinical  value.  Piltz  found  that  the  pupil  contracted 
synergically  with  the  orbicularis  palpebrarum  in  43  per  cent,  of  tabet- 
ics. The  writer  found  it  present  in  only  3  per  cent,  of  his  cases  and 
in  most  of  these  it  was  slight  and  not  pronounced.  In  one  of  the 
writer's  cases  the  pupil,  instead  of  contracting,  distinctly  dilated. 

For  the  most  part  also,  the  reflex  dilatation  of  the  pupils  to  sensory 
irritation  is  found  to  be  absent  in  tabetics.  It  was  actually  present  in 
but  two  of  the  writer's  cases;  faint  dilatation  was  noted  in  a  slightly 
larger  number. 

SUMMARY. 

The  ocular  findings  of  tabes  consist  briefly  of  the  Argyll-Robertson 
pupil,  of  atrophy  of  the  optic  nerve  and  of  ocular  palsies,  the  latter 
being  usually  fugacious  and  rarely  persistent.  Any  or  all  of  these 
symptoms  may  make  their  appearance  early  and  not  infrequently  ante- 


TABES  507 

date  the  general  symptoms  of  tabes.  Impairment  of  the  light  reflex 
and  beginning  optic  atrophy  vie  with  each  other  as  to  the  frequency 
of  their  early  appearance.  Ocular  palsies  also,  as  we  have  seen,  may 
make  their  appearance  at  an  early  stage.  Again,  irregularity  of  the 
pupils  may  antedate  a  distinct  impairment  of  the  light  reflex  and  its 
occurrence  should  always  be  regarded  with  suspicion.  The  same  is 
true  of  inequality,  though  this  is  not  observed  as  often  in  tabes  as  in 
paresis. 

Myosis,  other  things  equal,  is  also  an  early  symptom ;  mydriasis,  on 
the  other  hand,  is  a  relatively  late  symptom  and  is  frequently  associated 
with  advanced  optic  nerve  atrophy  and  blindness.  At  times  it  is  also 
associated  with  loss  of  the  reaction  to  accommodation  and  of  the  reaction 
to  convergence;  ophthalmoplegias  also  are  frequently  found  associated 
with  a  mydriatic  pupil.  Finally,  loss  of  the  accommodation  and  con- 
vergence reactions,  if  present,  are  usually  observed  in  a  rather  advanced 
stage  of  the  disease.  An  initial  ophthalmoplegia  at  an  early  stage,  it 
will  be  remembered,  speaks  rather  for  a  syphilitic  lesion  than  for 
tabes. 

The  eye  symptoms  of  juvenile  tabes  on  the  whole  resemble  those  of 
the  disease  in  the  adult.  An  analysis  of  twenty-four  cases,  reported  by 
various  authors,  shows  that  an  Argyll-Robertson  pupil  and  beginning 
optic  atrophy  are  among  the  early  symptoms.  Isolated  muscle  palsies, 
strabismus  and  ptosis  are  also  present.  In  a  case  reported  by  Bloch 
there  was  inequality  of  the  pupils,  the  left  being  myotic.  The  right 
pupil  did  not  respond  either  to  light  or  accommodation,  the  left  reacted 
to  light.  In  a  case  reported  by  Janus,  a  woman  of  twenty,  the  upper 
eyelids  were  slightly  retracted,  the  left  more  than  the  right,  the  pupils 
unequal,  the  right  larger  than  the  left,  both  Argyll-Robertson  and  com- 
plete paralysis  of  the  external  rectus.  In  another  case,  a  boy  of  sev- 
enteen, there  were  gross  choroido-retinal  changes  in  the  right  eye,  to- 
gether with  vitreous  opacities.  The  right  pupil  was  sluggish  to  direct  light 
but  active  consensually.  In  another  case,  a  girl  of  fifteen,  the  changes 
were  limited  to  unequal  pupils,  the  right  being  larger  than  the  left; 
both  reacted  feebly  to  light.  In  four  out  of  six  cases  in  infantile  tabes, 
collected  by  Idelsohn,  the  Argyll-Robertson  pupil  was  present.  In  four 
cases  of  juvenile  tabes,  reported  by  Maas,  there  was  in  one  gray  optic 
atrophy  and  Argyll-Robertson  pupils  in  both  eyes.  In  another  unequal 
pupils,  loss  of  the  reflex  to  light  and  convergence  in  both  eyes  and  also 
choroido-retinitis.  In  two  others  there  were  present  unequal  pupils, 
gray  atrophy  and  Arg\dl-Robertson  pupils  upon  both  sides.  In  one  of 
these  there  was  a  coloboma  of  the  left  eye. 


508  THE    EYE    AND    NERVOUS    SYSTEM 

FUNCTIONAL   INSANITIES. 

In  the  insanities, — that  is,  the  functional  psychoses  (excluding,  of 
course,  under  this  head  paresis,  tabes  with  psychoses  and  organic  brain 
disease  generally), — the  ocular  symptoms  presented  are  relatively  unim- 
portant and  possess  but  little  clinical  value.  There  are  no  pupillary 
changes  which  are  peculiar  to  any  mental  disease.  All  efforts  that  have 
aimed  to  bring  special  ocular  symptoms  in  accord  with  individual  in- 
sanities have  failed.  Notwithstanding  it  is  of  importance  to  review 
briefly  some  of  the  studies  that  have  been  made  in  this  field  and  to  con- 
sider briefly  the  inference  that  it  is  possible  to  draw  from  them. 

The  changes  that  have  been  studied  relate  mainly  to  the  pupil. 

According  to  Dagonet,  disturbances  of  the  motility  of  the  iris  and 
inequality  of  the  pupils  may  be  found  in  both  normal  and  insane  in- 
dividuals. In  the  latter  it  is  in  the  chronic  forms  that  anomalies  are 
more  frequently  observed.  In  mania  a  dilatation  was  observed  by 
Dagonet,  a  dilatation  which  may  alternate  with  exaggerated  contrac- 
tion of  the  pupils.  Minck  found  spasmodic  myosis  in  periodic  mania 
and  in  the  puerperal  psychoses,  and  mydriasis  in  acute  mania.  Regis 
writes,  pupillary  inequality  with  variable  light  and  accommodation  re- 
flexes is  often  observed  in  psychoses  due  to  autointoxications,  but  this 
inequality  is  very  changeable  from  one  day  to  another  and  often  on  the 
same  day.  Stephani  and  Morpujo  see  a  relation  between  the  degree  of 
myosis  and  development  of  the  psychic  disturbances,  Thomson  says  that 
pupillary  immobility  exists  in  2.1  per  cent,  of  the  insane,  exclusive,  of 
course,  of  paresis.  He  found  it  once  among  one  hundred  and  eighty- 
nine  epileptics,  in  four  out  of  two  hundred  and  ninety-eight  patients 
with  systematized  delusions,  and  in  fourteen  out  of  six  hundred  and 
seventy  cases  of  other  forms  of  insanity.  In  1885,  Siemerling  examined 
seven  hundred  insane  women.  He  found  an  absence  of  the  light  reflex 
in  three  cases  of  senile  dementia,  in  one  of  syphilitic  dementia,  in  three 
tabetics  with  psychoses,  in  two  epileptics,  and  in  one  case  of  systematized 
insanity;  in  all  the  other  cases  the  light  reflex  was  unaffected.  In 
1896  he  published  a  statistical  study  concerning  nine  thousand  one  hun- 
dred and  sixty  cases.  He  noted  the  absence  of  the  light  reflex  in  twenty- 
nine  cases  of  tabes  with  psychoses,  in  seventeen  cases  of  cerebral  syph- 
ilis, in  fifteen  cases  of  alcoholic  dementia,  in  nineteen  cases  of  senile 
dementia,  seven  cases  of  paranoia,  in  four  cases  of  hysteria,  and  in  four 
cases  of  epilepsy.  The  above  facts  certainly  justify  the  inference  that 
disturbance  of  the  light  reflex  is  very  rare  among  the  insane. 

Moeli  examined  nineteen  hun.dred  patients.     He  found  immobility 


FUNCTIONAL     INSANITIES  509 

in  fifty-six,  but  excluding  organic  cases,  such  as  paresis,  the  percentage 
was  only  0.8,  Oebeker  noted  inequality  in  2  per  cent.  Mignot  says 
that,  generally  speaking,  the  percentage  will  be  2,  and,  since  the  differ- 
ence between  paresis  and  other  forms  is  so  great,  one  can  safely  con- 
sider pupillary  disturbances  as  belonging  to  paresis  more  than  to  any 
other  form  of  mental  disease.  According  to  Verga  and  Castigliani, 
no  changes  of  pupils  are  pathognomonic  of  insanity. 

Mignot  analyzed  one  hundred  and  forty-four  cases ;  paresis  was  not 
included.  He  found  inequality  in  39  per  cent.,  disturbed  light  reflex 
in  43  per  cent.,  disturbed  accommodation  in  69  per  cent.,  deformities  in 
38  per  cent.,  myosis  in  17  per  cent.,  mydriasis  in  35  per  cent.  These 
results  are  analogous  to  the  observations  of  Verga  and  Castigliani. 

In  psychoses  following  organic  lesions  of  the  brain,  Mignot  found 
marked  pupillary  changes  in  all.  Thus  in  a  case  of  maniacal  excite- 
ment, following  hemiplegia,  the  left  pupil  was  larger  than  the  right, 
the  light  reflex  was  absent  and  the  pupils  were  deformed.  In  a  case 
of  melancholia  with  mental  debility  after  hemiplegia  in  a  diabetic,  the 
right  pupil  was  larger  than  the  left  and  deformities  were  present.  In 
a  man  suffering  from  paranoia,  but  also  presenting  a  history  of  cerebro- 
spinal syphilis,  the  right  pupil  was  larger  than  the  left,  there  were 
loss  of  the  reflexes  to  light  and  accommodation  and  deformities  of  both 
pupils.  In  a  fourth  case,  a  woman  suffering  from  dementia  following 
cerebral  syphilis,  the  pupils  were  5  millimetres  in  diameter,  presenting 
incomplete  light  reflex  and  no  convergence  reaction. 

In  senile  dementia,  Mignot  found  pupillary  changes  in  all  of  the  five 
cases  examined  by  him.  Thus  one,  a  man  of  eighty,  presented  a  myosis 
of  1  millimetre  with  absence  of  the  reflexes  to  both  light  and  accommo- 
dation. A  second,  a  man  of  eighty-seven,  presented  pupils  of  2.5  milli- 
metres with  very  sluggish  reaction  to  light,  no  accommodation  reaction, 
and  deformities.  In  a  third,  a  man  of  seventy-eight,  presented  pupils  of 
2  millimetres  with  loss  of  both  light  and  accommodation  reactions.  A 
fourth,  a  woman  of  eighty-one,  presented  pupils  of  3  millimetres  with 
likewise  loss  of  light  and  accommodation  reactions.  A  fifth  case,  a 
man  of  seventy-three,  presented  pupils  of  5  millimetres  with  impaired 
light  and  accommodation  reactions.  Mignot  also  studied  the  pupillary 
changes  in  the  precocious  dementias  in  sixty-three  cases.  Of  these 
sixty-three  cases,  nineteen  had  dilatation  of  the  pupils ;  nineteen  pre- 
sented inequality.  The  light  reflex  was  examined  in  fifty-three  cases; 
four  times — i.e.,  in  7.54  per  cent. — it  was  found  lost  and  in  twenty- 
nine — i.e.,  54.71  per  cent. — it  was  found  altered.  In  the  twenty-nine 
were  included  twelve  cases  in  which  variations  of  the  light  reflex  were 


510  THE    EYE    AND    NERVOUS    SYSTEM 

noticed, — i.e.,  at  certain  times  it  was  absent,  at  other  times  diminished, 
at  other  times  normal.  This  variability  appears  to  Mignot  to  be  charac- 
teristic of  the  pupillary  disturbances  in  dementia  praecox.  In  thirty- 
nine  cases  examined  for  the  convergence  reflex  five  presented  dis- 
turbances. Finally  thirty-one  out  of  fifty-nine  cases  showed  deformity. 
In  detail  the  results  are  as  follows:  Inequality,  32.20  per  cent.;  dis- 
turbed light  reflex  62.26  per  cent.,  disturbed  accommodation  reflex  12.81 
per  cent.,  dilatation  30.15  per  cent.,  and  deformities  52.64  per  cent. 

Marandon  de  Montyel's  investigations  show  that  abnormality  of 
pupils  occurs  far  more  frequently  in  the  chronic  insane  than  in  the 
acute.  In  twenty  acute  cases  it  was  normal  in  50  per  cent,  and  abnor- 
mal in  50  per  cent.  In  fifty-seven  chronic  cases  it  was  normal  in  5.2 
per  cent,  and  abnormal  in  94.8  per  cent.  He  characterizes  as  chronic 
cases  those  that  have  a  duration  of  two  years  at  least  of  confirmed  in- 
sanity. Even  after  this  long  lapse  of  time  it  is  not  extremely  rare  for 
recovery  to  follow.  Paresis  is  doubtful  only  in  the  initial  phase  of 
the  disease;  after  a  duration  of  two  years  the  symptoms  are  so  pro- 
nounced as  to  leave  no  doubt  as  to  the  diagnosis.  Therefore  this  rule 
can  be  of  little  avail  for  the  differentiation  of  paresis.  One  out  of 
two  cases  of  acute  insanity  has  an  abnormal  pupil.  Disturbances  and 
deformities  were  noted  in  the  acute  and  chronic  cases  in  the  following 
percentages.  Thus,  in  the  acute,  disturbances  were  noted  in  15  per 
cent. ;  deformities  in  20  per  cent,  and  disturbances  and  deformities  to- 
gether in  15  per  cent.  In  the  chronic  cases,  disturbances  were  noted  in 
50.8  per  cent.,  deformities  in  8.7  per  cent,  and  disturbances  and  de- 
formities together  in  35  per  cent.  The  disturbances  are  nearly  three 
times  more  frequent  in  the  acute  than  in  the  chronic  cases.  In  detail  the 
special  symptoms  were  found  in  the  following  proportions: 

Acute.  Chronic. 

Per  Per 

cent.  cent. 

Inequality 3(15)  26(45.6) 

Myosis 0(0)  10(17.5) 

Mydriasis 0(0)  12(21) 

Deformities 7  (35)  25  (43.8) 

Alterations  of  light  reflex 2  (10)  32  (56.1) 

Alteration  of  accommodation  reflex 1(5)  20  (35    ) 

Bilateral  identical  alteration  .    0(0)  15(26.3) 

Argyll-Robertson  pupil 2(10)  19(33.3) 

Inverse  sign    1(5)  6  (10.5) 

Paradoxical  reaction 0(0)  1  (  1.7) 

Hippus 1(5)  2(3.5) 

Regarding  the  involvement  of  one  or  both  eyes,  de  Montyel  found 
that  the  deformities  were  unilateral  in  four  of  his  acute  cases  and  nine 


FUNCTIONAL     INSANITIES  511 

of  the  chronic.  In  three  acute  cases  and  in  sixteen  chronic  cases  the 
deformities  were  bilateral.  As  regards  the  inequality  of  the  pupils,  he 
found  a  difference  of  less  than  a  half  millimetre  in  two  acute  and  eigh- 
teen chronic,  from  one-half  to  one  millimetre  in  none  of  the  acute  and 
six  chronic,  one  millimetre  and  more  in  one  acute  and  two  chronic. 

In  regard  to  deformities,  de  Montyel  found  them  present  upon  one 
side  in  four  acute  and  eight  chronic  cases;  upon  the  two  sides  in  two 
acute  and  four  chronic  cases;  two  deformities  in  one  eye  in  none  of 
the  acute  and  one  of  the  chronic  cases ;  two  deformities  in  each  eye  in 
none  of  the  acute  and  none  of  the  chronic  cases ;  one  deformity  in  one 
eye  and  two  in  the  other  in  one  of  the  acute  and  two  of  the  chronic 
cases. 

These  facts  prove  that  one  may  find  in  the  acute  insanities  de- 
formities of  the  pupils  as  pronounced  as  in  the  chronic  insanities.  De 
Montyel  concluded  also  that  the  maximum  deformities  were  more  fre- 
quent in  the  acute  than  in  the  chronic  cases.  The  acute  cases  would 
furnish  a  deformed  pupil  in  the  proportion  of  one  in  seven  and  the 
chronic  one  in  more  than  a  dozen.  De  Montyel  further  analyzed  fif- 
teen of  his  acute  and  fifty  of  his  chronic  cases  to  show  the  evolution 
of  the  pupillary  changes  with  the  following  result :  He  found  the 
pupils  normal  throughout  in  40.1  per  cent,  of  the  acute  cases  and  in 
only  4  per  cent,  of  the  chronic  cases.  He  found  them  abnormal 
throughout  in  33.3  per  cent,  of  his  acute  cases  and  in  90  per  cent,  of 
his  chronic  cases.  It  was  first  normal  and  later  abnormal  in  26.6  per 
cent,  of  his  acute  cases  and  in  only  6  per  cent,  of  his  chronic  cases.  It 
was  abnormal  and  later  normal  in  none  of  the  acute  cases  and  in  none 
of  the  chronic  cases.  According  to  this  result,  40  per  cent,  of  the 
acute  cases  have  normal  pupils  as  long  as  they  are  in  the  acute  stage, 
while  only  4  per  cent,  have  normal  pupils  in  the  chronic  stage.  There- 
fore 36  per  cent,  of  normal  pupils  of  acute  cases  will  become  altered 
should  these  cases  become  chronic.  In  none  of  the  cases,  acute  or 
chronic,  examined  by  the  author  did  he  see  pupils,  once  changed,  again 
become  normal,  while  in  over  one-fourth  of  the  cases  pupils  normal  in 
the  beginning  presented  changes  later. 

Marandon  de  ]\Iontyel  also  made  a  study  of  the  pupillary  dis- 
turbances in  summer  and  winter  and  he  analyzed  seventy-four  in  sum- 
mer and  sixty-nine  in  the  winter,  a  large  proportion  of  the  latter  being 
cases  studied  by  him  in  the  preceding  summer.  In  the  summer  he 
found  25.7  per  cent,  normal  and  74.3  per  cent,  abnormal.  In  the  win- 
ter he  found  15.9  per  cent,  normal  and  84.1  per  cent,  abnormal.  The 
abnormal  changes  showed  the  following:   Changes  were  present  in  43.2 


512 


THE    EYE    AND    NERVOUS    SYSTEM 


per  cent,  in  the  siininier  and  in  42  per  cent,  in  the  winter.  Deformities 
were  present  in  12.1  per  cent,  in  the  summer  and  13  per  cent,  in  the 
winter.  Changes  and  deformities  together  were  noted  in  16.2  per  cent, 
in  the  summer  and  in  29  per  cent,  in  the  winter, — that  is,  there  was  a 
noticeable  increase  in  keeping  with  the  duration  and  the  tendency  to 
chronicity. 

De  Montyel's  conclusions  are  as  follows:  In  the  majority  of  cases 
the  pupils  are  altered  in  form  and  function  in  the  insane;  while  the 
changes  are  mostly  slight,  there  is  nevertheless  a  large  proportion  in 
which  they  are  marked.  They  are  almost  always  persistent  and  even 
increase  in  intensity.  These  facts  de  Montyel  considers  of  great  im- 
portance for  the  differential  diagnosis  of  paresis. 

Marandon  de  Montyel  has  studied  the  pupillary  disturbances  and  de- 
formities in  the  various  forms  of  insanity.  He  makes  an  analysis  of 
seventy-seven  cases.  The  pupils  were  normal  in  20  per  cent,  of  mania, 
in  10  per  cent,  of  melancholia  and  in  0  per  cent,  of  dementia,  in  0 
per  cent,  of  circular  insanity  and  in  100  per  cent,  of  insanity  with  sys- 
tematized delusions.  They  were  abnormal  in  80  per  cent,  of  mania, 
in  90  per  cent,  of  melancholia,  100  per  cent,  of  dementia,  in  100  per 
cent,  of  circular  insanity  and  in  0  per  cent,  of  insanity  with  system- 
atized delusions.     The  abnormalities  noted  were  as  follows: 


Inequality 

Myosis 

Mydriasis 

Deformities 

Changed  light  reflex 

Changed  accommodation  reflex 

Argyll-Robertson 

Reversed  sign 

Paradoxical  pupil 

Hippus 


Mania 


Melancholia 


Dementia 


36.3) 
54.4) 
18.1) 


Circular 


Per 
cent. 

2(40) 
0 

1  (20) 

2  (40) 
4 
1 
3 
0 
0 
0 


;60) 


The  unilateral  or  bilateral  character  of  the  symptoms  is  indicated 
in  the  table  on  the  next  page. 

The  general  conclusion  can  be  drawn  from  it  that  bilateral  changes 
predominate.  All  symptoms  which  show  some  tendency  to  inequality 
and  deformities  are  to  be  noted. 

As  to  the  method  of  involvement  of  the  special  reflexes  of  light  and 
accommodation,  de  Montyel  achieved  the  following  results :     In  mania 


FUI^CTIONAL     INSANITIES 


513 


the  reflex  was  at  no  time  exaggerated,  in  melancholia  he  found  it  exag- 
gerated twice,  in  dementia  never,  never  in  circular  insanity.  He  found 
it  diminished  three  times  in  mania,  eleven  times  in  melancholia,  three 
times  in  dementia  and  three  times  in  circular  insanity.     It  was  much 


Myosia 

Mydriasis 

Deformities 

Light  reflex 

Accommodation . 
Paradoxical . . .'. . 
Hippus 


1 

g 

g 

s        « 

H           3 

s       s 

S          3 

1     a 

i     a 

a           O 

o 

E3 

& 

Mania 

Melancholia 

Dementia 

0 

0 

2 

6 

0 

2 

0 

0 

3 

7 

0 

1 

3 

4 

7 

10 

1 

5 

0 

5 

0 

17 

0 

8 

0 

6 

0 

10 

0 

4 

0 

0 

0 

1 

0 

0 

0 

1 

0 

2 

0 

0 

Circular 


diminished  in  one  case  of  mania,  in  one  case  of  melancholia,  in  four  of 
dementia,  and  in  none  of  circular  insanity.  It  was  lost  in  one  case  of 
mania,  three  of  melancholia,  one  of  dementia,  and  one  of  circular  in- 
sanity. 

The  accommodation  reflex  was  not  found  exaggerated  at  any  time 
in  mania,  melancholia,  dementia,  or  circular  insanity.  It  was  dimin- 
ished in  four  cases  of  mania,  four  of  melancholia,  four  cases  of  de- 
mentia and  one  of  circular  insanity.  It  was  much  diminished  in  one 
case  of  mania,  two  of  melancholia,  in  none  of  dementia  and  in  none  of 
circular  insanity.  It  was  lost  in  one  case  of  mania,  and  in  four  of 
melancholia,  in  none  of  dementia  and  in  none  of  circular  insanity. 

De  Montyel's  conclusions  are  as  follows:  In  systematized  forms 
of  insanity  the  pupils  are  constantly  normal. 

In  dementia  the  pupils  are  constantly  abnormal.  Identical  changes 
are  present  in  both  reflexes  to  light  and  accommodation,  while  deformi- 
ties are  present  in  general  and  are  multiple ;  a  number  of  disturbances 
are  present  in  the  same  patient  and  at  the  same  time.  There  is  a  marked 
diminution  of  the  light  reflex.  Deformities  alone  are  rare.  Paradoxical 
reaction  is  not  found  and  there  is  no  hippus. 

In  circular  insanity  there  is  a  constant  abnormality  of  the  pupils. 
There  is  a  maximum  of  association  of  deformities  and  disturbances  in 
the  same  individual.  There  is  a  tendency  to  unilaterality  of  deformities 
which  in  themselves  are  slight.  There  is  no  myosis,  no  paradoxical 
reaction,  no  hippus. 
33 


514 


THE    EYE    AXD    NERVOUS    SYSTEM 


In  mania,  abnormalities  are  likewise  found  to  occur  frequently. 
There  is  a  maximum  of  a  weak  accommodation  and  a  minimum  of  a 
weak  light  reflex.  There  is  no  myosis,  no  mydriasis  and  no  para- 
doxical reaction. 

Marandon  de  Montyel  also  studied  the  influence  of  age  upon  the  dis- 
turbances and  deformities  of  the  pupils  in  the  insane.  He  made  an 
analysis  of  seventy-seven  cases.  Between  the  ages  of  twenty  and  thirty, 
16.1  per  cent,  were  normal,  while  83.9  per  cent,  were  abnormal.  Be- 
tween thirty  and  forty,  21.2  per  cent,  were  normal,  while  78.8  per 
cent  were  abnormal.  Between  forty  and  fifty,  11.1  per  cent,  were  nor- 
mal, while  98. 9  per  cent,  were  abnormal.  Between  the  ages  of  fifty  and 
sixty,  11.1  per  cent,  were  normal,  while  88.9  per  cent,  were  abnormal. 


20-30 

30-40 

40-50 

60-60 

1 

Per 
cent 

Disturbances 5  (41.6) 

Deformities '    1  (8.3) 

Disturbances  and  deformities i    4  (33.2) 

Per 
cent 

10  (  3.4) 
3  (10.3) 
9  (30.9) 

Per 

cent. 

7  (38.8) 
6  (27.7 
4  (22.2) 

Per 
cent 

10  (65.6) 
0 
6  (33.3) 

! 

Inequality 

Myosis , 

Mydriasis 

Deformities 

Light  reflex 

Accommodation 

Argyll-Robertson , 

Reversed  reaction 

Paradoxical  reaction ;    0 

Hippus I    1(  8.3) 


20-30 


Per 
cent 

6  (50    ) 
1  (  8.3) 

4  (33.2) 

5  (41.6) 
4  (33.2) 


1  (  8.3) 

2  (16.6) 


30-40 


UCUU  UCUk 

(41.8)  I  6  (33.3) 

(  3.4)  !  3  (16.6) 

(13.6)  i  2  (11.1) 


Per 
cent 

41.8 
3.4 

(13.6) 

41.3 

37.9 

20.6 

(27.2 

(10.3 

(  3-4) 


40-50 


Per 
cent 

6  (33.3) 

3  (16.6) 

2(11.1) 
9  (49.9) 
8  (44.4 
6  (33.3 

4  (22.2) 
1  (  6.6 
1  (  5.6) 
1  (  6.6) 


60-60 


Per 
cent. 

(27.7) 
27.7) 

111) 
(33.3) 
(61.1) 
(44.4) 
(38.8) 
(16.6) 


The  frequency  of  variations  in  the  light  and  accommodation  reflexes 
is  shown  in  the  following  tables : 


20-30 

30-40 

40-50 

50-60 

Per 

cent. 

Per 
cent. 

Per 
cent 

Per 

cent 

Exaggerated 

Diminished                                 

1  (25) 
3  (75) 
0 
0 

0 

7  (63.8) 
2    18.1) 
2  (18.1) 

1  (12.5) 
3  (37.5) 

2  (25    ) 
2  (26    ) 

0 

7  (63.8) 

Very  diminished 

2  (18.1) 

Lost 

2  (18.1) 

FU^^CTIONAL     INSANITIES 


515 


ACCOMMODATION    REFLEX. 


Exaggerated .... 

Diminished 

Very  diminished 
Lost 


20-80 

30-40 

40-50 

Per 
cent. 

0 
0 
0 
1  (100) 

Per 
cent. 

0 

4  (67.6) 
1  (16.2) 
1  (16.2) 

Per 
cent. 

0 

3  (51.4) 

1  (16.2) 

2  (32.4) 

50-60 


Per 

cent. 

0 

6(75  } 
1  (12.5) 
1  (12.5 


3) 


De  Monty  el  comes  to  the  following  conclusions:  Age  has  no  in- 
fluence upon  the  frequency  of  myosis,  upon  the  number  of  disturbances 
present  at  the  same  time,  upon  the  intensity  of  changes  in  the  light 
and  accommodation  reflexes,  upon  the  number,  frequency,  and  extent 
and  unilaterality  of  deformities.  Age  decreases  the  frequency  and  in- 
tensity of  the  inequality,  but  increases  the  frequency  of  changes  in  the 
light  and  accommodation  reflexes  and  the  frequency  of  the  Argyll- 
Robertson  pupil.  It  is  especially  before  thirty  and  after  fifty  that  the 
pupils  are  altered  in  the  insane. 

In  the  immense  majority  of  cases  he  found  the  pupils  abnormal; 
it  may  be  in  their  movements,  in  their  shape,  or  it  may  be  in  both  of 
these  qualities  at  the  same  time.  He  also  found  that  though  the  altera- 
tions are  usually  slight,  they  are  in  quite  a  noticeable  proportion  pro- 
nounced. Further,  they  are  almost  always  persistent  and  often  in- 
crease with  time. 

Because  of  their  detailed  character,  the  results  of  Marandon  de 
Montyel  have  in  these  pages  been  given  special  prominence,  but  not  be- 
cause the  author  indorses  them  or  accepts  their  accuracy.  Unfortunately 
they  do  not  bear  criticism.  Especially  is  this  true  in  regard  to  the 
Argyll-Robertson  pupil.  Here  his  results  must  be  wholly-  rejected.  As 
Cestan  and  Depuy-Dutemps  point  out,  mere  weakness  of  the  pupil  in  it^s 
reaction  to  light  or  variability  of  this  reaction  from  day  to  day  are  not 
the  signs  which  appertain  to  the  true  Arg^^ll-Robertson  symptom.  The 
Argyll-Robertson  symptom  either  exists  or  does  not  exist  and  is  not  sub- 
ject to  daily  variations.  If  we  apply  this  rigid  interpretation  to  the 
work  of  Mignot,  for  instance,  we  find  that  Argyll-Robertson  pupil 
was  noted  by  him  only  in  one  case  of  precocious  dementia  of  the  para- 
noid form,  and  in  two  observations  in  chronic  alcoholics.  This  gives  in 
reality  a  very  small  proportion,  besides  which  the  possibility  of  syphilitic 
infection  in  the  above  cases  was  mentioned  by  Mignot.  Mignot  very 
properly  concludes  that  disturbance  of  the  light  reflex,  such  as  is  char- 
acterized by  a  complete  and  persistent  abolition,  offers  an  extreme  prob- 
ability of  a  psychosis  with  organic  lesions. 


516  THE    EYE    AND    NERVOUS    SYSTEM 

Very  different,  on  the  other  hand,  are  the  statistics  of  Marandon  de 
Montyel.  In  a  fourth  of  his  cases,  he  claims  an  Argyll-Robertson  sign, 
and  in  a  fifth,  simultaneous  weakness  of  the  two  reflexes,  light  and 
accommodation.  These  statements  are  surprising,  for  they  are  in  com- 
plete opposition  to  current  views.  Marandon  de  Montyel  gives  the 
following  proportions  as  to  the  existence  of  the  Argyll-Robertson  sign 
in  the  insanities:  Mania,  26  per  100;  lypemania,  8  per  100;  de- 
mentia, 54  per  100;  folic  circulaire,  60  per  100;  affirming  thus  the 
extreme  frequency  of  the  loss  of  the  light  reflex  with  preservation  of 
accommodation  to  distance,  exclusive  of  all  general  paralysis. 

Astonished  at  these  results,  Cestan.and  Depuy-Dutemps  in  turn,  ex- 
amined a  large  number  of  cases  of  insanity.  The  examination  of  the 
pupil  was  always  practised  in  a  dark  chamber,  with  the  light  falling 
directly  upon  the  macular  region;  illumination  of  the  peripheral  por- 
tions of  the  retina  may  provoke  only  a  slight  pupillary  reaction  even 
in  normal  persons.  Among  other  things,  Cestan  and  Depuy-Dutempa 
point  out  the  necessity  for  rejecting  procedures  of  examination  which 
can  accidentally  put  into  play  accommodation.  If  such  precaution  be 
not  taken,  the  hand  or  the  screen  being  suddenly  withdrawn,  and  the 
eye  fixing  itself  upon  a  distant  point  of  illumination,  may  contract  only 
very  slightly  or  may  indeed  actually  dilate.  Cestan  and  Depuy-Du- 
temps noted  only  the  exact  Argyll-Robertson  sign, — that  is  to  say,  the 
complete  loss  of  the  light  reflex,  with  integrity  of  the  accommodation 
to  distance.  They  make  mention  of  the  fact  that  the  investigation  of 
this  sign  among  the  insane  is  sometimes  impossible  because  of  their  agi- 
tation, their  refusal  to  obey,  or  their  dementia  which  interferes  with  the 
careful  study  of  the  light  and  accommodation  reflexes. 

Cestan  and  Depuy-Dutemps  excluded  all  cases  of  paresis.  They 
studied  fifty  epileptics  presenting  various  mental  manifestations,  thirty 
old  men  attacked  with  cerebral  senile  lesions,  twelve  cases  of  dementia 
proBcox,  three  cases  of.  intermittent  mania,  two  cases  of  folie  a  double 
form,  five  cases  of  melancholia,  sixteen  cases  of  paronoia,  sixteen  cases 
of  secondary  dementia,  two  cases  of  neurasthenia  with  obsessions,  one 
case  of  confusional  insanity,  one  case  of  chronic  chorea  with  mental 
symptoms,  nine  feeble-minded,  nine  imbeciles,  and  six  idiots.  All  of 
the  cases  above  mentioned  were  examined  in  a  dark  chamber  by  the  pro- 
jection of  a  flame  of  light  on  the  macular  region.  In-  every  case  was 
the  reflex  to  light  preserved.  Thus  the  difference  in  the  results  of 
Cestan  and  Depuy-Dutemps  and  those  of  Marandon  de  Montyel  is 
absolute;  it  could  not  be  greater.  On  the  other  hand,  their  results 
approximate  those  of  Siemerling  as  well  as  those  of  Mignot.     It  is 


FUNCTIONAL     INSANITIES  51Y 

probable  that  if  Marandon  de  Montyel  had  tested  his  cases  according 
to  the  method  of  Cestan  and  Depuy-Dutemps,  and  in  addition  had  care- 
fully excluded  all  cases  capable  of  being  interpreted  as  tabes  or  paresis, 
or  cases  presenting  gross  lesions  of  the  optic  nerve,  the  difference  in 
results  would  not  have  been  so  glaring. 

The  results  of  Cestan  and  Depuy-Dutemps  show,  first,  that  in  men- 
tal affections,  as  in  diseases  of  the  nervous  system,  the  presence  of  the 
Argyll-Robertson  pupil  means  organic  disease,  and  secondly  the  finding 
should  suggest  a  previous  history  of  syphilis'.  How  important  this  con- 
clusion is,  we  can  only  realize  when  we  remember  that  among  the  insane 
an  antecedent  personal  history  is  often  impossible  to  obtain. 

Cestan  and  Depuy-Dutemps  maintain  that  the  Argyll-Robertson 
pupil  consists  in  the  complete  absence  of  all  contraction  to  light  with 
persistence  of  the  reaction  to  accommodation,  no  matter  what  may  be 
the  intensity  of  the  light«and  in  spite  of  the  preservation  of  retinal  sensi- 
bility. The  sign  is  not  susceptible  of  quantitative  variations  or  grada- 
-  tions  in  intensity ;  it  either  exists  or  does  not  exist.  Weakness  of  the 
light  reflex  should,  of  course,  be  noted,  and  if  such  weakness  be  asso- 
ciated with  other  organic  signs  of  the  symptom  group  of  tabes  or  of 
paresis,  it  assumes  some  value.  If  such  a  weakness  is  the  only  symptom 
present,  it  cannot  be  interpreted  as  an  Argjdl-Robertson  sign  without 
loss  of  all  precision.  The  degree  of  the  pupillary  reflexes  is,  of  course, 
variable  under  physiological  conditions,  not  to  speak  of  functional  dis- 
turbances, and  great  care  should  therefore  be  exercised  in  assigning  a 
special  value  to  a  mere  diminution  of  these  reflexes.  In  the  aged,  for 
example,  the  pupillary  reactions  are  normally  feeble.  They  can  scarcely 
be  perceived  in  the  case  of  intense  mydriasis  or  myosis  observed  conse- 
quent upon  drug  intoxication  or  upon  exhaustion  or  destruction  of  the 
cervical  sympathetic.  Myosis  of  the  pupils,  for  instance,  is  frequent 
among  the  aged,  and  by  very  reason  of  the  narrowness -and  limited  ex- 
cursion of  the  pupils  a  feeble  reaction  may  be  present.  Further,  a 
feeble  reaction  may  be  the  effect  of  an  alteration  in  the  structure  of  the 
irides,  without  any  lesion  of  the  nervous  system.  Finally  it  should  be 
remembered  that  a  diminution  of  the  light  perception  by  change  in'  the 
retina  or  by  an  optic  atrophy  is  accompanied  by  a  lessening  of  the  light 
reflex. 

Not  only  is  it  difficult  to  reconcile  the  pupillary  findings  of  Maran- 
don de  Montyel  with  common  clinical  experience,  but  especially  is  this 
the  case  in  his  grouping  of  the  insanities  according  to  his  findings.  He 
groups  the  insanities  as  follows :  First,  a  group  formed  by  dementia  and 
circular  insanity  in  which  abnormality  of  the  pupils  is  constant  in  some 


518  THE    EYE    AND    NERVOUS    SYSTEM 

form  or  other;  second,  a  group  formed  by  mania  and  melancholia,  in 
which,  Avithout  being  constant,  abnormality  of  the  pupils  is  the  most 
common  ;  and  third,  systematized  insanity  in  which  abnormalities  of  the 
pupils  are  completely  absent.  Very  curiously,  he  finds  a  constant  ab- 
normality of  the  pupils  in  circular  insanity,  while  it  is  merely  common 
in  mania  and  melancholia.  If  there  is  one  fact  which  has  been  estab- 
lished within  recent  years  and  which  is  universally  accepted,  it  is  that 
mania,  melancholia  and  circular  insanity  form  one  symptom  group,  and 
it  seems  unreasonable  to  ^ippose  that  circular  insanity,  the  phases  of 
which  are  indistinguishable  from  those  of  mania  or  melancholia,  should 
present  an  especial  peculiarity  in  regard  to  the  pupillary  reactions.  It 
is  only  in  regard  to  his  findings  in  systematized  insanity  that  Marandon 
de  Montyel  is  in  agreement  with  other  observers.  The  suspicion  is 
more  than  justified  that  his  reason  for  finding  no  abnormalities  in  the 
pupillary  reflexes  in  cases  of  paranoia  is  that -such  cases  can  be  exam- 
ined with  almost  the  same  readiness  as  can  normal  individuals,  a  fact 
which  is  certainly  not  true  of  other  forms  of  mental  disease. 

When  we  turn  our  attention  to  studies  by  other  authors,  we  find  that 
evidence  as  to  the  absence  of  characteristic  pupillary  disturbances  in 
the  psychoses  is  practically  universal.  Thus  the  studies  of  Thomsen, 
already  referred  to,  and  made  twenty  years  ago  can  be  accepted  to-day 
as  still  indicative  of  the  actual  facts  as  to  the  fixed  pupil.  Thomsen  in 
1885  studied  the  pupillary  phenomena  in  seventeen  hundred  insane. 
His  conclusions  are  that  by  far  the  greater  number  of  patients  who 
show  fixed  pupil  are  paretic ;  •  secondly,  that  the  symptom  is  of  great 
value,  because  it  occasionally  is  present  at  the  stage  of  the  disease  when 
the  other  symptoms  of  paresis  are  still  but  slightly  developed;  third, 
the  fixed  pupil  in  cases  other  than  paresis  is  met  with  (a)  in  patients 
of  advanced  age  (sixty  to  seventy  years)  and  who  present  the  symptoms 
of  senile  dementia;  (6)  in  chronic  alcoholics  in  rare  cases;  (c)  in 
other  forms  of  mental  disease  which  present  a  history  of  previous 
syphilis  or  head  injury;  (d)  under  circumstances  in  which  it  is  also 
observed  in  individuals  psychically  normal, — i.e.,  in  tabes,  multiple 
sclerosis,  cerebral  focal  disease,  brain  syphilis,  cerebrospinal  meningitis, 
tuberculous  meningitis,  and  oculomotor  palsy;  (e)  in  very  rare  cases 
without  ascertainable  cause. 

Thomsen  concludes  that  the  value  in  differential  diagnosis  of  pu- 
pillary inequality  and  pupillary  contracture  without  fixation  of  the 
pupils  is  very  slight.  Loss  of  the  light  reflex  was  present  in  47  per 
cent,  of  his  cases  of  paresis.  In  the  non-paretic  cases  only  2.2  per  cent, 
of  such  pupils  were  found. 


FUN^CTIOXAL     INSANITIES  519 

That  no  special  ocular  symptoms  are  in  accord  with  individual 
forms  of  insanity  is  proven  not  only  by  actual  experience  but  also  by  a 
brief  review  of  writings  of  various  authors.  Beginning  with  the  manic- 
depressive  group  of  insanities,  we  find  an  absence  of  all  mention  of  char- 
acteristic puj)illary  reactions  in  the  works  of  standard  authors  upon 
insanity.  Mere  variations  in  size,  dilatation  or  contraction  of  the 
pupil  are  mentioned.  Among  writers  who  have  especially  studied  the 
subject,  we  find  similar  statements.  Thus  Cowen  states  that  in  acute 
mania  with  great  excitement,  one  may  notice,  though  infrequently,  hip- 
pus.  Occasionally  also  a  wide  pupil  is  seen,  but  at  the  same  time  a 
normal  reaction.  Later,  when  exhaustion  is  pronounced,  small  pupils 
with  a  sluggish  reaction  to  light  may  at  times  be  seen.  Again,  E.  M. 
Thomson  has  described  a  case  of  acute  mania  with  dilated  pupils  and 
sluggish  reaction  to  light.  However,  in  the  great  majority  of  cases  of 
acute  mania,  the  pupils  are  quite  normal  in  size,  shape  and  reactions. 

In  acute  melancholia,  there  is  usually  nothing  abnormal,  but  in 
cases  with  marked  toxaemic  symptoms  the  pupil  tends  to  become  small 
and  reacts  sluggishly  to  light. 

Austin  has  seen  inequality  of  the  pupils  in  recurrent  insanities  and 
says  that  it  passes  from  the  right  to  the  left  when  exaltation  succeeds 
depression  (  ?).  Kitti  has  seen  inequality  as  has  also  Regis.  Of  eleven 
cases  examined  by  Mignot,  only  eight  could  be  examined  completely. 
Five  times  inequality  was  noted.  One  case  showed  a  disappearance  of 
the  inequality  during  the  quiet  period.  The  light  reflex  was  examined 
in  seven  cases.  It  was  never  lost  and  in  only  one  case  disturbed.  It 
was  normal  in  six.  Six  times  convergence  was  obtained  and  was  nor- 
mal. In  three  cases  deformities  were  noted.  Inequality  was  present  in 
33  per  cent.,  altered  light  reflex  in  28  per  cent.,  deformities  in  5  per 
cent,  and  altered  convergence  reflex  in  none.  • 

Among  cases  of  senile  melancholia,  ^linck  found  spasmodic  myosis 
in  melancholia  with  hallucinations,  while  mydriasis  existed  in  the  ter- 
minal stage.  Preston  likewise  found  mydriasis.  In  melancholia  with 
stupor,  Ball  and  Regis  found  dilated  pupils  with  loss  of  light  reflex. 
Austin  says  that  in  old  people  with  melancholia  the  right  pupil  is  often 
larger  and  more  sluggish  than  the  left.  Mairet  and  Athanassio  write 
that  loss  of  the  accommodation  reflex  and  persistence  of  the  light  reflex 
deserve  to  take  a  place  in  the  symptomatology  of  melancholia;  it  is 
quite  evident,  however,  that  abnormalities  of  accommodation  must  be 
referred  to  the  inattention  and  psychic  indifference  of  the  patient.  In 
twelve  patients  examined  by  Mignot,  the  pupillary  disturbances  pre- 
sented special  features :    four  showed  inequality,  in  two  the  inequality 


520  THE    EYE    AND    NERVOUS    SYSTEM 

was  not  constant  but  was  always  due  to  changes  on  the  same  side.  With 
the  exception  of  one  patient,  in  whom  the  light  reflex  could  not  be 
investigated  and  in  another  in  whom  the  reflex  was  normal,  the  pupils 
reacted  poorly.  The  reflex  to  convergence  was  normal  in  seven  cases. 
In  five  others  the  results  varied  at  different  examinations.  Deformities 
existed  in  eight  cases.  No  relation  was  found  between  the  state  of  the 
pupils  and  the  variations  in  the  mental  condition. 

In  paranoia,  ocular  phenomena  are  quite  constantly  absent.  This 
is  true  not  only  of  the  pupillary  reactions,  but  also  of  the  fundus. 
Thus,  J.  C.  Connell,  w'ho  studied  the  eyes  in  paranoia,  states  that  no  con- 
stant changes  were  found.  Conditions  of  depression  and  exaltation  do 
not  furnish  corresponding  appearances  in  the  fundus.  Connell  agrees 
with  the  great  mass  of  observers  when  he  states  that  when  affections  of 
the  eye  are  present,  they  are  due  to  some  material  cause  such  as  tabes, 
syphilis  or  albuminuria  in  the  course  of  which  the  insanity  develops. 
Of  five  cases  studied  by  Mignot,  only  one  had  pupillary  changes  but  this 
case  also  showed  cerebral  arterio-sclerosis.  Of  seventeen  degenerates 
affected  with  systematized  delusions  of  persecution  with  hallucination, 
there  were  found  one  case  of  inequality,  one  of  myosis  with  sluggish 
reaction  to  light,  five  with  a  diminished  light  reflex,  and  only  five  with 
deformities.  Inequality  was  present  in  54  per  cent.,  diminished  light 
reflex  in  27  per  cent.,  myosis  in  54  per  cent.,  deformities  in  36  per  cent., 
disturbances  of  convergence  in  none. 

In  delirium,  confusion  and  stumor  characteristic  changes  are  likewise 
absent.  Beyond  variations  in  the  size  of  the  pupils,  nothing  is  noted. 
This  is  true  of  even  delirium  grave  as  witness  the  case  reported  by 
Pritchard.  Occasionally,  however,  the  pupils  may  be  unequal  as  in  the 
case  of  amentia  reported  b}'  Pilcz,  in  which  the  right  pupil  was  larger 
thaji  the  left.  At  times  sluggishness  of  reaction  is  noted  as  in  the  case 
reported  by  A.  N.  V.  Johnson,  who  describes  a  case  of  acute  confusional 
insanity  following  influenza  in  a  woman  of  43.  The  pupils  were  mod- 
erately dilated  and  sluggisli  to  light  and  accommodation. 

Alcoholic  insanities  are  exceedingly  interesting  but  likewise  show 
nothing  characteristic.  Thus,  Minck  found  in  delirium  tremens,  myosis 
and  during  the  terminal  phase,  mydriasis.  Ball  and  Regis  noticed  not 
only  frequent  myosis,  but  also  inequality.  In  the  course  of  the  attacks 
of  delirium  with  hallucinations  which  lead  the  chronic  alcoholic  toward 
dementia,  Magnan  has  often  seen  inequality  of  pupils.  The  latter  he 
has  also  seen  in  degenerates  who  had  attacks  of  delirium  under  the 
influence  of  alcohol.  Krafft-Ebing  mentions  also  dilatation  of  the  pupils. 
^Mignot  found  that  of  seven  patients  affected  with  delirium  and  hallu- 


FUKCTIOKAL     IXSAXITIES  521 

cinations,  examined  at  the  beginning  or  during  the  disappearance  of 
the  symptoms,  only  two  showed  no  pupillary  disturbances.  In  four 
cases  there  was  found  inequality ;  in  one  of  them  the  inequality  disap- 
peared under  the  influence  of  abstention  from  alcohol.  In  two  patients 
the  light  reflex  was  diminished;  in  one  it  subsequently  again  became 
normal.  In  four  patients  deformities  were  observed.  Fourteen  per 
cent,  of  Mignot's  acute  cases  presented  inequality ;  fifty-seven  per  cent, 
diminution  of  the  light  reflex,  fourteen  per  cent,  deformities,  while  none 
of  them  presented  alterations  of  convergence.  Among  the  chronic  alco- 
holics studied  by  him,  one  only  had  normal  pupils ;  six  showed  inequal- 
ity; two  suffered  from  loss  of  the  light  reflex;  five  from  diminished 
light  reflex.  In  one  case  a  diminished  convergence  reflex  was  observed, 
in  another  myosis  with  inequality.  All  except  one  showed  irregular 
pupils. 

Raimann  in  1901  published  a  study  of  alcoholic  palsies  of  ocular 
muscles.  He  states  that  ocular  muscles  are  not  infrequently  paralyzed 
in  alcoholic  cases  characterized  by  Korsakow's  psychosis.  Statistically 
he  found  that  30  per  cent,  of  such  cases  presented  ocular  troubles,  such 
as  inequality  and  impaired  reaction  of  the  pupils.  A.  Westphal  noted 
paralysis  of  the  eye  muscles  in  a  case  of  Korsakow's  psychosis.  The 
case  was  that  of  a  man  of  thirty-six.  The  eyes  could  not  be  moved 
upward  or  downward.  Visual  acuity  was  diminished,  the  left  pupil 
was  larger  than  the  right.  The  light  reflex  was  sluggish  on  both  sides, 
while  the  consensual  reflex  was  also  slight.  The  ophthalmoscopic 
examination  revealed  a  slight  pallor  of  the  disks  on  the  temporal  sides. 
J.  Turner  has  made  an  analysis  of  twelve  cases  of  Korsakow's  psycho- 
sis. In  eight  cases  there  were  no  eye  symptoms.  In  four  the  following 
conditions  Avere  noted.  In  the  first,  a  woman  of  53,  the  pupils  were 
equal  and  contracted,  and  either  rigid  to  light  and  accommodation 
or  at  most  reacted  very  slightly.  In  the  second  case,  a  man  of  29, 
the  pupils  were  equal  and  reacted  to  light  with  a  restricted  range.  In 
the  third  case,  a  woman  of  39,  the  right  pupil  was  larger  than  normal. 
In  a  fourth  case,  a  woman  of  40,  the  right  pupil  was  larger  than  the  left ; 
both  jDupils  reacted  to  accommodation,  but  the  left  seemed  rigid  to  light, 
and  the  right  reacted  very  slightly  to  both  direct  and  consensual  light 
stimulation. 

Regarding  the  pupillary  reflexes  of  dementia  praecox,  definite 
knowledge  is  still  wanting.  According  to  Kraepelin,  the  pupils  are  fre- 
quently strikingly  dilated,  especially  in  the -stages  of  excitement.  This 
I  think  is  undoubtedly  in  accord  with  common  experience.  Bumke,  who 
has  records  of  thirty-three  cases,   of  which  nine  were  hebephrenics, 


522  THE    EYE    AND    KERVOUS    SYSTEM 

twenty-two  catatonics  and  two  of  the  paranoid  form,  states  that  in  all 
of  them  the  pupils  were  throughout  larger  than  normal.  They  averaged 
six  and  one-half  mm.  Rarely  was  the  pupillary  diameter  less  than  five 
and  one-half  mm.  In  two  cases,  however,  nine  mm.  were  measured. 
Inequality  of  the  pupils  was  observed  only  three  times  and  a  marked 
difference  between  the  right  and  left,  over  two  mm.,  was  only  observed 
once.  lie  observed  remarkably  rapid  changes  of  the  diameter  of  the 
pupils  in  six  of  his  cases.  Such  indeed,  for  example,  that  upon  one  occa- 
sion five  and  one-half  mm.  were  measured  and  several  days  later  under 
the  same  conditions  seven  mm.  were  measured.  Bumke  does  not  venture 
to  assign  a  reason  for  this  phenomenon.  A  difference  as  to  the  size  of 
the  pupils  does  not  seem  to  obtain  among  the  three  forms  of  dementia 
pnecox. 

Changes  in  the  light  reflex  are  doubtful  in  character.  Kraepelin, 
for  instance  is  unwilling  in  view  of  the  possibilities  of  error  in  the  exam- 
inations, to  make  definite  statements  as  to  this  point.  Bumke  believes 
as  far  as  catatonia  is  concerned,  that  if  disturbances  of  the  light  reflexes 
are  present,  they  are  always  insignificant.  He  has  frequently  received 
the  impression  that  the  pupillary  contraction  in  catatonic  cases  comes  on 
more  suddenly  and  disappears  more  suddenly  than  in  normal  cases. 
He  does  not,  however,  regard  the  symptom  as  of  value.  He  regards  the 
reflex  as  active,  but  does  not  venture  to  say  that  it  is  regularly  exag- 
gerated. 

The  accommodation  reaction  appears  to  be  entirely  undisturbed. 
This  is  also  true  as  to  the  relationship  between  the  light  and  conver- 
gence reaction.  On  the  other  hand  the  Piltz  reaction  of  the  pupil  fre- 
quently departs  from  the  normal.  It  seems  to  be  more  pronounced  and 
more  readily  observed  tjian  normally.  For  instance,  in  eighteen  of 
Bumke's  cases  and  in  all  of  the  catatonics,  a  contraction  from  two  to 
three  millimetres  was  readily  observed.  Bumke  does  not  venture  to  give 
an  explanation  of  this  condition. 

The  condition  which  Bumke  would  regard  as  typical  for  dementia 
pra^cox  is  the  absence  of  the  i)sychic  reflex,  the  reduction  of  the  pupil- 
lary motility  and  the  absence  of  the  reflex  dilatation  to  sensory  stimu- 
lation. In  hebephrenics,  the  psychic  reflexes — i.e.,  the  movements  of 
tlie  iris  in  response  to  the  play  of  concepts  and  emotions — were  always 
absent  in  cases  in  which  the  disease  had  lasted  for  a  relatively  long  time 
and  when  distinct  psychic  enfeeblement  had  become  established.  In 
TWO  cases,  Bumke  noted  the  presence  of  these  reactions  at  an  early  stage 
and  later  noted  their  disappearance.  He  states  that  he  has  never  ob- 
served a  return  of  this  reflex  in  this  form  of  dementia  prajcox. 


FUXCTIOXAL     INSANITIES  523 

In  melancholia,  abnormalities  are  very  frequent  bnt  not  constant. 
There  is  frequently  a  tendency  to  imilateral  myosis  or  unilateral 
mydriasis,  furthermore  none  of  the  patients  that  he  observed  escaped 
without  persistent  psychic  defect.  In  three  of  his  cases,  the  reflex 
dilatation  to  sensory  stimulation  was  preserved  at  least  when  very  pain- 
ful stimuli  were  employed ;  twice  such  preservation  was  temporary  and 
once  it  was  permanent.  In  one  of  these  patients,  this  reflex  dilatation 
was  preserved  in  the  beginning  of  the  disease  only.  In  another  patient, 
in  whom  it  had  been  absent,  it  appeared  when  the  psychic  symptoms 
improved. 

In  the  catatonic  form  of  dementia  praecox,  Bumke  has  never  been 
•able  to  confirm  the  existence  of  the  psychic  reflexes.  In  six  of  his 
twenty-two  cases,  however,  he  was  able  to  bring  about  a  feeble  dilatation 
of  the  pupils  through  very  painful  sensory  stimulation.  In  one  of  the 
two  cases  of  the  paranoid  form,  both  the  psychic  reflexes  and  the  reflex 
dilatation  to  irritation  was  wanting.  In  the  other  the  pupillary  psychic 
reflexes  were  wanting  while  the  sensory  stimulation  was  still  active  in 
producing  dilatation  of  the  pupil. 

Bumke  also  tested  the  action  of  cocaine,  hematropine  and  eserine  in 
dementia  praecox.  Definite  results,  however,  were  not  obtained.  Hem- 
atropine and  eserine  act  exactly  as  with  healthy  individuals.  Cocaine 
appeared  at  times  to  have  a  slight  action.  However,  nothing  definite 
can  be  said  concerning  this  point. 

It  would  appear,  then,  that  in  dementia  praecox  definite  changes  in 
the  pupillary  reactions  are  not  found  save,  first,  such  as  can  be  attributed 
to  defective  innervation, — i.e.,  the  dilatation  of  the  pupil;  and  sec- 
ond, such  changes  or  deficiencies  of  action  as  depend  on  diminished 
psychic  activity.  Definite  changes  in  the  light  and  accommodation  re- 
flexes are  not  present.  Loss  of  the  psychic  reflexes  occurs  of  course  in 
other  forms  of  dementia,  notably  in  paresis.  Here,  however,  definite 
changes  in  pupillary  reactions  make  their  appearance  either  early  or 
concomitantly. 

In  secondary  dementia,  the  pupils  are  usually  quite  normal,  though 
in  middle-aged  cases  there  is  not  infrequently  a  sluggish  reaction  to 
light  with  a  rather  small  pupil.  In  senile  dementia,  the  only  symptom 
noted  is,  as  a  rule,  a  small  pupil  with  a  distinctly  sluggish  reaction  to 
light.  Occasionally  unequal  pupils  are  found.  In  dementia  depending 
upon  gross  lesions,  such  as  tumor,  softening  or  meningitis,  the  pupillary 
reactions  may  or  may  not  be  altered.  If  changes  be  present,  they 
depend,  other  things  equal,  upon  the  site  and  the  nature  of  the  lesion 
and  these  are  so  various  that  they  cannot  be  considered  here. 


524  THE    EYE    AND    NERVOUS    SYSTEM 

SUMMARY. 

In  summarizing  the  ocular  symptoms  of  the  functional  psychoses, 
we  have  to  repeat  the  opening  paragraph  of  this  section.  First,  the  ocu- 
lar symptoms  are  relatively  unimportant  and  possess  but  little  clinical 
value ;  second,  there  are  no  pupillary  changes  which  are  peculiar  to  any 
mental  disease. 

In  all  cases  in  which  actual  and  persistent  pupillary  changes  are 
found  to  exist,  we  can  predicate  the  presence  of  grave  organic 
disease. 

Many  of  the  elaborate  studies  that  have  been  made  have  failed  of 
definite  result.  With  tlie  exception  of  the  loss  of  the  psychic  reflexes 
noted  in  dementia  prsecox  and  in  other  forms  of  dementia,  all  of  the 
elaborate  studies  as  to  pupillary  changes  in  the  functional  psychoses 
may  be  regarded  as  having  established  nothing  save  the  single  fact  that 
in  stages  of  excitement  and  stages  with  marked  diminution  of  inner- 
vation, the  pupils  become  dilated.  However,  even  this  generalization 
can  be  regarded  as  having  only  a  limited  application.  It  is  true  that 
a  dilated  pupil  is  particularly  frequent  in  the  affective  psychoses,  but 
even  here  no  rule  can  be  formulated. 

Because  of  the  etiological  relationship  between  degeneracy  and  in- 
sanity, we  meet  with — though  it  must  be  admitted  rarely — morpholog- 
ical peculiarities.  Though  rarely  present,  they  are  always  significant 
of  arrest  or  malformation.  We  may,  for  instance,  meet  with  unusual 
prominence  of  the  supraorbital  ridges,  unusual  narrowness  of  the  space 
between  the  two  eyes,  or  on  the  other  hand  unusual  width  of  this  space, 
so  that  the  eyes  seem  to  be  too  close  together  or  too  widely  separated. 
Again,  the  palpebral  fissures  are  sometimes  found  unusually  wide  or 
unusually  narrow,  or  a  little  too  straight  or  perhaps  somewhat  oblique  or 
^fongolian  in  direction.  Rarely  gross  defects,  such  as  coloboma,  may  be 
noted.  The  iris  may  also  be  congenitally  absent,  or  there  may  be  several 
openings,  or  the  pupils  may  be  abnormally  situated.  Again,  there  may 
be  irregularities  of  the  pupils  or  perhaps  an  imperforate  iris,  or  there 
may  be  anomalies  of  coloring,  such  as  patches  of  pigment,  or  albinoism 
may  be  noted.  The  pupils  may  be  congenitally  asymmetrical  and  un- 
equal. Congenital  disease  of  the  crystalline  lens  may  be  noted,  ^^^^ile 
the  anomalies  and  malformations  here  mentioned  should  be  borne 
in  mind,  it  is  on  the  whole  surprising  that  they  are  not  met  with 
more  frequently,  especially  when  we  bear  in  mind  the  obvious  re- 
lationship existing  between  some  forms  of  mental  disease  and  defective 
or^ranization. 


CHRON^IC  ALCOHOLIC  INTOXICATION 


525 


CHRONIC  ALCOHOLIC   INTOXICATION. 

The  ocular  symptoms  of  chronic  alcoholic  intoxication  consist  chiefly 
in  disturbances  of  vision.  It  has  long  been  known  that  the  chronic 
abuse  of  alcoholic  stimulants  may  result  in  a  more  or  less  pronounced 
amblyopia.  It  would  appear  that  this  amblyopia  is  dependent  upon  an 
interstitial  inflammation  followed  by  an  atrophy  of  the  nerve-fibres. 
The  early  symptoms  are  those  of  dimness  of  vision,  and  it  is  worthy  of 
note  that  the  patient  can  read  better  in  a  somewhat  subdued  light, — 
that  is,  a  light  that  is  not  very  bright.  Vision  is,  as  a  rule,  somewhat  bet- 
ter at  night.  There  is  present  a  central  scotoma  for  colors  and  for 
white.     The  impairment  of  sight  comes  on  gradually.     Visual  acuity  is 


jo5V__¥L 


Fig.  1.— Absolute  central  scotomata ;  peripheral  boundaries  of  the  field  normal  In  extent. 

(De  Schweinitz.) 


below  normal  in  both  eyes.  Occasionally,  however,  the  amblyopia 
comes  on  rapidly,  or  there  may  be  a  sudden  accentuation  of  symptom^ 
attendant  iTpon  renewed  excesses  or  upon  undue  fatigue.  According 
to  Ulithoff,  the  scotomata  are  at  first  for  red  and  green.  Terrien  states 
that  the  first  color  affected  is  green,  and  it  is  only  much  later  that  blue 
and  yellow  disappear.  The  ophthalmoscopic  examination  reveals  in  the 
beginning,  even  when  vision  is  already  much  disturbed,  a  normal  eye- 
ground,  though  in  some  cases  there  may  be  a  hyperemia  of  the  papilla. 
When,  however,  the  scotoma  for  colors  and  for  white  is  extensive,  atrophy 
of  a  part  of  the  papilla  may  be  noted.  According  to  Uhthoff,  the  changes 
which  belong  to  chronic  alcoholic  intoxication  as  well  as  those  of  tobacco 
and  arsenic,  consist  of  a  partial  retrobulbar  neuritis.  The  process  re- 
mains limited  to  the  papillomacular  bundle  of  fibres.  The  inflamma- 
tory manifestations  in  the  papilla  are  usually  slight,  while  the  secondary 


52G  THE    EYE    AND    NERVOUS    SYSTEM 

atrophy  is  usually  confined  to  the  temporal  half.  There  is  a  pallor  of 
the  temporal  portion  of  the  papilla,  or  pallor  of  the  lower  and  outer 
quadrant.  According  to  TJhthoff,  there  is  a  pallor  of  the  temporal  side 
of  the  papilla  in  63  per  cent,  of  the  cases.  A  complete  optic  atrophy 
is  very  rare. 

According  to  all  investigators — Erisman,  Leber,  Samuelsohn,  Net- 
tleship,  Vossius,  Magnan  and  Uhthoff — there  is  an  inflammation  of  the 
interstitial  connective  tissue  of  the  optic  nerve  analogous  to  that  which 
takes  place  in  the  liver  in  alcoholic  subjects.  The  fibres  of  the  macula 
seem  especially  to  suffer  from  the  alcohol.  The  change  consists  in  an 
overgrowth  of  the  interstitial  connective  tissue,  which  is  followed  by  a 
secondary  degeneration  and  atrophy  of  nerve-fibres.  Noel,  however, 
thinks  that  the  primary  change  is  after  all  to  be  sought  for  in  the  nerve- 
fibres  and  that  this  is  secondarily  followed  by  an  overgrowth  of  inter- 
stitial connective  tissue, — that  is,  he  believes  that  there  is  a  parenchy- 
matous neuritis,  with  later  a  secondary  connective  tissue  prolifera- 
tion and  atrophy. 

Uhthoff  found  0.43  per  cent,  of  alcoholic  amblyopia  in  thirty  thou- 
sand eye  cases.  Adler  found  0.42  per  cent,  in  seventy-one  thousand 
eight  hundred  and  sixty-five  cases.  It  is  remarkable  that,  in  three 
hundred  and  four  cases  of  alcoholic  amblyopia,  only  four  occurred  in 
women. 

The  percentage  of  cases  at  various  ages  Adler  found  as  follows: 
Between  thirty  and  forty,  18  per  cent. ;  between  forty  and  fifty,  46  per 
cent. ;  between  fifty  and  sixty,  26  per  cent.,  and  between  sixty  and 
seventy,  8  per  cent. 

Fleet  reports  a  case  occurring  in  a  woman  who,  after  imbibing  a 
large  quantity  of  alcohol,  became  blind.  Eight  weeks  after  the  onset  of 
the  blindness,  an  examination  revealed  the  following:  The  pupils  were 
dilated  ad  maximum;  there  was  no  reaction  to  light ;  vision  in  the 
right  eye  consisted  in  the  ability  to  count  fingers  at  a  distance  of  two 
feet,  while  in  the  left  eye  there  was  barely  a  perception  of  light;  the 
optic  nerves  were  dead  white,  while  the  blood-vessels  were  small.  Fleet 
says  that  chronic  subacute  retrobulbar  neuritis  in  persons  addicted  to 
alcoholic  beverages  is  not  uncommon,  and  we  may  expect  a  gradual  or 
passive  inflammation  of  the  optic  nerve  from  a  gradual  alcoholic  poi- 
soning. It  results  from  a  bilateral  interstitial  inflammation  of  the 
orbital  portions  of  the  optic  nerves.  The  first  symptoms  are  those  of 
failing  vision;  the  patient  can  see  better  in  a  subdued  light;  there  is 
a  ])eculiar  stare ;  the  i)upils  are  slightly  dilated ;  the  light  reaction 
is  sluggish ;    the  peripheral  vision  is  better  than  the  central,  and  there 


CHRONIC  ALCOHOLIC  INTOXICATION  527 

is  inability  to  distinguish  red  and  green  in  the  centre  of  the  visual  field. 
In  acute  retrobulbar  neuritis,  we  find  the  pupils  widely  dilated ;  there 
is  no  light  reflex,  and  there  is  no  perception  of  light. 

In  acute  alcoholism,  visual  phenomena  are  infrequent  and  with  rare 
exceptions  they  are  transient  in  duration.  Interference  with  the 
visual  field  is  almost  never  noted.  On  the  other  hand,  paresis  of  ex- 
ternal ocular  muscles  and  disturbances  of  the  pupillary  reactions  may 
be  met  with.  Now  and  then,  owing  to  damage  to  the  nuclei  of  the 
nerves  supplying  the  extrinsic  muscles,  a  permanent  paralysis  may 
result.  Blindness  has,  very  rarely,  been  noted  in  acute  alcoholism,  but 
usually  with  a  recovery  of  vision.  Noel  states  that  in  acute  alcoholic 
amblyopia  there  may  be  a  rapid  loss  of  sight  in  both  eyes  following  the 
excess  or  overindulgence.  Blindness  may  be  complete  from  the  start, 
but  sight  may  later  be  recovered.  The  pupils  may  be  much  dilated  and 
motionless. 

The  reflex  to  light  may  be  impaired  or  lost  during  profound  alco- 
holic intoxication  but  may  reappear  subsequently.  Hans  Gudden 
thinks  that  the  reaction  of  the  pupil  to  light  is  a  good  test  for  determin- 
ing the  state  of  consciousness.  The  light  reflex  may,  of  course,  be 
impaired  or  even  lost  in  chronic  retrobulbar  neuritis. 

Raimann  has  made  a  study  of  the  ocular  palsies  in  alcoholism,  and 
believes  that  the  ophthalmoplegia  occasionally  noted  is  due  either  to  a 
neuritis  or  to  a  polioencephalitis  superior.  In  two  cases  reported  by 
him,  there  was  in  the  first  a  right  ophthalmoplegia,  both  internal  and 
external ;  on  the  left  side  the  abducens  only  was  paralyzed ;  the  palsies 
gradually  disappeared.  In  the  second  case,  the  pupils  were  small  and 
of  the  Argyll-Robertson  type.  There  were  conjunctivitis,  nystagmic 
movements,  ophthalmoplegia  interna,  and  double  abducens  paralysis. 
The  autopsy  revealed  a,  hemorrhage  in  the  aqueduct  of  Sylvius.  Alto- 
gether, about  twenty-three  cases  of  polioencephalitis  superior  have  been 
reported  in  which  alcoholism  was  present.  Out  of  forty-four  cases  of 
alcoholism  studied  by  Raimann,  there  were  only  seven  with  distinct  eye- 
muscle  palsies.  Although  there  were  polyneuritis  symptoms  in  these 
cases,  Raimann  is  of  the  opinion  that  neuritis  cannot  be  considered  as 
explaining  the  palsies  of  the  eye  muscles,  but  believes  the  latter  to  be 
central  in  their  origin. 

A  case  with  autopsy,  reported  by  BonhoefFer,  corroborates  this  view. 
Of  the  extrinsic  muscles  the  external  rectus  is  the  most  frequently 
affected.  In  slight  cases  there  is  usually  merely  a  weakness  of  this 
muscle.  Nystagmic  movements  may  be  noted  when  the  patient  at- 
tempts to  look  toward  the  side  of  the  paralyzed  muscle.     Next  in  fre- 


528  THE    EYE    AND    NERVOUS    SYSTEM 

quency  is  involvement  of  the  internal  rectus.  Ptosis  also  is  not  infre- 
quently noted.     These  palsies  usually  come  on  suddenly. 

Bonhoeffer  has  reported  a  case  of  alcoholism  with  focal  cortical  symp- 
toms in  which,  before  death,  disturbances  in  the  eye  muscles  occurred. 
Jolly  has  reported  twenty-one  cases  of  alcoholism,  of  which  two  pre- 
sented paresis  of  eye  muscles. 

Thomsen  examined  six  hundred  and  seventy  alcoholics.  He  found 
no  light  reaction  on  either  side  in  nine  cases,  and  in  one  case  loss  of 
the  light  reaction  upon  one  side.  There  was  sluggish  reaction  on  both 
sides  in  fifty-seven  cases  and  on  one  side  in  eleven  cases.  In  12  per 
cent,  of  his  patients  there  were  pupillary  disturbances.  Siemerling 
found,  in  twenty-nine  cases  of  alcoholism  in  women,  only  one  case  of 
loss  of  light  reflex.  Moeli,  in  a  series  of  cases,  found  only  four  of  per- 
manent and  three  of  temporary  loss  of  the  light  reaction. 

Raimann's  experience,  based  upon  two  hundred  and  seven  cases  of 
alcoholism,  yields  twenty-five  per  cent,  of  disturbances  of  the  innerva- 
tion of  the  eye  muscles.  Among  all  of  the  eye  symptoms,  pupillary 
disturbances  were  the  most  frequent.  Myosis,  mydriasis,  or  slow  and 
unequal  reaction  of  the  pupils,  may  be  present.  Pupillary  involvement 
may,  of  course,  be  present  at  the  same  time  that  the  extrinsic  muscles 
are  involved.  Raimann  believes  that  alcoholic  ophthalmoplegias  are 
almost  all  of  central  origin.  The  possibility  of  a  polyneuritis  of  the 
nerves  supplying  the  eye  muscles  must,  however,  be  admitted.  The 
prognosis  of  alcoholic  ophthalmoplegias  is  uncertain. 

As  might  be  expected,  amblyopia  not  infrequently  occurs  in  per- 
sons who  are  at  one  and  the  same  time  the  victims  of  chronic  alcoholic 
and  chronic  tobacco  poisoning,  and  in  such  cases  it  may  be  difficult  and 
indeed  impossible  to  separate  the  two  conditions.  It  is  well  known  that 
both  alcohol  and  tobacco  may  independently — and  this  is  true  also  of 
arsenic — cause  an  amblyopia  with  central  scotomata  and  temporal  dis- 
colorations  of  the  papilla,  while  the  peripheral  vision  is  intact. 

OCULAR   SYMPTOMS    OF   LEAD    INTOXICATION. 

Blindness,  as  a  result  of  lead  intoxication,  has  long  been  known, 
though  it  was  not  until  Grisole,  in  1835,  and  shortly  after  Tanquerel 
des  Planches,  in  1839,  described  the  cases  studied  by  them  that  the  role 
of  lead  in  the  production  of  blindness,  together  with  lead  colic  and  other 
general  symptoms,  was  properly  appreciated. 

As  in  the  case  of  alcohol,  chronic  lead  poisoning  may  give  rise  to 
both  amblyopia  and  disturbances  of  muscle  innervation.  The  eye  symp- 
toms are,  however,  always  (or  nearly  always)  preceded  by  the  general 


LEAD   INTOXICATION 


529 


symptoms  of  lead-poisoning.  The  amblyopia  presents  typical  central 
scotomata.  At  the  same  time,  there  may  be  concentric  contraction  o£ 
the  fields  for  form  and  colors.  Not  infrequently  the  scotoma  assumes 
the  form  of  a  hemianopsia. 

Actual  organic  changes — such  as  optic  neuritis,  neuroretinitis,  optic 
atrophy,  or  changes  in  the  vessels  (perivasculitis) — may  supervene. 
Exceptionally  the  amblyopia  is  recovered  from  and  is  then  probably  due 
to  an  anaesthetic  effect  of  the  poison,  rather  than  to  organic  changes. 
Ophthalmoscopic  appearances,  as  may  be  surmised,  vary  from  nega- 
tive findings  or  mere  vascularity  of  the  nerve  head  to  the  decided 
organic  changes  just  detailed.  Of  sixty-four  cases  analyzed  by  de 
Schweinitz,  thirteen  presented  optic  neuritis,  four  neuroretinitis  and 
seventeen  optic-nerve  atrophy.      In  seventeen  cases  of  the  sixty-four, 


Slue 

■Ked 

Green 

^^^^^Absolute  defect 

Fig.  2.— Field  of  vision  from  Uhthoff's  case  of  lead-amblyopla  with  ophthalmoscopic  appearances 
resembling  in  toxica  tion-amblyopia  (temporal  half  of  papilla  discolored).  Normal  form  field  and  abso- 
lute central  scotoma.    (De  Schweinitz.) 


blindness  was  noted  vi^ithout  description  of  the  ophthalmoscopic  appear- 
ances. De  Schweinitz  thinks  that  no  doubt  in  many  of  these  atrophy 
or  neuritis  was  present,  and  he  regards  Stood's  result  of  fifty  per  cent, 
of  organic  nerve  change,  based  on  a  study  of  thirty-four  cases,  as  not 
too  high.  Some  authors — for  example,  Hirschberg  and  Knies — con- 
sider lead  amaurosis  identical  with  uremic  amaurosis.  Most  writers 
regard  the  influence  of  lead  upon  the  optic  nerves  as  direct.  Others, 
like  Oliver,  regard  the  action  of  lead  upon  the  retina  as  anaesthetic; 
while  others  still,  such  as  Jaccoud  and  Weber,  lay  stress  upon  the  cir- 
culatory disturbances.  Elschnig  has  reported  a  case  in  which  the  cir- 
culation of  the  retina  seems  to  have  been  at  fault ;  there  was  an  arterial 
spasm  and  the  blindness  was  attributed  to  the  ischa?mia. 

A  clear  conception  of  the  visual  and  ophthalmoscopic  findings  is 
afforded  by  the  following  illustrative  cases  taken  from  the  literature : 
34 


530  THE    EYE    AND    NERVOUS    SYSTEM 

Westphal,  in  1888,  reported  a  case  in  which  there  were  hemianop- 
sia, cataract  of  one  eye,  and  optic  neuritis  in  the  other.  Hertel,  in 
1890,  reported  a  case  in  which  there  was  a  left  homonymous  hemianop- 
sia, narrowness  of  the  retinal  arteries,  and  sluggish  reaction  of  the 
pupils.  Elschnig  reported,  in  1898,  a  case  of  temporal  hemianopsia. 
Bihler  reported,  in  1899,  the  case  of  a  compositor,  thirty-nine  years  of 
age,  in  whom  in  the  left  eye  the  visual  field  of  the  nasal  side  was  lost 
and  in  the  right  eye  the  temporal  visual  field.  The  hemianopsia  was 
partial,  but  there  was  complete  hemiachromatopsia.  There  was  slight 
nystagmus  and  the  pupils  were  narrow  on  both  sides.  Taylor,  in  1898, 
reported  the  case  of  a  woman,  twenty  years  of  age,  in  whom  there  was 
failing  sight  with  marked  central  scotomata  in  both  eyes.  There  was 
white  optic  atrophy  on  the  left  side,  while  the  right  papilla  was  ap- 
parently normal;  there  was  here  an  amblyopia  without  obvious  tissue 
change.  In  two  cases  of  lead  poisoning,  reported  by  de  Schweinitz, 
there  was  partial  optic-nerve  atrophy  together  with  central  scotomata. 
In  the  first  case,  that  of  a  house  painter,  aged  thirty-five,  vision  was  re- 
duced in  both  eyes.  The  disks  were  nearly  circular,  with  a  sharp  cen- 
tral excavation ;  there  was  moderate  chorioidal  disturbance  in  the  cir- 
cumference ;  there  were  central  scotomata  with  contraction  of  the  visual 
field  of  the  temporal  side  of  the  right  eye.  In  the  left  eye  the  visual 
field  was  normal;  there  were  no  muscle  palsies.  In  the  second  case, 
also  a  painter,  aged  fifty-nine,  besides  marked  impairment  of  vision,  it 
was  noted  tliat  both  optic  nerves  were  grayish,  the  vessels  were  small 
and  there  were  present  central  scotomata  for  red  and  green.  Later,, 
there  was  a  general  grayish-white  atrophy  and  a  marked  perivasculitis,, 
with  marked  concentric  contraction  of  the  visual  fields,  especially  upon 
the  right  side,  and  decided  contraction  of  the  red  field  and  blindness 
for  green. 

Eye  symptoms  due  to  lead  poisoning  are  surprisingly  rare.  Tan- 
querel  des  Planches  found  only  twelve  cases  of  amblyopia  in  one  thou- 
sand two  hundred  and  seventeen  cases  of  lead  poisoning.  According  to 
de  Schweinitz,  also,  lead  amblyopia,  compared  with  the  number  of 
cases  of  chronic  lead  poisoning,  is  infrequent.  Packard  examined  forty- 
eight  workers  in  lead ;  thirty-tliree  of  them  showed  symptoms  of  the 
toxic  influence  of  lead,  but  no  case  was  found  of  amblyopia,  traceable 
to  lead.  Again,  lead  amblyopia  is  rare  in  comparison  with  the  whole 
number  of  ocular  diseases,  although  it  is  difiicult  to  give  exact  figures. 
De  Schweinitz  states  that  among  more  than  twelve  thousand  cases  of 
ocular  disorders  recorded  at  the  Jefferson  Hospital  and  the  Philadel- 
phia Polyclinic  during  five  years,  only  three  cases  of  optic-nerve  or 
retinal   changes   attributable    to   lead    were   found    and   of   these   two 


LEAD    1NT0X1CATI0:Ni  531 

were  doubtful.  Further,  the  number  of  amblyopias,  among  the  total 
number  of  toxic  amblyopias,  is  small.  Uhthoff  reports  two  hundred 
and  four  cases  of  retrobulbar  neuritis  among  thirty  thousand  patients ; 
of  these  one  hundred  and  thirty-eight  were  toxic  in  origin,  but  only 
one  was  due  to  lead.  The  ocular  manifestations  of  chronic  lead  poison- 
ing may  consist  of  transient  amblyopia  due  to  the  anaesthetic  effect 
of  lead  upon  the  optic  nerve  and  retina,  or  of  a  permanent  amblyopia 
due  to  retrobulbar  neuritis.  The  latter  may  terminate  in  permanent 
atrophy,  optic  neuritis,  or  neuroretinitis  specifically  due  to  lead  and 
which  last  may  in  turn  be  followed  by  optic-nerve  atrophy.  Finally, 
amblyopia  may  be  due  to  a  vasculitis  of  the  retinal  vessels. 

The  least  knoAvn  of  the  ocular  symptoms  due  to  lead  poisoning  are 
the  disturbances  of  the  movements  of  the  eyes  and  the  reactions  of  the 
pupils.  De  Schweinitz  states  that  chronic  lead  poisoning  is  a  well  rec- 
ognized cause  of  paralysis  of  the  external  ocular  muscles.  According 
to  Schroeder,  the  abducens  is  the  one  most  frequently  involved,  a  con- 
dition which  is,  perhaps,  analogous  to  the  palsies  of  the  extensors  of 
the  extremities. 

Elschnig  has  described  seventy-six  cases  of  palsies  of  the  motor 
nerves  of  the  eye.  Renaut,  Galezowski,  Dejerine-Klumpke,  Manna- 
berg,  Lilienfeld,  Lagrange,  and  lately  Aurant  and  Burnot,  have  de- 
scribed double  palsies  of  the  external  recti.  x\ccording  to  Elschnig, 
there  are  two  varieties  of  ocular  palsies  occurring  in  the  course  of  lead 
intoxication :  First,  palsy  of  central  origin,  associated  with  vertigo, 
headache,  and  vomiting,  the  cases  terminating  in  rapid  death ;  second, 
palsies  of  peripheral  origin.  The  latter  are  more  frequent,  less  rapid  in 
their  onset,  and  are  less  fatal.  Ptosis,  strabismus  and  diplopia  may  be 
present.  The  third  nerve  may  be  involved  in  its  entirety,  or  the  paraly- 
sis may  affect  only  one  of  its  branches.  Isolated  pupillary  and  accom- 
modation disturbances  do  not  frequently  come  under  observation.  Elsch- 
nig twice  found  unilateral  palsy  of  the  sympathetic,  narrowness  of  the 
palpebral  fissure,  and  sluggish  light  reaction.  In  one  case  he  found 
symptoms  of  irritation  of  the  sympathetic.  In  a  case  reported  by  Red- 
lich  there  was  pupillary  rigidity  to  both  light  and  convergence,  while 
vision  was  preserved.  This  finding,  however,  mijst  be  regarded  as  ex- 
ceedingly rare, — so  rare,  indeed,  as  to  be  open  to  doubt.  Crooke  has 
placed  on  record  a  case  of  lead  poisoning  accompanied  by  delirium, 
coma,  and  convulsions,  followed  by  blindness.  In  this  case  the  pupils 
were  at  first  contracted  and  later  dilated.  According  to  de  Schweinitz, 
the  iris  movements  vary  with  the  condition  of  the  optic  nerve  and  retina 
from  absolute  immobility  to  normal  reaction. 


CHAPTER    XIII. 

AMAUROTIC   FAMILY    IDIOCY   AND   CEREBRAL    PALSY    OF 

CHILDHOOD. 

By  B.  SACHS,  M.  D. 

AMAUROTIC  FAMILY  IDIOCY. 

The  above  title  was  given  by  me  to  a  rare  disease  affecting  several 
members  of  the  same  family,  and  characterized  by  a  distinct  lack  of 
mental  development,  by  a  progressive  weakness  of  all  the  muscles  of  the 
body,  and  by  a  defect  in  vision  (associated  with  changes  in  the  macula 
lutea  and  optic  nerve  atrophy)  terminating  in  complete  blindness.  The 
disease  is  generally  fatal,  the  children  dying  as  a  rule  in  a  condition  of 
complete  marasmus  before  the  end  of  the  second  year  of  life. 

History. — In  1881  Waren  Tay  described  a  case  presenting  "  sym- 
metrical changes  in  the  region  of  the  yellow  spot  in  each  eye  of  an 
infant.  The  child  was  twelve  months  old.  It  was  deficient  in  holding 
up  its  head  or  moving  its  limbs.  There  was  weakness  but  no  absolute 
paralysis  of  any  part.  Its  cerebral  development  was  slow  and  poor.  At 
the  first  examination,  March  7,  1881,  the  optic  disks  were  apparently 
healthy,  but.  in  the  region  of  the  yellow  spot  of  each  eye  there  was  a  con- 
spicuous, tolerably  diffuse,  large  white  spot  more  or  less  circular  in 
outline,  and  showing  at  its  centre  a  brownish-red,  fairly  circular  spot 
contrasting  strongly  with  the  white  patch  surrounding  it.  This  central 
spot  did  not  look  at  all  like  a  hemorrhage,  nor  as  if  due  to  a  pigment, 
but  seemed  a  gap  in  the  white  patch,  through  which  one  saw  healthy 
structures."  The  author  likened  these  appearances  to  those  one  is 
familiar  with  in  cases  of  embolism  of  the  central  artery  of  the  retina. 
He  believed  the  changes  in  the  retina  to  be  "possibly  congenital." 
Five  months  later  another  examination  was  made,  showing  that  the 
disks  had  become  atrophied,  but  that  the  changes  in  the  macula  lutea 
were  the  same  as  before.  In  the  same  family,  according  to  Waren 
Tay's  later  reports,  three  similar  cases  had  occurred,  each  one  of  the 
children  presenting  ocular  symptoms  and  exhibiting  physical  condi- 
tions that  were  similar  in  all  respects,  and  all  three  dying  before  the 
age  of  two  years.  This  peculiar  ophthalmoscopic  finding  was  noted  by 
Magnus,  Goldzieher,  Wadsworth  of  Boston,  Ilirschberg  of  Berlin,  and 
H.  Knapp. 
532 


AMAUROTIC  FAMILY  IDIOCY  533 

In  1887,  without  any  knowledge  of  the  cases  described  by  the 
oculists,  I  published  the  history  and  the  post-mortem  record  of  a  patient 
suffering  from  what  aj)peared  to  be  a  peculiar  form  of  idiocy  associated 
with  blindness.  The  family  character  of  the  affection  was  not  evident 
until  a  sister  of  my  first  patient  became  similarly  affected.  In  still 
another  family  I  saw  another  instance  of  this  affection,  and  received 
the  history  of  three  other  children  who  had  been  afflicted  with  and  had 
died  of  this  disease. 

Kingdon,  of  Nottingham,  called  attention  to  the  fact  that  the  rare 
condition  reported  by  the  oculists  was  part  of  the  disease  which  I  had 
described.  In  1894  Carter  collected  all  cases  of  this  disease  known  up 
to  that  time,  and  in  1896  I  was  able  to  give  a  list  of  nineteen  cases  of 
which  eight  had  come  to  my  own  notice.  Since  the  publication  of  this 
last  paper  other  cases  of  this  sort  have  been  described  and  published 
by  American  and  some  European  writers.  In  Europe  cases  have  been 
published  by  Kingdon  and  Russell,^  Higier,  Falkenheim,  Shaffer,  Frey, 
and  others.  In  1901  Falkenheim  analyzed  a  series  of  sixty-four  cases 
and  since  that  time  others  have  been  reported  both  here  and  abroad. 

Symptomatology. — As  a  rule,  the  children  affected  with  this  disease 
are  born  at  full  term  and  apparently  in  perfect  health.  They  do  well 
until  the  first  three  to  six  months  of  life,  when  they  become  listless  and 
apathetic,  move  their  limbs  very  little,  and  show  the  first  signs  of  visual 
disturbance  which  ultimately  leads  to  blindness.  The  child  is  not  able, 
as  the  months  go  on,  to  hold  up  its  head  or  to  sit  up.  Its  muscles  are 
either  flaccid  or  spastic ;  the  reflexes  are  normal,  a  trifle  subnormal,  or 
exaggerated.  In  some  cases  there  is  an  unusual  sensitiveness  to  touch 
and  to  sound  (hyperacusis),  the  child  being  startled  by  the  slightest 
noise  occuTring  in  the  room.  Convulsions  are  present  in  some  cases 
but  are  not  an  integral  symptom  of  the  disease.  All  the  functions  of  the 
body  are  in  a  low  state  of  activity.  The  children  are  subject  to  frequent 
bronchial  attacks  and  soon  show  gastro-intestinal  disturbances.  An 
examination  of  the  fundus  reveals  the  peculiar  condition  so  well  de- 
scribed by  Tay.  (See  Fig.  1).  There  is  a  gradual  increase  of  all  the 
symptoms,  the  mental  defect  becomes  more  and  more  noticeable,  the 
palsy  more  extreme,  complete  blindness  is  established  and  the  child 
gradually  lapses  into  a  condition  of  marasmus  in  which  it  dies  as  a  rule 
before  the  end  of  the  second  year.  The  chief  symptoms  may  be  summed 
up  as  follows:  (1)  Mental  impairment  observed  during  the  first  months 
of  life  and  leading  to  absolute  idiocy;  (2)  paresis  or  paralysis  of  the 


^  Russell  has  used  the  designation  "Infantile  Cerebral  Degeneration;"  the  term 
is  unsatisfactory  and  wholly  inadequate. 


534  THE    EYE    AND    NERVOUS    SYSTEM 

greater  part  of  the  body,  and  this  paralysis  may  be  either  flaccid  or 
spastic;  (3)  the  reflexes  may  be  normal,  deficient  or  increased;  (4)  a 
diminution  of  vision  terminating  in  absolute  blindness  (the  cherry-red 
spot  in  the  region  of  the  macula  lutea  and  later  optic  nerve  atrophy)  ; 
(5)  marasmus  and  a  fatal  termination,  as  a  rule,  before  the  age  of  two 
years;  (6)  the  occurrence  of  tlie  affection  in  several  members  of  the 
same  family. 

In  some,  but  not  in  all  of  the  cases,  nystagmus,  strabismus,  and 
hyperacusis  are  added  to  the  above  cardinal  symptoms.  A  loss  of  the 
sense  of  hearing  was  noted  in  two  cases.  Falkenheim  refers  to  im- 
pulsive laughter,  but  I  have  not  been  able  to  persuade  myself  that  this 
had  occurred,  in  any  except  in  the  one  case  described  by  him.  Some 
slight  variations  will  naturally  occur  in  the  degree  and  character  of 
paralysis.  The  changes  in  the  macula  lutea  are  so  striking  that  they 
constitute  a  very  important  symptom  of  the  disease.  But  it  is  to  be 
noted  that  the  disease  can  be  diagnosticated  even  in  the  absence  of 
this  one  symptom,  and  that  in  some  of  the  cases  the  general  cerebral 
symptoms  are  developed  some  months  prior  to  the  retinal  changes. 
Koller  has  reported  a  case  in  which  at  the  first  examination  the  changes 
in  the  macula  lutea  were  not  in  evidence  although  they  appeared  later 
on,  and  in  Higier's  case  the  optic  nerve  atrophy  was  much  more  pro- 
nounced than  the  changes  in  the  macula  lutea. 

Etiology. — The  causes  underlying  this  disease  are  still  obscure.  In 
some  of  the  families  there  has  been  blood  relationship  between  the 
parents,  in  many  others  no  such  relationship  existed.  Injury  to  the 
mother  during  pregnancy  has  been  noted  in  several  of  the  cases  and  the 
tendency  to  mental  derangement  in  the  families  of  one  or  both  parents 
is  also  suggestive  of  another  possible  factor.  The  family  predisposition 
is  evident  from  the  fact  that  twenty-eight  cases  have  occurred  to  my 
knowledge  in  fifteen  families.  Carter  was  the  first  to  call  attention  to 
the  fact  that  all  of  the  cases  reported  have  occurred  among  Hebrews, 
and  even  at  this  day  I  can  say  that  I  know  of  no  indubitable  case  occur- 
ring in  others.  The  racial  feature  of  the  disease  is  all  the  more  astound- 
ing, because  other  diseases  to  which  it  must  necessarily  be  more  or  less 
closely  allied  have  been  observed  and  recorded  among  all  races  and  all 
nationalities. 

The  absence  of  syphilis  has  been  distinctly  noted  in  most  of  the 
histories.  This  is  a  matter  of  some  importance  for  there  is  always  a 
tendency  to  attribute  a  mysterious  family  affection  to  this  hereditary 
factor,  and  furthermore,  the  disease  bears  some  resemblance  to  specific 
disorders  which  are  characterized  by  various  forms  of  dementia  and  by 
ocular  disturbances    (Batten).       Starr  was  mistaken   in  quoting  the 


AMAUROTIC  FAMILY  IDIOCY  535 

present  writer  as  favoring  the  syphilitic  etiolog}^  in  this  disease.  In 
passing  we  may  take  note  of  the  fact  that  there  is  an  hereditary  optic 
nerve  atrophy  occurring  later  in  life  which  is  due  in  many  instances  to 
hereditary  syphilis,  and  with  this  optic  nerve  affection  the  present  dis- 
ease might  possibly  be  confounded. 

The  disease  runs  its  course,  as  was  intimated  above,  in  a  little  less 
than  two  years.  I  have  encountered  but  a  single  prominent  exception 
to  this  rule,  and  in  this  instance  the  child  had  attained  the  age  of  five 
and  a  half  years  when  I  examined  it,  and  it  is,  so  far  as  I  know,  still 
living.  The  characteristic  symptoms  of  the  malady  were  present,  and 
the  same  symptoms  had  been  presented  by  two  or  three  other  members 
of  this  family.  Spiller  threw  out  the  hint  that  the  symptoms  and  the 
anatomical  changes  may  vary  somewhat  if  some  of  these  children  should 
live  beyond  the  usual  term  of  years.  Many  of  these  children  are  ex- 
tremely well  nourished  at  birth  and  for  the  first  few  months  of  life. 
By  degrees  they  lapse  into  a  state  of  marasmus  to  which  they  slowly 
succumb.  I  wish  to  insist  on  marasmus  as  a  very  important  symptom 
of  the  condition,  and  the  emaciation  observed  in  such  cases  is  so  extreme 
that  it  unfortunately  makes  of  the  child  a  most  disagreeable  object. 
One  writer  has  referred  to  sudden  death  as  characteristic  of  the  disease, 
but  the  very  opposite  of  this  has  been  observed  in  my  experience. 

Pathological  Anatomy — In  my  first  case  of  palsy,  in  1887,  the  outer 
surface  of  the  brain  exhibited  abnormalities  which  we  were  accustomed 
to  associate  with  brains  of  inferior  development.  There  was  a  con- 
fluence of  the  central  and  Sylvian  fissures  and  a  complete  exposure  of 
the  island  of  Reil.  There  was  unusual  hardening,  the  knife  grating 
on  removing  a  small  section  of  the  cortex.  On  microscopical  examina- 
tion the  most  important  changes  were  found  in  the  cortex,  and  sections 
were  taken  from  the  frontal  lobes,  motor  areas,  from  the  base  of  the  3d 
convolution,  from  the  first  temporal,  and  from  a  part  of  the  apex.  The 
same  changes  were  found  practically  throughout  the  cortex.  It  was 
possible  to  make  out  the  various  layers  of  cells,  but  examination  and  a 
most  careful  search  on  removal  of  the  sections  showed  that  there  were 
scarcely  half  a  dozen  pyramidal  cells  which  presented  anything  like 
their  normal  appearance.  The  contour  of  the  cells  was  either  rounded 
or  elongated,  and  the  cell  protoplasm  exhibited  every  possible  change 
such  as  we  note  in  degenerated  cells.  In  some  cells  the  nucleus  and 
necleolus  were  entirely  wanting  or  were  relegated  to  the  margin  of  the 
cell.  In  later  examinations  further  changes  had  been  noted.  Hirsch 
was  the  first  to  point  out  that  these  same  cellular  changes  occurred  not 
only  in  the  gray  matter  of  the  cortex,  but  in  the  gray  matter  of  the 


536  THE    EYE    AND    NERVOUS    SYSTEM 

entire  central  nervous  system,  in  the  cortex  of  the  brain,  in  the  basal 
ganglia,  in  the  gray  matter  of  the  spinal  cord,  and  even  in  the  spinal 
ganglion.  I  was  able  to  corroborate  this  finding  by  a  later  examination 
in  another  case  of  my  own,  and  in  1903  I  felt  warranted  in  declaring 
amaurotic  family  idiocy  to  be  a  disease  chiefly  of  the  cortex  and  of  the 
gray  matter  of  the  entire  central  nervous  system.  These  findings  have 
received  further  and  most  satisfactory  corroboration  in  a  careful  report 
recently  published  by  Spiller.  Kingdon,  Schaffer,  and  others,  including 
nirsch  and  myself,  have  found  degeneration  in  the  pyramidal  tracts, 
yet  this  degeneration  does  not  seem  to  be  as  marked  as  we  would  expect 
it  to  be  from  the  very  considerable  involvement  of  the  gray  matter. 

Holden,  Mohr,  Treacher  Collins,  Schumway,  and  Buchanan  have 
examined  the  eyes  post-mortem.  There  is  a  degeneration  of  the  ganglion 
cells  of  the  retina,  and  of  the  nerve  fibres  of  the  optic  nerves  and  tracts. 
There  is  some  doubt  as  to  the  existence  of  an  cedema  in  the  region  of 
the  macula. 

The  question  has  arisen  whether  the  disease  is  invariably  congenital, 
or  whether  it  is  an  acquired  disease.  The  latter  is  claimed  to  be  the  case 
by  Hirsch,  who  would  place  it  in  the  category  of  toxic  affections ;  but, 
as  I  have  stated  in  several  communications,  it  is  hard  to  accept  such  a 
view  for  any  family  disease,  and  the  fact  that  it  cannot  be  attributed 
to  the  influence  of  the  mother's  milk  is  proved  by  the  one  circumstance 
that  several  of  my  patients  were  raised  by  strange  wet  nurses.  There 
is  no  doubt  in  my  own  mind  that  the  disease  is  developed  as  the  result 
of  a  congenital  defect.  The  nervous  system  of  such  a  child  is  able  to 
perform  tolerably  normal  functions  until  the  age  of  four  or  six  months, 
then  it  is  no  longer  able  to  meet  the  strain  put  upon  it,  and  an  active 
process  of  degeneration  sets  in.  This  degeneration  is,  to  my  thinking, 
a  natural  result  of  an  arrest  of  development.  Gowers  has  claimed  very 
much  the  same  for  some  of  the  scleroses  that  have  developed  very  much 
later  in  life,  and  the  fact  that  the  first  symptoms  of  amaurotic  family 
idiocy  become  evident  about  six  months  after  birth,  does  not  militate 
against  the  theory  of  a  congenital  defective  development. 

Amaurotic  family  idiocy  should  not  be  regarded  as  an  isolated 
though  interesting  disease,  but  all  the  symptoms  and  anatomical  findings 
suggest  a  relationship  to  other  hereditary  family  affections. 

Treatment. — Unfortunately,  little  can  be  said  in  favor  of  any  thera- 
peutic measures.  In  several  cases  under  my  observation  I  have  hoped 
against  hope  that,  by  most  careful  nursing  and  feeding,  some  of  the 
children  thus  afflicted  might  escape,  but  up  to  the  present  time  all  such 
efforts  have  been  entirely  fruitless.     Kingdon  has  suggested  the  use  of 


INFANTILE  CEREBRAL  PALSIES  537 

thymus  extract,  but  he  is  not  yet  able  to  state  that  any  advantage  has 
been  gained  thereby.  It  would  be  natural  to  infer  that  the  wisest  thing 
to  do  would  be  to  avoid  bringing  such  children  into  the  world ;  but,  as 
several  healthy  children  have  been  born  to  parents  who  have  had  one 
or  two  children  afflicted  with  amaurotic  family  idiocy,  advice  bearing 
upon  this  point  cannot  readily  be  given.  In  conclusion  I  consider  it 
necessary  to  remind  the  oculist  that  these  patients  can  no  longer  be 
regarded  as  afflicted  with  a  rare  ocular  condition,  but  that  the  ocular 
condition,  interesting  as  it  is,  is  merely  one  symptom  of  an  easily 
recognizable  family  affection. 

INFANTILE   CEREBRAL  PALSIES    (SPASTIC   HEMIPLEGIA, 
DIPLEGIA,  AND  PARAPLEGIA). 

Although  varying  widely  in  their  mode  of  origin,  the  cerebral 
palsies  of  children  deserve  to  be  treated  as  a  distinct  clinical  group  of 
diseases.  A  study  of  the  subject  many  years  ago  led  me  to  divide  them 
into  three  classes  in  accordance  with  the  mode  of  onset, — viz.,  those  that 
were  prenatal  in  origin,  those  that  were  due  to  difficulties  during  parturi- 
tion, and  those  which  were  distinctly  acquired  some  years  after  birth. 

Whatever  the  time  or  manner  of  origin  may  have  been,  most  of 
these  cerebral  palsies  are  observed  in  the  earlier  years  of  life, — from  the 
time  of  birth  up  to  the  age  of  ten  years,  even  later;  but  by  far  the 
greater  proportion  of  them  occur  during  the  first  three  years  of  life.  The 
paralysis  is  of  the  distinctly  spastic  order  and  according  to  the  distri- 
bution of  the  palsy  we  may  distinguish  between  a  hemiplegia  and  a 
diplegia  (double  hemiplegia),  or  paraplegia.  Monoplegias  are  rela- 
tively rare,  and  some  of  those  that  appear  to  be  monoplegic  in  character 
are  practically  the  incomplete  diplegias  or  hemiplegias.  The  rigidity 
of  the  muscles,  the  contractures  resulting  from  the  same  causes,  and  in- 
crease of  all  the  deep  reflexes  in  the  paralyzed  members  are  the  constant 
accompaniment  of  these  palsies.  Coma  and  convulsions  may  occur  in 
the  initial  stage  of  the  acute  forms,  and  the  convulsions  may  be  often 
repeated  during  the  later  stages  of  the  disease.  The  cerebral  palsies 
of  children  are  more  frequently  associated  with  coma  and  convulsions 
than  are  those  of  the  adult;  the  former  are  generally  due  to  cortical 
processes,  the  latter  to  intra-cerebral  conditions.  To  say  that  the  con- 
vulsions may  frequently  be  repeated  is  equivalent  to  saying  that  an 
epilepsy  has  been  established  which  may  continue  throughout  life,  and 
the  same  changes  which  have  caused  both  the  paralysis  and  the  epilepsy 
may  also  be  responsible  for  the  defective  mental  development,  which 
may  range  from  weakmindedness  to  marked  imbecility  and  complete 


538  THE    EYE    AND    XERVOUS    SYSTEM 

idiocy.  It  is  especially  to  be  noted  that  disturbances  of  motion,  asso- 
ciated movements,  ataxic,  athetoid,  choreiform,  and  even  cataleptic 
movements,  occur  often  enough  in  connection  with  these  diseases. 

Aphasia,  so  common  in  apoplectic  disorders  of  the  adult,  is  a  much 
rarer  complication  in  infantile  palsies.  It  goes  without  saying  that  all 
these  conditions  are  characterized  by  certain  negative  symptoms  which 
are  of  greatest  importance  in  attempting  a  differential  diagnosis  be- 
tween the  cerebral  spastic  and  the  spinal  flaccid  palsies.  Such  symp- 
toms include  the  entire  absence  of  changes  in  the  electrical  reactions 
and  the  absence  of  any  marked  atrophy  in  association  with  the  palsy, 
although  a  slight  atrophy  from  disuse  is  common  enough. 

Etiology. — In  discussing  the  origin  of  cerebral  palsies,  we  must  bear 
in  mind  the  three  groups  alluded  to  above.  As  for  the  prenatal  cases, 
they  may  be  due  to  hereditary  taints,  which  is  equivalent  to  saying  that 
fiuch  cases  occur  in  families  with  a  decided  history  of  various  neuroses, 
with  a  history  of  hereditary  epilepsy,  or  of  hereditary  insanity.  The 
next  most  important  etiological  factor  is  unquestionably  the  occurrence 
of  some  traumatism  to  the  mother  during  the  period  of  pregnancy.  The 
manner  in  which  such  traumatism  may  injure  the  brain  of  the  child  was 
clearly  shown  by  Cotard,  who  found  in  the  brain  of  a  still-bom  child  an 
old  lesion  in  the  right  hemisphere  in  the  vicinity  of  the  lateral  ventricle. 
Illness  of  the  mother  during  pregnancy,  exhausting  fevers,  particularly 
such  as  accompany  pneumonia  and  typhoid  fever,  ursemic  convulsions, 
and  severe  fright,  are  other  causes  which  have  been  made  out  in  cases 
of  prenatal  origin. 

My  own  experience  would  lead  me  to  deny  the  influence  of  heredi- 
tary syphilis,  but  so  many  cases  of  this  character  have  been  described 
by  others  that  I  must  accept  this  as  a  possible  etiological  factor  in  some 
of  the  palsies. 

The  etiology  of  a  birth  palsy  is  a  very  simple  one.  While  the  brain 
of  the  new-born  child  tolerates  a  surprising  amount  of  pressure,  it  is 
natural  that  harm  should  occasionally  be  done.  Asphyxia  at  birth,  as 
Little  was  the  first  definitely  to  point  out,  has  been  considered  a  most 
potent  factor.  My  own  statistics  have  shown  that  tedious  labor  is  a 
more  frequent  and  a  more  disastrous  factor  than  is  instrumental  de- 
livery. This  explains  why  cerebral  birth  palsies  occur  with  greatest 
frequency  in  first-born  children. 

Acute  cerebral  palsies  may  be  due  to  a  variety  of  causes.  They  may 
come  on  after  measles,  scarlatina,  typhoid  fever,  small-pox,  and  even 
tonsillitis.  Their  onset  after  pneumonia  and  whooping  cough  is  very 
common,  but  in  the  case  of  the  latter  it  is  questionable  whether  the 


INFANTILE  CEREBRAL  PALSIES  539 

palsy  is  due  to  the  eflFect  of  the  toxic  agent  or  to  the  mechanical  injury 
during  a  spasm  of  coughing.  Fright,  which  Freud  has  dwelt  upon,  as 
the  equivalent  of  a  psychic  trauma  is  an  occasional  cause  of  acute  cere- 
bral paralysis,  but  actual  injury  to  the  skull  is  a  much  more  powerful 
factor.  These  palsies  occur  also  after  a  simple  or  cerebrospinal  menin- 
gitis, after  an  exhausting  gastroenteritis,  and  after  other  fevers.  Of 
ninety-one  cases  of  acquired  cerebral  palsy  which  I  studied  many  years 
ago,  the  exact  cause  could  not  be  ascertained  in  twenty-seven. 

There  was  a  tendency  some  years  ago,  particularly  among  French 
writers,  to  claim  that  all  cases  of  acquired  cerebral  palsy  were  due  to 
acute  infectious  diseases.  This  view  was  also  insisted  upon  by  Striim- 
pell,  who  established  the  analogy  between  the  acute  cerebral  and  the 
acute  spinal  paralyses  of  children  and  concluded  that  the  former  are 
due  to  a  polioencephalitis  just  as  the  latter  are  due  to  a  poliomyelitis. 
While  the  dependance  of  acute  or  acquired  cases  of  polioencephalitis  has 
been  very  much  over-stated,  there  is  evidence  enough  to  compel  us  to 
concede  that  some  of  these  acquired  cases  may  be  due  to  an  encephalitis. 
A  discussion  has  arisen  between  other  authors  and  myself  regarding  the 
etiological  importance  of  convulsions.  Freud  and  Rie  are  of  the  opin- 
ion that  convulsions  denote  the  onset  of  the  cerebral  process,  but  that 
they  never  hold  a  causal  relation  to  the  palsy  which  results  from  the 
cerebral  lesion.  While  one  may  be  willing  to  grant  that  in  a  very  large 
number  of  cases  this  statement  of  the  Austrian  authors  holds  good, 
Osier  and  myself  have  advanced  the  view  that  in  many  others  the  palsy 
seems  to  be  more  or  less  the  immediate  result  of  the  convulsive  seizure. 
Anyone  who  has  observed  the  marked  disturbances  of  circulation  at  the 
acme  of  a  convulsion,  can  readily  conceive  how  easily  a  blood-vessel 
could  burst  during  this  period  as  a  result  of  excessive  stasis ;  and  that 
such  venous  hemorrhages  occur  in  epileptic  convulsions  I  have  had  rea- 
son to  know  from  the  examination  of  the  brain  of  a  girl  of  seventeen 
years,  who  died  three  days  after  a  severe  epileptic  attack.  In  this  case 
there  was  a  large  subpial  extravasation,  which  covered  quite  completely 
the  entire  left  hemisphere  and  part  of  the  right.  If  such  an  occurrence 
could  take  place  in  the  brain  of  a  girl  of  seventeen,  there  is  no  reason 
why  similar  accidents  of  lesser  extent  should  not  happen  frequently 
in  the  case  of  children.  There  is  also  further  clinical  evidence  on  this 
point,  and  those  who  are  specially  interested  in  this  subject  are  referred 
to  a  discussion  of  it  in  my  book  on  "  Nervous  Diseases  of  Children," 
page  531. 

Sjmiptomatology. — The  distribution  of  the  paralysis  may  vary  with 
the  condition  of  the  paralyzed  limb  and  is  the  same  in  all  cases.     The 


540  THE    EYE    AND    NERVOUS    SYSTEM 

hemiplegic  form  resembles  in  every  respect  the  hemiplegia  of  the  adult, 
and  further  analogy  is  observed  in  this  that  the  leg  recovers  very  much 
more  quickly  than  the  arm,  just  as  in  adult  hemiplegia.  Under 
diplegia,  we  may  classify  all  those  cases  in  which  both  halves  of  the 
body  have  been  involved,  and  it  is  better  to  attach  the  greatest  impor- 
tance to  this  bilateral  character  of  the  palsy  even  though  the  palsy  be 
incomplete,  for  the  bilateral  affection  points  to  a  double  brain  lesion 
and  that  is,  after  all,  the  salient  point  for  consideration.  In  diplegias, 
the  legs  may  be  much  more  affected  than  the  arms,  there  may  be  cross- 
legged  position,  and  cross-legged  progression,  and  in  some  instances  the 
arms  may  appear  to  have  escaped  to  such  an  extent  that  what  is  an  in- 
complete diplegia  may  appear  to  be  a  paraplegia,  but  a  paraplegia  asso- 
ciated with  athetoid  or  other  disturbances  of  motion  in  the  upper  ex- 
tremities could  be  designated  as  an  incomplete  diplegia ;  but  whether  the 
form  of  paralysis  be  a  diplegia  or  a  paraplegia,  such  paralysis  must 
necessarily  be  due  to  a  double  cerebral  lesion  and  these  are  almost  in- 
variably prenatal  in  origin  or  the  result  of  traumatism  during  labor, 
and  yet  it  is  well  to  state  that  both  diplegias  and  paraplegias  also  occur 
every  now  and  then  in  the  acute  cerebral  cases,  contrary  to  the  opinion 
of  Striimpell,  who  holds  that  infantile  hemiplegia  is  synonymous  with 
the  acute  cerebral  paralysis  of  children. 

Aphasia  may  be  associated  with  these  infantile  cerebral  palsies. 
It  is  invariably  motor,  never  sensory,  in  character.  It  need  not  be 
added  that  aphasia  will  be  developed  only  in  children  who  have  acquired 
articulate  speech  before  the  onset  of  the  paralysis.  This  excludes  from 
the  list  all  diplegias  and  paraplegias  which  have  come  on  before  or 
during  birth.  It  must  be  remembered  that  a  complete  absence  of  speech 
development  may  be  present  in  children  who  exhibit  cerebral  and  mental 
defects  and  I  have  seen  some  children  in  whom  the  mental  defect  seemed 
to  be  due  more  largely  to  defective  development  of  the  speech  centres 
than  of  any  other  part  of  the  brain. 

In  addition  to  the  form  of  paralysis,  it  is  well  to  pay  special  atten- 
tion to  the  rigidities  and  contractures.  These  abnormal  conditions  of  the 
muscles  are  so  prominent  that  doubt  has  arisen  whether  some  of  the 
diplegias  or  paraplegias  would  not  be  more  properly  spoken  of  as  cases 
of  congenital  spastic  rigidity,  for  the  rigidity  is  most  marked  and  the 
paralysis  often  very  slight.  In  the  case  of  Cotard  quoted  above  in 
which  the  palsy  was  due  to  an  intrauterine  lesion,  the  child  was  bom 
with  contractured  extremities.  The  contractures  may  vary  in  degree 
and  in  the  number  of  joints  affected.  The  flexors  and  pronators  of  the 
arms,  flexors  of  the  legs  and  of  the  feet  are  most  frequently  involved. 


INFANTILE  CEKEBRAL  PALSIES  541 

In  the  case  of  diplegia  and  paraplegia  there  is,  in  addition,  contracture 
of  the  adductors  of  the  thighs,  which  is  responsible  for  the  peculiar 
cross-legged  progression  and  cross-legged  position.  Pes  equinus  or  pes 
equino-varus  is  the  most  common  deformity  of  the  foot.  In  a  few  cases 
an  equino-valgus  is  present.  If  the  upper  extremity  is  contractured  the 
arm  is  held  in  close  juxtaposition  to  the  trunk,  is  flexed  at  the  elbow, 
and  the  hand  is  generally  in  a  position  of  extreme  flexion,  the  fingers 
often  being  firmly  pressed  into  the  palm  of  the  hand. 

The  post-paralytic  disturbances  of  motion  constitute  a  very  charac- 
teristic group  of  symptoms  in  cases  of  infantile  cerebral  paralysis. 
While  they  are  seen  occasionally  in  adult  apoplexy,  they  are  far  more 
common  in  the  paralytic  disturbances  of  youth.  The  choreiform  move- 
ments are  of  importance  because  cases  of  that  kind  are  often  mistaken 
for  cases  of  ordinary  chorea,  but  of  course  the  presence  of  paralysis  or  of 
contractions,  and  the  exaggeration  of  the  reflexes  in  the  choreic  ex- 
tremity, will  be  sufficient  to  indicate  the  nature  of  the  choreic  move- 
ments. Athetoid  movements  are  of  special  interest,  inasmuch  as  they 
are  observed  invariably  in  connection  with  cerebral  palsies.  Associated 
movements  are  often  developed  to  a  marked  degree,  the  paralyzed  hand 
imitating  all  movements  of  the  normal  hand. 

Epilepsy  is,  perhaps,  the  most  serious  of  all  conditions  associated 
with  infantile  cerebral  paralysis.  The  special  point  of  importance  is 
that  the  palsy  may  disappear  or  may  be  so  slight  as  to  give  little  in- 
convenience to  the  child,  whereas  the  epilepsy  remains.  Special  reason 
for  insisting  upon  this  point  is  that  in  such  cases  the  epilepsy  as  well  as 
the  paralysis  is  of  organic  origin  and  we  cannot  therefore  expect  to  cure 
the  disease  by  the  use  of  the  ordinary  measures. 

Idiocy  is  also  developed  very  often  both  in  association  with  epilepsy 
and  with  cerebral  palsies.  The  severer  forms  of  mental  retardation  are 
present  in  cases  of  diplegia  and  in  cases  of  paraplegia  rather  than  hemi- 
plegia, and  this  association  can  be  explained  easily  enough  if  we  remem- 
ber that  the  diplegias  and  paraplegias  point  to  a  double  lesion,  whereas 
the  hemiplegia  results  from  a  lesion  in  only  one-half  of  the  brain. 

As  for  the  eye  symptoms  observed  in  these  conditions,  the  com- 
monest are  strabismus  and  occasionally  nystagmus,  and  as  Freud  has 
pointed  out,  hemianopsia  may  be  associated  with  the  brain  palsies  of 
children.  The  latter  condition  is  surely  a  very  uncommon  one  and 
would  depend  largely  upon  the  accidental  involvement  of  the  occipital 
region  in  a  case  of  cerebral  defect  or  in  a  case  in  which  considerable 
blood  had  been  exuded  over  the  occipital  region  of  the  brain  during 
parturition.     The  pupillary  reflexes  are  normal  in  such  a  vast  majority 


542 


THE    EYE    AND    NERVOUS    SYSTEM 


of  these  children  that  they  are  of  no  symptomatic  value.  Freud  has 
reported  some  eases  of  optic  atrophy  associated  with  diplegias  and  he 
thinks  that  these  conditions,  which  are  rare  enough,  may  bear  a  very 
close  relation  to  the  changes  found  in  cases  of  amaurotic  family  idiocy. 
I  have  never  seen  changes  in  the  macula  lutea  in  any  case  of  distinct 
cerebral  spastic  paralysis. 

Morbid  Anatomy. — The  entire  subject  of  morbid  anatomy  of  infantile 
cerebral  palsies  will  be  sufficiently  stated  in  this  connection  by  the  repro- 
duction of  a  table  which  was  first  embodied  in  the  chapter  on  these 
diseases  in  the  author's  text-book,  and  which  has  been  very  fully  quoted 
by  later  authors.  I  have  nothing  to  add  to  it,  and  believe  that  it  fully 
represents  our  present  knowledge  of  the  subject. 

CLASSIFICATION    OF    INFANTILE    CEREBRAL    PALSIES. 


I.  Paralyses   of   intra-uterine  onset. 


II.  Birth    palsies 


III.  Acute   (acquired)   palsies 


MOSBU)   LESIONS. 

Large  cerebral  defects   ( porencephaly ) . 

Defective  development  of  pyramidal 
tracts. 

Agenesis  corticalis  (highest  nerve  ele- 
ments involved). 

Meningeal  hemorrhage,  rarely  intra- 
cerebral hemorrhage.  Later  condi- 
tions: meningo-encephalitis  chronica, 
sclerosis,  and  cysts;    partial  atrophies. 

Hemorrhage  (meningearl  and  rarely  in- 
tracerebral )  ;  thrombosis  from  syph- 
ilitic endarteritis  and  in  marantic 
conditions:  atrophy,  cysts,  and  scle- 
rosis   (diffuse  and  lobar). 

Meningitis  chronica. 

Hydrocephalus   (seldom  the  sole  cause). 

Primary  encephalitis;  polioencephalitis 
acuta    ( StrUmpell ) . 


Differential  Diagnosis. — First  of  all  it  is  important  to  distinguish 
between  the  cerebral  and  the  spinal  paralyses  of  children,  but  anyone 
who  is  able  to  recognize  the  difference  between  brain  and  spinal  lesions 
will  be  able  to  make  this  differential  diagnosis.  In  the  one  case  we  have 
spastic  paralysis,  increased  reflexes,  normal  electrical  reactions,  no 
atrophy,  and  generally  a  hemiplegic  or  diplegic  form  of  paralysis.  In 
the  spinal  group  the  paralysis  is  flaccid,  the  knee-jerks  are  absent,  there 
is  very  marked  atrophy  and  the  paralysis  may  affect  several  members 
of  the  body,  or  several  large  groups  of  muscles,  but  is  never  hemiplegic. 
Moreover,  the  electrical  reactions  in  the  spinal  cases  are  always  in- 
volved and  altered.    Difficulties  may,  however,  arise  in  some  mild  cases. 


INFANTILE  CEREBRAL  PALSIES  543 

of  cerebral  paralysis  in  which  the  spastic  contractions  are  but  slightly 
developed  and  the  reflexes  not  very  much  increased.  The  presence  of 
athetoid  or  choreic  movements  in  one-half  of  the  body  points  to  cerebral 
paralysis,  but  these  are  never  associated  with  spinal  palsies. 

The  oculist  should  bear  in  mind  that  the  nystagmus  and  strabismus 
may  be  symptoms  of  multiple  sclerosis,  but  the  differential  diagnosis 
should  be  based  upon  scanning  speech  and  upon  the  absence  of  associated 
movements,  and  the  post-paralytic  disturbances  of  motion  which  are  so 
common  in  the  infantile  spastic  palsies.  Inasmuch  as  there  is  a  heredi- 
tary form  of  multiple  sclerosis  this  differential  diagnosis  may  have  to 
be  made  with  the  greatest  possible  care.  A  very  distinct  hemiplegia  or 
a  diplegia  would  militate  against  the  diagnosis  of  multiple  sclerosis. 

Treatment. — The  treatment  of  infantile  cerebral  palsies  calls  for 
deliberate  non-interference  at  the  start.  The  treatment  of  the  later 
stages  will  be  practically  the  same  whether  the  case  be  congenital  or 
acquired.  If  a  young  infant  exhibits  a  tendency  to  drowsiness  or  to 
convulsions,  its  brain  has  in  all  probability  sustained  a  serious  injury 
during  labor.  Strict  attention  should  be  paid  to  the  nutrition  of  the 
child,  and  milk  properly  prepared  according  to  the  age  of  the  child,  or 
else  breast  milk,  is  the  only  food  to  be  administered.  If  there  is  a  very 
marked  tendency  to  convulsions,  small  doses  of  bromide  or  minimum 
doses  of  morphia  or  chloral  should  be  given.  If  convulsions  continue  in 
spite  of  these  measures,  careful  inhalation  of  chloroform  may  be  prac- 
tised. All  other  measures,  as  counter-irritation,  mustard  baths  and  the 
like,  are  useless. 

In  a  case  of  an  acute  cerebral  palsy  in  a  child,  the  fewer  drugs  that 
are  administered  the  better.  The  physician  will  do  well  to  limit  his 
therapeutic  efforts  to  the  administration  of  calomel  in  doses  sufficient 
to  procure  a  free  purging  of  the  bowels,  and  small  doses  of  bromides  to 
secure  rest  for  the  disturbed  brain.  At  a  later  period  bromides  may  be 
combined  with  small  doses  of  iodide,  but  whenever  nutrition  is  dis- 
turbed, it  is  best  to  abandon  all  drug  treatment.  After  the  symptoms  of 
the  initial  period  have  passed,  the  physician  is  called  upon  to  prescribe 
some  form  of  treatment  for  the  paralysis  and  the  other  s^Tuptoms  of  the 
disease.  After  the  paralytic  condition  of  the  active  period  of  the 
disease  has  passed,  the  condition  is  to  be  treated  by  massage  and  elec- 
tricity applied  to  the  paralyzed  parts,  but  let  the  brain  severely  alone. 
While  electricity  applied  to  the  extremities  can  do  no  harm,  the  effect  on 
the  injured  brain  is  altogether  too  uncertain.  When  epilepsy  is  devel- 
oped the  condition  must  be  treated  as  all  other  organic  epilepsies  should 
be  and  in  some  of  these  cases,  according  to  the  experience  of  Horsley, 


644  TUE    EYE    AND    NEKVOUS    SYSTEM 

Wjeth,  Gerster,  and  myself,  there  is  sufficient  reason  to  attempt  surgical 
interference,  particularly  if  all  the  symptoms  point  to  a  well  localized 
lesion,  and  above  all,  if  there  is  a  probability  of  the  existence  of  an  old 
hemorrhagic  cyst,  a  condition  which  is  found  in  not  a  few  of  the  con- 
genital and  acute  cases.  In  children  in  whom  the  rigidity  is  the  most 
marked  symptom,  therapeutic  measures  are  in  order  and  tenotomies,  as 
well  as  transplantation  of  tendons,  yield  good  results  in  the  hands  of 
competent  men. 

BIBLIOGRAPHY. 

(For  additional  references,  see  author's  article  in  Journal  of  Nervous,  and  Mental 

Disease,  January,  1903.) 
^Cakteb:    Archives  of  Ophthalmology  and  Otology,  1894. 
"Claiboeke:    Archives  of  Pediatrics,  1900,  vol.  x.  p.  3. 
*CoLLiEB:    Brain,  1899,  vol.  xxii.  p.  373. 

*  Freud  :    Zur  Kenntniss  der  Cerebralen  Diplegien  des  Kindesalters,  Vienna,  1893 ; 

Die  Infantile  Cerebrallilhmung,  Vienna,  1897. 

•  Falkenheim  :    Jahrbuch  f.  Kinderheilkunde,  1901,  N.  Y.,  vol.  liv, 
•Frost:    Fundus  Oculi,  Edinburgh,  1896. 

^Goldzieher:     Hirschberg's  Centralblatt,  1885,  S.  219. 

"Heiman:    Archives  of  Pediatrics,  April,  1897. 

•HiGiER:  Zur  Klinik  der  Familiaren  Opticusaffectionen ;  Zeitschr.  f.  Nervenheil- 
kunde,  1897,  Bd.  x. 

*"  HiBSCHBERG :    Centralblatt  f.  Augenheilkunde,  1888. 

"  HiRSCH,  W. :     Journal  of  Nervous  and  Mental  Disease,  1898,  vol.  xxv.  p.  538. 

"KiNGDON:  Transactions  of  the  Ophthalmological  Society,  Heidelberg,  1895;  with 
Russell,  Medico-Chirurgical  Transactions,  1897,  vol.  Ixxx. 

"Roller:    New  York  Medical  Record,  1896. 

"Koplik:    Archives  of  Pediatrics,  1897. 

"Magnus:    Zehender's  klinischer  Monatsbliitter,  1885,  Bd.  xxiii.  S.  357. 

"MuRATOW:    Zeitschr.  f.  Nervenheilkunde,  Bd.  x.  S.  234. 

"Pelizaeus:    Archiv  f.  Psychiatric,  Bd.  xvi.  S.  698. 

"Sachs,  B.:  Journal  of  Nervous  and  Mental  Disease,  1887,  1892,  and  1903;  Volk- 
mann's  Vortriige,  1892,  Nos.  46,  47;  Nervous  Diseases  of  Children,  p.  396; 
New  York  Medical  Journal,  May  30,  1896. 

"Spiller:  Journal  of  Nervous  and  Mental  Disease,  1901,  p.  140;  American  Journal 
of  the  Medical  Sciences,  January,  1905. 

"Tay,  Waren:  Transactions  of  the  Ophthalmological  Society  of  the  United  King- 
dom, vols.  i.  and  iv. 

"  Wadswortii  :    Transactions  of  the  American  Ophthalmological  Society,  1887. 


CHAPTER    XIV. 

ABNOEMALITIES  IK  THE  DEVELOPMENT  OF  THE  BEAJN 
AND  SKULL ;  FACIAX  HEMIATEOPHY ;  FACIAL  HEMI- 
HYPERTROPHYj  ACROMEGALY. 

By  CHARLES  W.  BURR,  M.  D. 

I  SHALL  not  consider  anencephaly  or  any  of  the  conditions  in  which 
the  abnormality  of  development  or  the  intrauterine  disease  is  so  great 
as  necessarily  to  cause  fetal  death.  Such  conditions  are  of  great  interest 
in  the  study  of  the  causation  of  monsters,  but  are  of  no  clinical  impor- 
tance. The  normal  development  of  the  brain  and  that  of  the  skull  are 
closely  associated  with  each  other  and  pathological  development  in  either 
may  affect  the  other,  but  for  convenience  sake  they  are  studied  sepa- 
rately. Strictly  speaking,  the  term  "  defects  of  development"  should  be 
used  to  mean  only  defects  due  to  an  improper  growth  of  the  brain  on 
account  of  inherent  deficiency  in  the  germ  or  sperm  cell  and  should 
not  include  abnormalities  caused  by  acquired  disease  occurring  in  the 
embryo ;  but  our  ignorance  of  embryonal  pathology  is  so  great  that  such 
a  distinction  can  rarely  be  made. 

Apart  from  the  abnormal  development  which  causes  gross  defects 
there  is  another  kind  of  lack  of  development,  really  congenital  in  origin 
though  the  symptoms  of  disease  do  not  appear  till  some  time,  it  may 
be  years,  after  birth.  I  mean  that  condition  in  which  the  patient  may 
at  birth  seem  normal,  may  indeed  pass  normally  through  infancy,  and 
then,  or  even  later,  show  symptoms  of  disturbance  of  the  functions  of 
the  brain  or  cord,  not  produced  by  any  of  the  known  causes  of  disease, 
but  by  inherent  weakness  and  inability  to  withstand  the  physiological 
stress  of  life.  This  weakness  may  exist  not  only  in  the  nervous  system 
but  in  any  part  of  the  body.  It  has  been  recently  described,  or  rather 
offered  as  an  explanation  (for,  of  course,  it  is  a  theory  and  not  an  estab- 
lished fact),  by  Sir  William  Gowers  under  the  title  "Abiotrophy." 
He  states,  what  has  been  long  known,  that  life  is  a  double  thing, — 
somatic,  that  of  the  body  as  a  whole,  and  local,  that  of  each  element  by 
itself.  Death  of  some  of  the  elements  may  occur  without  somatic  life 
being  affected.  This  process  we  call  degeneration,  but  it  is  really  a 
slow  decay,  a  local  death  due  to  an  essential  failure  of  vitality.  This 
process  may  affect  not  only  some  one  system  of  nerve-fibres  in  the  brain 
35  545 


646  THE    EYE    AND    NERVOUS    SYSTEM 

or  cord,  as  in  Friedreich's  ataxia  or  congenital  cerebellar  atrophy,  but 
also  the  muscles,  as  in  the  various  forms  of  idiopathic  muscular  atrophy. 
Gowers  believes  that  there  is  a  certain  type  of  degeneration  of  the  optic 
nerve,  occurring  in  families,  which  is  really  abiotic,  really  due  to  an 
inherent  weakness  of  the  nerve-fibres  and  in  which  tobacco  or  some  other 
poison  acts  only  as  an  exciting,  not  a  true,  cause.  This  theory  does  not 
claim  that  all  degeneration  in  the  pathologist's  sense  of  the  word  is 
abiotic. 

Porencephaly. — The  word  porencephaly  was  first  used  by  Herschl 
and  the  first  elaborate  monograph  was  written  in  1882  by  Kundrat.  By 
them  it  was  used  to  mean  a  hole  going  into  the  brain,  not  the  entire 
absence  of  brain  structure  and  not  minute  cavities  resulting  from  small 
vascular  lesions.  Porencephaly  is  classified  as  congenital,  coming  on 
during  intrauterine  life,  or  acquired,  occurring  at  or  very  soon  after 
birth.  Clinically  the  differentiation  is  always  difficult,  often  impossible. 
Practically  it  does  not  matter  to  which  type  a  case  belongs.  The  con- 
genital cases  like  the  acquired  are  probably  always  due  to  disease  and 
not  the  result  of  a  true  developmental  defect. 

The  cavity  may  occur  in  any  part  of  the  brain  but  is  most  frequent 
in  the  anterior  or  middle.  Usually  there  is  only  one  cavity  but  there 
may  be  two  situated  in  the  same  position  in  either  hemisphere.  For 
example,  the  parietal  region  may  be  affected  on  both  sides  or  both 
frontal  lobes.  When  there  is  only  one  cavity  it  is  more  apt  to  be  in 
the  right  hemisphere.  Brill  states  that  no  case  has  been  reported  in- 
volving the  median  surface  of  the  hemisphere,  but  Brush  reports  a  case 
in  which  the  cuneus  was  much  affected. 

Kundrat  holds  that  the  immediate  cause  is  ansemic  necrosis. 
Schultze  would  sharply  separate  the  cases  of  uterine  origin  from  those 
occurring  after  birth  by  the  following  rule:  Tn  the  former,  he  states, 
the  convolutions  are  so  arranged  as  to  radiate  toward  the  margin  of  the 
cavity  or  towards  its  centre,  while  in  the  latter  the  hole  is  irregular  in 
outline  and  the  convolutions  have  no  regular  arrangement.  The  most 
frequent  direct  cause  is  probably  vascular  or  meningeal  inflammation. 
Traumatic  encephalitis  may  occur  during  intrauterine  life  and  cause 
the  formation  of  a  cavity.  Schultze,  for  example,  holds  that  spasmodic 
contraction  of  the  uterus  arising  from  causes  within  itself  may  injure 
the  fetal  brain.  Alcoholism  in  the  mother  and  syphilis  in  either  parent 
have  been  cited  as  causes  of  obliterating  arteritis  in  the  foetus.  B. 
Sachs  speaks  of  the  possibility  of  some  purulent  disease  in  the  mother 
(luring  pregnancy  as  being  sufficient  to  cause  meningitis  or  encephalitis 
which  might,  occurring  in  a  growing  foetus,  produce  porencephaly.     In 


a:n^omalies  of  brain  and  skull  547 

acquired  porencephaly  the  primary  lesion  seems  almost  always  to  be  a 
meningeal  hemorrhage  occurring  at  birth  and  resulting  from  pressure  on 
the  skull  during  labor.  Whether  meningeal  hemorrhage  occurs  most 
frequently  from  the  use  or  non-use  of  the  obstetric  forceps  is  much  dis- 
cussed. Probably  more  injury  has  been  done  by  the  non-use  of  the 
forceps  than  by  their  unskilful  application.  Whatever  may  cause  the 
hemorrhage,  if  it  be  large  enough,  there  is  compression  of  the  brain 
substance,  atrophy  of  the  cortex  and  underlying  white  matter  and  a 
consequent  cavity.  The  pia  and  cortex  are  apt  to  become  inflamed, 
agglutinate,  form  a  dense  membrane  lining  the  cavity  which  is  filled 
with  fluid,  thus  forming  a  cyst  covered  by  the  outer  membranes.  The 
time  required  for  the  absorption  of  the  brain  tissue  and  the  formation 
of  the  cavity  is  unknown.  Acquired  porencephaly  is  most  apt  to  occur 
in  the  parietal  region  and  is  usually  unilateral,  but  may  be  bilateral. 

There  are  no  pathognomonic  symptoms.  It  is  not  difficult  to  deter- 
mine the  existence  of  organic  brain  disease,  but  it  is  impossible  to  say 
with  certainty  whether  the  symptoms  are  due  to  porencephaly  or  to 
cerebral  atrophy  without  cavity  formation.  Almost  always,  but  not 
invariably,  there  is  marked  mental  defect.  Usually  there  is  idiocy  or 
at  least  imbecility.  Rarely  in  unilateral  cases  without  great  destruction 
of  the  cerebral  tissue  the  mind  may  be  unimpaired.  The  most  frequent 
physical  symptoms  are  spastic  hemiplegia  or  diplegia.  Epileptiform 
convulsions  are  very  frequent.  If  the  cuneus  is  involved  hemianopsia 
must  result.  Often  there  is  a  spastic  disorder  of  speech  and  sometimes 
a  true  aphasia  or  rather,  from  the  destruction  of  the  potential  speech 
centres,  an  inability  to  learn  to  speak.  Probably  the  majority  of  the 
patients,  certainly  a  very  large  number,  die  in  early  life ;  a  few  survive 
to  maturity,  and  even  old  age. 

Microcephalus. — The  term  is  used  to  mean  only  those  cases  of  a 
small  brain  associated  with  a  small  skull  and  due  to  defective  develop- 
ment of  the  entire  organ.  It  must  not  be  forgotten  that  a  skull  of 
normal  size  may  contain  a  brain  of  very  small  volume,  the  surplus 
space  being  filled  by  fluid.  Any  skull  under  seventeen  inches  in  cir- 
cumference is,  according  to  Dr.  Ireland,  to  be  classed  as  microcephalic. 
The  head  is,  as  a  rule,  cone-shaped  or  oxycephalic.  The  forehead  slants 
backward  in  marked  manner.  The  palate  is  generally  flat  and  the  base 
of  the  skull  asymmetrical.  The  face  is  never  proportionally  as  small 
as  the  cranium.  The  causes  of  the  condition  are  very  obscure.  There 
is  no  doubt  that  a  few  cases  are  produced  mechanically  by  a  pre- 
mature ossification  of  the  sutures  preventing  the  growth  of  the  brain. 
This  was  Yirchow's  theory.      Klebs's  theory  that  hour-glass  contrac- 


548  THE    EYE    AND    NERVOUS    SYSTEM 

tion  of  the  uterus  on  the  fetal  head  is  a  cause  is  no  longer  regarded 
with  iavor.  It  is  probable  that  some  cases  are  caused  by  intrauterine 
cerebral  inflammation.  B.  Sachs  describes  cases  occurring  after  birth 
and  following  convulsions  in  which  it  was  probable,  he  thinks,  that  the 
convulsions  resulted  in  extensive  meningeal  hemorrhage  or  pachy- 
meningitis hemorrhagica,  in  consequence  of  which  there  was  atrophy 
of  the  brain  and  associated  with  it  a  lessening  in  the  growth  of  the 
skull.  In  these  cases  the  children  were  normal  at  birth  and  with  skulls 
of  average  circumference.  The  relation,  under  normal  conditions,  be- 
tween the  growth  and  development  of  the  brain  and  skull  is  very 
obscure.  Since,  however,  there  may  be  a  small  and  universally  diseased 
brain  associated  with  a  skull  of  normal  size,  the  space  being  filled  with 
fluid,  it  is  not  probable  that  the  brain  exerts  any  trophic  influence  on 
skull  growth. 

Though  the  entire  brain  is  affected  in  microcephaly  the  cerebral 
hemispheres  are  most  deficient.  The  cerebellum  and  the  basal  ganglia 
are  less  abnormal.  The  spinal  cord,  as  a  rule,  is  also  shorter  and 
smaller  than  normal.  The  columns  of  Goll,  the  anterior  horns,  and 
pyramidal  tracts  are  especially  affected.  Sometimes  even  with  spastic 
diplegia  the  cord  is  only  small  and  shows  no  degeneration.  Small  as 
the  brain  may  be,  and  Ireland  reports  one  the  weight  of  which  was 
seven  ounces,  it  is  distinctly  human  in  form,  and  could  never  be  mis- 
taken for  that  of  one  of  the  lower  animals.  The  primary  fissures  are 
always  present,  the  secondary  may  be  absent  or  may  be  replaced  by 
others  much  more  numerous,  making  a  large  number  of  small  convolu- 
tions (microgyria).  In  old  times  the  normal  brain  was  compared  in 
appearance  to  a  bunch  of  worms;  the  resemblance  is  much  greater  in 
some  cases  of  microcephaly.  In  the  cases  in  which  there  are  fewer 
fissures  than  normal  the  surface  of  the  brain  is,  of  course,  smooth. 
It  is  of  historic  interest  that  at  the  time  when  Darwinism  was  a  new 
thing,  Charles  Vogt  wrote  a  book  to  prove  that  microcephaly  was  atavis- 
tic,— was  a  reappearance  of  the  simian  type.  This  opinion  has  not 
stood  the  test  of  time. 

The  brain  is  deficient  not  only  macroscopically  but  also  microscop- 
ically. There  are  fewer  nerve-cells  than  normal  in  the  cortex  and  in  the 
anterior  horns  of  the  spinal  cord,  and  many  of  the  cells  present  in  the 
cortex  either  have  no  processes  or  greatly  stunted  ones.  In  some  cases 
there  is  an  overgrowth  of  connective  tissue. 

The  condition  is  not  common ;  indeed  it  ranks  among  the  rarer 
forms  of  idiocy.  It  can,  of  course,  be  diagnosed  at  sight,  but  except  in 
the  cases  of  extreme  smallness  of  the  head,  it  is  not  always  possible  to 


ANOMALIES  OF  BEAIN  AND  SKULL  549 

predicate  the  extent  of  idiocy  from  the  appearance  of  the  head.  In 
other  words,  barring  the  extremes,  the  idiot  with  the  smaller  head  may 
have  more  intelligence  than  he  who  has  the  larger.  The  explanation 
of  this  is,  that  in  the  former  case  the  cortical  cells  themselves  are  less 
diseased.  There  may  be  no  physical  symptoms  beyond  a  general  dwarf- 
ing of  the  entire  body;  on  the  other  hand  the  patient  may  not  be 
able  to  stand  or  walk,  feed  himself,  speak,  or  indeed  do  anything  save 
exist  if  cared  for.  Those  of  a  higher  grade  are  active,  energetic,  easily 
made  angry,  affectionate  but  wilful,  having  no  power  of  continuous 
attention, — indeed  resembling  superficially,  but  only  superficially,  the 
monkey  tribe.  As  a  rule,  they  either  cannot  speak  at  all  or  only  a  few 
words.  This  is  probably  not  a  true  aphasia,  but  due  to  their  general 
low  mental  state.  They  are  apt  to  be  thieves,  but  to  appropriate  things 
useless  or  useful  indiscriminately.  Fortunately,  the  sexual  instinct  in 
the  majority  of  cases  is  wanting.  In  some  the  organs  of  generation 
develop,  but  I  know  of  only  one  case,  that  of  Ireland,  in  which  a  micro- 
cephalic woman  became  pregnant.     The  child  was  born  dead. 

There  is  no  treatment  save  to  give  what  little  education  is  possible. 
The  surgical  intervention  (craniectomy)  proposed  by  Lannelongue  has 
fallen  into  disuse.  It  was  based  on  a  false  pathology  and  had  no 
good  results. 

Local  Cerebral  Defects. — As  said  above,  a  skull  of  normal  size 
may  contain  an  abnormally  small  brain.  Besides  this  there  may  be  an 
atrophy  or  a  lack  of  development  of  some  one  part  of  the  brain, — for 
example,  one  cerebral  hemisphere.  Most  frequently  this-  is  caused  by 
hemorrhage  in  the  meninges  at  birth,  but  the  disease  process  may  occur 
during  intrauterine  life.  Osier,  for  example,  reports  the  case  of  a 
foetus  about  six  months  old,  in  the  left  basal  ganglia  of  which  was  a 
recent  clot  which  had  broken  into  the  ventricle.  The  mother  had  died 
of  typhoid  fever.  It  is  scarcely  possible  that  any  foetus  could  survive 
till  birth  after  suffering  a  cerebral  hemorrhage,  but  it  is  very  possible 
that  many  of  these  cases  are  caused  by  intrauterine  obliterating  vascu- 
lar disease  causing  lack  of  growth.  Whether  any  of  them  are  really 
developmental  defects  is  not  yet  determined.  Clinically,  atrophy  of 
one  hemisphere  is  a  frequent  cause  of  a  congenital  spastic  hemiplegia. 
The  paralyzed  side  is  the  seat  of  choreic  or  athetoid  movements.  Epilep- 
tiform convulsions  are  frequent.  Frequently  there  is  imbecility.  Mills 
relates  a  case,  which  was  not,  however,  intrauterine  in  origin,  but 
followed  convulsions  at  eighteen  months,  in  which  mirror-writing  was 
present.  This  is  met  with  now  and  again  in  both  the  intrauterine 
and  birth  hemiplegias.  Hemianopsia  or  irregular  contraction  of  the  field 


550  THE    EYE    AND    NERVOUS    SYSTEM 

of  vision  may  occur.  There  is  often  no  visual  disturbance.  In  many 
of  the  cases  in  which  the  atrophy  occurs  during  intrauterine  life  the 
skull  is  very  asymmetrical,  being  of  much  smaller  volume  on  the  dis- 
eased side.  This  was  so  marked  in  one  patient  on  whom  a  surgeon 
operated  that  the  longitudinal  sinus  was  displaced  and  he  cut  into  it, 
causing  an  outflow  of  blood,  and  death. 

There  are  still  more  localized  defects  of  development  or  diseased 
areas.  Thus  the  frontal  lobes  may  be  defective  and  the  child,  pre- 
senting no  paralytic  or  sensory  symptoms,  will  be  mentally  deficient. 
Peterson  reports  a  case  in  which  both  occipital  lobes  and  the  motor 
area  on  the  right  side  were  atrophied.  The  patient  was  idiotic,  epilep- 
tic, slightly  hemiplegic,  and  could  scarcely  distinguish  light  from 
darkness. 

Some  cases  of  congenital  cerebellar  ataxia  seem  to  be  due  to  real 
defects  in  development,  not  to  disease  but  to  an  error  in  development 
inherent  in  the  embryo.  Thus,  Xonne  describes  a  small  cerebellum, 
without  sclerosis  and  without  degeneration,  indeed  normal  in  all  regards 
except  as  to  relative  size.  The  spinal  cord  was  also  diminutive.  Before 
Xonne,  Marie  had  described  a  similar  condition  under  the  title  "  heredi- 
tary cerebellar  ataxia."  The  symptoms  are  somewhat  like  those  of 
Friedreich's  ataxia.  There  is  titubation,  choreiform  or  ataxiform  move- 
ments of  the  arms  and  legs,  and  increased  knee-jerks.  The  ocular  sjuip- 
toms  are  important.  There  is  nystagmus,  diplopia,  and  sometimes 
amblyopia  or  color-blindness,  or  contraction  of  the  visual  fields.  Optic 
nerve  atrophy  is  characteristic.  Other  authors  have  described  cases 
more  or  less  resembling  this  type,  and  more  or  less  departing  from  it. 
Indeed,  there  is  clinically  an  unbroken  chain  of  cases  from  Friedreich's 
ataxia  on  the  one  side  to  the  so-called  hereditary  spastic  palsy  on  the 
other.  The  difference  in  symptomatology  must  depend  upon  which 
part  of  the  cerebrospinal  axis  is  lacking  in  development.  If  the  cere- 
bellum alone  be  deficient  the  symptoms  can  not  be  the  same  as  when 
the  cord  alone  or  both  cord  and  cerebellum  are  involved.  The  symp- 
toms of  hereditary  ataxia  do  not  appear  at  birth  but  later,  usually  at 
about  puberty. 

Absence  of  the  Corpus  Callosum. — Nothing  is  definitely  known  of 
the  functions  of  the  corpus  callosum  except  that  it  contains  commissural 
fibres  binding  together  the  hemispheres.  Bruce  *  states  that  it  may  be 
entirely  absent  without  the  patient  showing  any  symptoms.  As  a  rule, 
however,  its  absence  is  associated  with  other  cerebral  defects  and  accom- 
panied by  idiocy  or  imbecility. 

'  Brain,  vol.  xii. 


AXOMALIES  OF  BRAIN  AND  SKULL  551 

Abnormal  Cerebral  Fissuration. — Slight  variation  in  the  direction 
and  number  of  the  minor  fissures  of  the  brain  is  common.  It  has  no 
pathological  significance.  Sometimes  the  variation  from  the  normal 
is  very  marked  and  it  has  been  attempted  to  set  up  a  type  of  criminal, 
epileptic,  and  congenitally  insane  brain.  Moritz  Benedikt  studied  the 
matter  for  years  and  concluded  that  in  either  of  these  conditions  certain 
cerebral  stigmata  are  present.  These  are  excessive  development  of  prin- 
cipal and  subordinate  fissures  and  excessive  confluence  of  fissures  either 
by  elongation  of  the  principal  fissures,  or  by  their  anastomoses  through 
the  great  development  of  subordinate  fissures  connecting  them.  Bene- 
dikt's  papers  led  to  much  discussion,  which  indeed  still  continues,  and 
the  question  remains  unanswered  whether  there  is  a  type  of  brain  which 
predestines  its  owner  to  become  a  criminal  or  an  epileptic.  In  at  least 
one  form  of  insanity  congenital  in  origin, — namely,  paranoia, — there 
are  sometimes  marked  deviations  from  the  normal  fissuration.  Berkley 
says  that  in  his  experience  it  is  the  most  striking  thing  on  gross  examina- 
tion of  the  brain  in  that  disease.  Really  no  other  gross  lesion  is  ever 
present. 

Cerebral  Heterotopia. — Occasionally  there  is  found  on  microscopic 
examination  of  a  brain,  usually  one  showing  gross  defects  or  abnormali- 
ties, small  gray  nodules  or  streaks  in  the  white  matter  of  the  cerebrum 
or  cerebellum  due  to  the  presence  of  tissue  rich  in  nerve-cells  and  poor 
in  medullated  fibres.  They  are  bits  of  gray  matter  out  of  place.  Van 
Gieson  has  shown  that  in  the  spinal  cord  heterotopia  is  frequently  an 
artefact,  occurring  while  the  cord  is  being  hardened  or  cut  and  mounted. 
This  is  not  always  the  case,  however.  Misplacement  of  tissue  may 
occur  in  the  brain  just  as  it  does  in  the  abdominal  organs.  Its  cause 
is  not  clear.  It  probably,  however,  is  caused  either  by  an  abnormal 
development  of  nerve-fibres  causing  the  detachment  of  a  small  bit  of 
gray  matter  from  its  proper  place,  or  by  the  primary  development  of 
gray  matter  in  such  a  situation  as  to  be  later  surrounded  by  white 
matter.  Occasionally  also  anomalous  bundles  of  white  fibres  are  found 
in  the  medulla.     Heterotopia  never  produces  any  symptoms. 

Congenital  Hydrocephalus. — External  hydrocephalus  is  an  accu- 
mulation of  cerebrospinal  fluid  within  the  subarachnoid  space.  It  is 
frequent  in  cerebral  atrophy.  In  internal  hydrocephalus  the  fluid  is 
within  the  ventricles.  It  is  caused  by  developmental  defect  of  struc- 
ture, intrauterine  inflammation  of  the  ependyma,  and  pathological  con- 
ditions of  the  blood.  It  may  occur  in  children  of  healthy  parentage 
and  whose  mothers  have  passed  through  pregnancy  without  any  illness. 
On  the  other  hand,  several  children  in  a  family  may  be  affected.     As  a 


552  THE    EYE    AND    NERVOUS    SYSTEM 

rule,  but  not  always,  the  head  shows  enlargement  at  birth.  Many  chil- 
dren with  this  disease  are  born  dead,  some  survive  for  a  short  time,  a 
few,  and  if  very  slight  cases  are  included,  a  quite  large  number,  attain 
full  maturity.  The  shape  of  the  head  is  characteristic :  the  cranium  is 
hemispherical  or  elipsoidal  and  the  face  appears  relatively  small.  The 
bones  of  the  vault  are  thin  and  separated.  Many  Wormian  bones  are 
present.  The  circumference  of  the  skull  varies  greatly.  It  has  been 
as  much  as  forty  inches.  The  amount  of  fluid  varies  greatly.  The 
lateral  ventricles  are,  of  course,  always  dilated  and  their  walls  may 
be  as  thin  as  paper,  the  upper  parts  of  the  hemispheres  being  mere  sacs. 
The  cortex  resists  the  pressure  better  than  the  white  matter,  but  it,  too, 
in  a  case  at  all  severe,  is  decreased  in  thickness.  The  membranes,  as  a 
rule,  are  normal  or  slightly  thinned.  If  the  cause  be  inflammatory  the 
choroid  plexuses  and  the  membrane  lining  the  ventricles  are  thickened. 
The  fontanelles  bulge  and  pulsate  with  the  heart-beat.  If  the  child 
survive,  many  nervous  symptoms  develop.  The  mental  symptoms  vary 
from  idiocy  to  high-grade  imbecility.  Some  very  slight  cases  present 
no  symptoms  at  all  except  the  deformity  of  the  skull.  Often  there  are 
cerebral  palsies  and  epileptiform  convulsions.  There  may  be  total 
blindness  from  atrophy  of  the  optic  nerves  produced  by  direct  pressure 
upon  the  chiasm  and  tracts.  Optic  neuritis  is  much  less  common. 
Strabismus  is  frequent.  The  diagnosis  presents  no  difficulty.  Treat- 
ment, either  medical  or  surgical,  has  so  far  availed  nothing. 

Defective  Development  of  the  Cranial  Nerve  Nuclei. — Gowers 
describes  under  the  title  "  infantile  oculofacial  palsy"  a  rare  condi- 
tion which  may  be  present  from  birth  or  appear  later,  characterized  by 
palsy  of  the  external  ocular  muscles,  paralysis  of  the  face,  and  ptosis, 
but  without  involvement  of  the  internal  ocular  muscles.  He  suggests 
that  it  is  due  to  defective  vital  endurance  in  the  nuclear  structures. 
Sachs,  using  Schapringer's  case  as  a  text,  shows  that  the  motor  branch 
of  the  fifth,  tlie  hypoglossal,  the  third,  the  sixth  and  seventh  nerve 
nuclei  may  all  be  affected.  On  the  other  hand,  only  the  nucleus  of 
one  facial  nerve  may  be  affected.  I  know  of  no  post-mortem  study  of 
this  condition,  but  it  would  seem  to  be  caused  by  a  true  defect  in 
development. 

Congenital  Absence  of  the  Visual  System. — This  is  a  very  rare 
condition.  Absence  of  the  visual  system  within  the  skull  never  occurs 
save  in  association  with  anophthalmos  or  microphthalmos.  It  is  difii- 
cult  to  tell  where  to  draw  the  line  separating  these  two  conditions.  E. 
Treacher  Collins  and  J.  H.  Parsons  lay  down  the  following  rule: 
When  there  is  complete  failure  of  the  essential  nervous  mechanism  of 


ANOMALIES  OF  BRAIN  AND   SKULL  553 

the  eyeball,  anophtlialmos  is  the  proper  term ;  when  any  of  the  nervous 
elements  are  present,  no  matter  how  imperfect,  microphthalmos.  Total 
absence  of  the  eyeball  is  probably  always  a  true  developmental  defect: 
microphthalmos  is  probably  sometimes  developmental,  sometimes  due 
to  intrauterine  disease.  Zentmayer  and  Goldberg  ^  describe  very  care- 
fully the  microscopic  appearances  in  a  case  of  microphthalmos.  More 
interesting  to  neurologists,  however,  are  the  studies  which  have  been 
made  of  absence  of  the  visual  system  within  the  cranium.  Quite  a 
number  of  cases  have  been  studied,  but  that  of  Spiller  ^  is  the  best,  and 
I  shall  quote  largely  from  it.  The  patient  was  an  idiot  boy  twenty-two 
years  old.  Spiller  had  regarded  him  in  life  as  a  case  of  cerebral  spastic 
paraplegia  with  absence  of  the  eyeballs.  At  autopsy  the  optic  foramina 
were  found  to  be  absent.  The  orbital  contents  were  not  allowed  to  be 
removed  and  hence  whether  there  was  a  rudimentary  eye  present  could 
not  be  determined.  Nothing  resembling  an  eyeball  was  seen.  No  trace 
of  optic  nerves,  chiasm,  or  optic  tracts  could  be  found.  There  was  no 
sign  of  an  external  geniculate  body  on  either  side,  and  the  thalamus 
on  each  side  had  nothing  resembling  an  optic  tract  passing  to  it. 
The  brain  was  small,  firm,  and  not  oedematous.  The  left  ascending 
frontal  convolution  in  the  centre  for  the  arm  was  very  small.  The  occip- 
ital lobes  and  the  cunei  were  very  small.  The  calcarine  fissure  was 
short.  The  external  arcuate  fibres  were  unusually  well  developed. 
The  spinal  cord  was  small,  but  otherwise  normal.  On  microscopic 
examination  the  cortex  of  the  left  calcarine  fissure  possibly  contained 
fewer  cells  than  normal.  The  optic  radiations  in  the  frontal  sections  of 
the  occipital  lobe  were  not  entirely  absent.  Spiller  draws  the  following 
conclusions :  The  chief  "  primary"  optic  centre  is  the  external  genicu- 
late body.  The  pulvinar  of  the  optic  thalamus  is  also  an  important 
"  primary"  optic  centre.  The  anterior  colliculus  of  the  quadrigeminal 
body  in  man  has  an  unimportant  relation  to  vision.  The  hypothalmic 
body,  the  habenula,  and  the  internal  geniculate  body,  probably  are  not 
parts  of  the  visual  system.  The  cortex  of  the  calcarine  fissure  may  con- 
tain nearly  the  normal  number  of  cell  bodies,  even  though  the  visual  sys- 
tem may  be  undeveloped.  The  nerves  to  the  ocular  muscles  and  their 
nuclei  may  be  developed,  even  though  the  visual  system  is  absent.  A 
condition  such  as  this  (See  Chapter  I,  Figs.  1  to  4)  is  developmental 
and  not  caused  by  acquired  disease,  but  as  to  the  cause  and  mechanism 
of  the  developmental  defect  we  know  nothing.     Nothing  is  known  with 


'  Annals  of  Ophthalmology,  January,  1904. 
University  of  Pennsylvania  Medical  Bulletin,  February,  1902. 


554  THE    EYE    AND    XERVOUS    SYSTEM 

any  certainty  as  to  any  hereditary  influence  which  might  act  as  a  pre- 
disposing cause.  All  the  cases  I  know  of,  have  occurred  in  people  with 
more  or  less  mental  deficiency.  It  is  sometimes  associated  with  cleft 
palate  or  harelip.  The  majority  of  the  patients  die  immediately  or  not 
very  long  after  birth.     Only  a  few  reach  maturity. 

Abnormalities  in  the  Development  of  the  Skull. — I  shall  consider 
only  the  defects  of  growth  which  cause  congenital  cranial  hernia  and 
the  abnormalities  which  are  associated  with  certain  ocular  disturb- 
ances. The  microcephalic  skull  has  already  been  described.  Cranial 
hernia  includes  encephalocele  (protrusion  of  the  brain  substance), 
hydrencephalocele  (encephalocele  containing  fluid  and  with  a  cavity 
usually  communicating  with  the  ventricles),  and  meningocele  (pro- 
trusion of  the  cerebral  membranes). 

Almost  always  the  hernia  occurs  between  bones  and  not  through  a 
hole  in  a  bone.  It  almost  always  occurs  in  the  median  line,  usually  in 
the  occipital,  rarely  in  the  frontal  region.  It  very  rarely  is  seen 
between  the  temporal  and  parietal  bones.  Cranial  hernia  produces  a 
tumor  under  the  scalp.  An  encephalocele  is  usually  opaque  and  with 
a  broad  base.  Meningocele  and  hydrencephalocele  are  translucent  and 
pedunculated.  It  is  justifiable  to  puncture  with  a  hypodermic  needle 
to  determine  whether  the  tumor  is  solid  or  contains  cerebrospinal  fluid. 
If  the  latter  be  present  the  tumor  must  be  intracranial  in  origin  and 
cannot  be  a  vascular  growth,  a  sebaceous  cyst,  or  an  abscess.  All  of 
these  have  been  mistaken  for  cranial  hernia.  Frequently  the  opening 
in  the  bone  can  be  felt,  the  mass  may  pulsate  with  the  heart  beat,  and 
may  increase  in  size  as  the  child  cries.  Various  other  anatomic  defects 
are  apt  to  be  present.  Convulsions  often  occur.  As  a  rule,  the  patients 
die  after  a  very  short  time.  In  meningocele  the  sac  has  been  success- 
fully removed. 

Congenital  Deformity  of  the  Skull  associated  with  Optic  Atrophy. 
— Post-neuritic  optic  atrophy  associated  with  deformity  of  the  skull 
has  been  reported  in  a  small  number  of  cases.  The  question  is  whether 
the  deformity  directly  causes  the  disease  of  the  nerves,  whether  both 
arise  from  a  common  cause,  or  whether  the  two  conditions  are  coinci- 
dental. All  authorities  agree  that  there  is  a  closer  relation  than  coin- 
cidence. According  to  Norris  and  Oliver,  vision  is  defective  in  greater 
or  less  degree  from  birth.  Some  are  born  blind,  others  have  fair  vision 
in  early  life,  but  slowly  sight  diminishes  until,  during  adolescence,  total 
amaurosis  is  reached.  Some  retain  a  little  vision  even  in  advanced 
life.  Virchow  and  Ilirshberg  attribute  the  optic  neuritis  to  some  intra- 
cranial inflammatory  trouble,  in  most  cases  probably  a  pachymeningitis, 


ANOMALIES  OF  BKAIX  AND  SKULL  555 

which  may  also  cause  the  cranial  deformity.  Friedenwald  ^  believes 
the  condition  is  caused  by  increased  intracranial  pressure  the  result 
of  premature  synostosis  of  the  cranial  bones.  Opposed  to  this  is  the 
fact  that  in  many  cases  of  microcephalus  there  is  no  disease  of  the  optic 
nerve.  !N^ecropsies,  of  which  there  have  been  three,  throw  some  light 
on  the  causation.  In  Michel's  case  there  was  hyperostosis  of  the  skull, 
narrowing  of  the  optic  foramina,  and  thickening  of  the  meninges;  in 
Manz's  the  base  of  the  skull  was  very  irregular  and  the  optic  foramina 
were  narrow;  in  Ponfick's  the  optic  foramina  were  small.  In  the  first 
two  cases  the  skulls  were  oxycephalic  (steeple-shaped)  ;  the  deformity 
in  the  third  I  do  not  know.  In  consequence  of  these  post-mortem  find- 
ings, Weiss  and  Brugger  examined  four  steeple-shaped  skulls  in  the 
Heidelberg  Institute.  They  found  the  optic  foramina  of  normal  width, 
but  the  orbital  cavities  reduced  in  size,  being  very  shallow,  and  the 
alteration  in  shape  being  caused  by  the  greater  wing  of  the  sphenoid 
forming  the  posterior  instead  of  the  lateral  wall  of  the  orbit.  The 
dorsum  sellae  turcica?  was  enlarged  and  pushed  forward,  which  they  sug- 
gest might  hav^e  caused  pressure  upon  the  chiasm  and  optic  nerves. 
Friedenwald,  in  discussing  their  paper,  points  out  that  such  pressure 
would  cause  a  simple,  not  a  neuritic  atrophy.  Deformities  other  than 
oxycephaly  may  be  associated  with  optic  atrophy.  In  Friedenwald's 
table  of  nineteen  cases,  twelve  were  oxycephalic,  three  long  skulls,  one 
leptocephalic,  one  with  broad  forehead  (synostosis  of  the  sagittal 
suture),  one  with  a  bulging  temporal  region,  and  one  in  which  the 
deformity  is  not  described.  Oliver  has  published  an  elaborate  paper 
•on  "  Blindness  from  Congenital  Malformation  of  the  Skull."  ^ 

Oliver  also  points  out  that  blindness  may  occur  in  association  with 
deformity  of  the  skull  without  any  disease  of  the  optic  nerve.  He 
reported  the  following  case.^  The  patient  was  a  four-year-old  girl  who, 
the  mother  stated,  could  see  a  little  till  her  third  year.  She  showed  no 
ophthalmoscopic  sign  of  disease.  The  eyes  were  prominent.  The  head 
was  disproportionately  small  and  quite  deformed  posteriorly.  The 
occipital  protuberance  was  almost  wanting.  The  occipitoparietal  suture 
was  not  well  closed,  the  thickened  and  serrated  edges  of  the  occipital 
and  parietal  bones  curving  outwardly.  Her  mental  condition  was  good. 
He  believes  the  blindness  was  cortical  in  origin,  and  suggests  as  a 
possible  cause  "  stretching  and  inflammatory  processes  taking  place  in 

*  American  Journal  of  the  Medical  Sciences,  May,  1893;  Archives  of  Ophthal- 
mology, 1901. 

"  Proceedings  of  the  American  Philosophical  Society,  vol.  xli..  No.  169. 

•  American  Journal  of  the  Medical  Sciences.  Januarv.  1902. 


556  THE    EYE    AND    NERVOUS    SYSTEM 

and  around  a  series  of  imperfectly  made  and  quite  readily  disturbed 
tissues  in  tlie  occipital  region."  The  most  frequent  cause  of  the  atrophy 
is  surely  that  described  by  Virchow  and  Hirshberg. 

PROGRESSIVE  FACIAL  HEMIATROPHY. 

This  aflFection  is  very  rare.  It  is  most  apt  to  begin  before  the  tenth 
year,  is  less  frequent  in  adolescence  and  very  rare  in  old  age.  In 
92  cases  collected  by  Hermann  Steinert,  60  cases  occurred  in  females, 
30  in  males,  and  in  two  the  sex  was  not  mentioned.  It  has  been  reported 
twice  in  parent  and  child.  Its  true  causes  are  unknown.  The  most 
frequent  preceding  event  which  could  stand  in  a  causal  relation  is 
trauma  of  the  face.  It  has  followed  the  acute  infectious  fevers  after 
a  longer  or  shorter  period,  and  has  occurred  during  the  course  of  many 
nervous  diseases, — e.g.,  hysteria,  multiple  sclerosis,  locomotor  ataxia, 
epilepsy,  and  syringomyelia.  Its  association  with  none  of  these  diseases, 
however,  has  been  more  frequent  than  can  be  accounted  for  by  the  law 
of  coincidence.  There  seems  to  be  a  relationship  between  it  and 
scleroderma.  The  two  diseases  occur  together  with  relative  frequency. 
Facial  neuralgia  so  often  precedes  the  appearance  of  the  atrophy 
that  it  may  properly  be  regarded  as  a  symptom  rather  than  an  asso- 
ciated disease. 

Little  is  known  as  to  its  morbid  anatomy  and  the  real  seat  of  disease. 
Mendel  had  one  autopsy.  The  patient  had  been  ill  twenty-five  years. 
Along  with  atrophy  of  the  face  there  was  atrophy  of  the  left  arm.  Post- 
mortem examination  revealed  proliferating  interstitial  neuritis  of  the 
left  trifacial,  most  marked  in  the  second  branch.  The  facial  was  nor- 
mal, but  the  left  musculospiral  was  affected  in  the  same  way  as  the 
trifacial.  In  an  atypical  case  reported  by  Ilomen  there  was  a  tumor 
pressing  upon  the  Gasserian  ganglion  and  the  branches  of  the  trifacial. 
There  was  not  only  facial  hemiatrophy  but  also  anaesthesia,  palsy  of  the 
oculo-motor  nerve,  and  atrophy  of  the  tongue.  There  was  degeneration 
of  all  branches  of  the  trifacial  nerve,  and  of  the  third,  fourth,  sixth, 
and  seventh  nerves.  Ileniiatrophy  may  also  follow  injury  to  the  fifth 
nerve.  Some  authors  tend  to  the  view  that  the  seat  of  the  disease  is  in 
the  cervical  sympathetic.  Thus  Seeligmuller  reports  a  case  following 
injury  in  that  region.  Certain  authors  regard  it  as  a  true  skin  disease. 
This  is  surely  an  error.  It  is  a  disease  of  nutrition  and  most  likely 
depends  upon  perversion,  not  mere  abolition,  of  function  in  the  fifth 
nerve  or  the  sympathetic  system,  or  both.  Congenital  predisposition 
seems  to  be  important  for  its  occurrence, — at  least  it  is  most  frequent 
in  the  neuropathic. 


PROGRESSIVE  FACIAL  HEMIATROPHY  557 

Symptomatology. — The  first  objective  sign  of  disease  appears  in  the 
skin.  A  circumscribed  spot  on  the  side  of  the  face  becomes  dead  white 
or  yellowish  and  at  the  same  time  the  skin  grows  thin.  Rarely  two 
spots  are  attacked  at  once.  The  disease  is  most  apt  to  commence  in  the 
orbital  region  or  the  lower  jaw.  Soon  there  is  a  loss  of  fatty  tissue 
under  the  skin  and  a  shallow  pit  is  formed.  The  affected  area  extends 
and  involves  not  only  the  skin  but  the  subcutaneous  tissues  and  bone, 
and  in  less  degree  the  muscles.  There  is  never  reaction  of  degeneration 
of  the  muscles  nor  complete  palsy.  The  fatty  tissue  of  the  orbit  disap- 
pears and  the  eyeball  sinks  in.  The  final  color  of  the  skin  is  a  shallow 
yellow  or  brown.  The  brow  and  beard  fall  out  or  become  white.  The 
hair  of  the  head  is  rarely  affected.  Perspiration  may  be  increased,  but 
the  secretion  of  the  sebaceous  glands  lessens  or  ceases.  The  disease  is 
progressive,  but  in  the  greater  number  of  cases  ceases  at  the  middle 
line.  It  may,  however,  pass  over  to  the  other  side.  The  neck  muscles 
and  the  arm  of  the  same  side  may  be  involved.  Except  at  the  begin- 
ning its  progress  is  very  slow,  and  though  recovery  never  occurs, — i.e., 
the  face  never  regains  its  natural  aspect  and  form, — the  process  may 
cease  at  any  time.  It  may  recur.  The  deformity  in  an  advanced  case  is 
marked  and  characteristic.  The  diseased  side  is  depressed  below  the 
level  of  the  other  from  the  forehead  to  the  chin.  T\^e  line  of  demarca- 
tion is  clear-cut. 

There  are  many  minor  symptoms.  Pain  or  itching  in  the  affected 
region  is  frequent.  The  tongue,  hard  and  soft  palate,  and  the  gums  may 
atrophy.  The  teeth  may  fall  out.  There  is  never  complete  anaesthesia, 
but  there  may  be  hypersesthesia.  The  atrophied  skin  may  feel  to  the 
patient  tense  and  mask  like.  Rarely  the  pupil  is  dilated  or  very  small. 
There  is  never  disturbance  of  vision,  but  taste  and  hearing  may  be 
impaired.  In  very  few  cases  neuroparalytic  ophthalmia  has  been 
observed.  Sachs  and  other  authors  refer  to  cases  in  which  there  were 
marked  spasm  of  the  masseters,  clonic,  tonic,  or  both. 

The  disease  does  not  shorten  life.  The  only  conditions  with  which 
it  can  be  confounded  are  congenital  facial  asymmetry,  facial  palsy,  and 
facial  hemihypertrophy  of  the  opposite  side.  These  need  only  to  be 
remembered  to  avoid  error.  It  may  be  associated  with  scleroderma, 
but  the  differentiation  is  not  difficult.  Scattered  through  the  litera- 
ture are  reports  of  atrophy  occurring  in  other  parts  of  the  body,  similar 
in  onset  and  progress  and  involving  the  same  tissues.  It  is  jirobable 
that  some  of  these  at  least  are  identical  pathologically  with  this 
disease. 

Treatment  has  no  influence. 


►58  THE    EYE    AND    NERVOUS    SYSTEM 


PROGRESSIVE   FACIAL   HEMIHYPERTROPHY. 

This  condition  is  still  rarer  than  hemiatrophy.  It  is  characterized 
by  an  overgrowth  of  one  side  of  the  face.  The  soft  parts  may  be  alone 
involved  or  together  with  the  bones.  It  usually  is  congenital,  but  may 
first  appear  in  childhood  or  youth.  Its  causation  and  morbid  anatomy 
are  unknown.  The  earliest  case  I  know  of  was  reported  by  Stilling  in 
1840.  The  hypertrophy  followed  an  injury  which  occurred  when  the 
patient  was  nine  years  old.  ^[ontgomery's  case  followed  two  years  after 
an  abscess  of  the  cheek.  It  has  followed  various  illnesses  and  without 
any  apparent  cause.  The  cases  reported  are  too  few  to  draw  any  con- 
clusions from  as  to  causation.  There  may  be  no  sign  of  disease  beyond 
the  mere  increase  in  size.  Only  the  soft  parts  with  or  without  the 
mucous  membrane  of  the  mouth  may  be  affected,  or  the  bones,  or  one 
or  more  of  them,  may  be  affected  also.  Only  a  part  of  the  face  may  be 
involved,  as  the  cheek.  In  Hoffman's  case,^  that  of  a  girl  of  fourteen 
years,  in  whom  the  change  was  first  noticed  at  two  years,  there  was  a 
growth  of  hair  on  the  upper  lip.  The  skin  may  be  normal  or  thickened 
and  pigmented.  Once  only  has  it  been  recorded  as  thick  and  coarse. 
It  rarely  is  hyperaemic.  There  are  no  characteristic  sensory  symptoms. 
Berger's  case,  however,  followed  an  old  trifacial  neuralgia.  Dana's 
case  occurred  in  a  young  giant.  The  sebaceous  and  sweat  glands  may 
be  overactive.  There  ma}'  even  be  an  increase  of  saliva  on  the  affected 
side.  There  are  no  ocular  defects  nor  disturbances  of  vision.  The 
only  changes  found  so  far  at  autopsy  have  been  hyperostosis,  uniform 
or  in  masses,  an  increase  in  the  adipose  tissue,  and  an  atrophy  of  muscle 
fibres.  It  is  progressive  during  youth  but  ceases,  or  almost  ceases,  at 
maturity.  It  never  causes  death.  Once,  by  pressure  of  the  thickened 
skull  upon  the  brain,  it  caused  epileptiform  convulsions. 

OTHER    PROGRESSIVE    HYPERTROPHIES. 

Occasionally  there  is  seen  an  hypertrophy  of  one  entire  half  of  the 
body,  or  of  one  arm  or  leg,  or  finger,  or  as  is  reported  in  one  case,  one 
side  of  the  face  and  the  corresponding  arm  and  the  leg  of  the  opposite 
side.  The  symptoms  are  precisely  the  same  as  in  the  facial  form  and 
it  is  probable  that  the  pathology  is  the  same.  It  seems,  however,  that 
the  latter  is  more  frequently  acquired  than  the  former.  All  the  tissues 
of  the  part  are  affected,  the  skin,  subcutaneous  soft  parts,  the  muscles 
and  the  bones.  Arnheim  indeed  describes  a  case  in  which  at  autopsy 
the  internal  organs  were  found  hypertrophied.     The  right  ovary,  kid- 

■  Deutsche  Zeitschrift  fiir  Nervenheilkunde,  vol.  xxiv.  p.  425. 


ACROMEGALY  559 

nej,  lymphatic  glands,  and  even  the  right  side  of  the  heart  were 
enlarged.  Hymanson  *  found  by  actual  measurement  that  in  his  case 
growth  was  much  more  rapid  on  the  affected  side.  We  know  nothing 
about  the  cause  of  the  condition,  but  Anderson  gives  the  following 
views  as  to  its  etiology:  (1)  A  congenital  lesion  of  the  vasomotor 
centres  leading  to  vascular  stasis,  (2)  a  primitive  vice  of  the  middle 
lamina  of  the  blastodermic  membrane,  (3)  partial  intrauterine 
strangulation  of  the  affected  member,  (4)  an  inherent  tendency  of  the 
tissues  to  appropriate  an  excess  of  nutriment.  England  found  at 
autopsy  on  a  child,  in  whom  the  left  side  of  the  face  and  head  was 
hypertrophied,  neurofibromata  involving  all  the  cranial  nerves. 

ACROMEGALY. 

Acromegaly  was  first  described  by  Pierre  Marie  in  1886.  Long 
before  that  time  many  writers  had  reported  cases  of  curious  deformity 
and  strange  alteration  in  physiognomy  coming  on  in  youth  or  adult 
life,  and  of  course  giantism  was  well  known ;  but  Marie  was  the  first 
to  separate  from  these  cases  of  acquired  abnormality  of  anatomic  struc- 
ture the  type  which  we  now  call  acromegaly  and  to  show  that  it  is  a 
distinct  disease  with  a  definite  symptomatology  and  probably  due  to  a 
single  cause.  It  is  of  interest  to  oculists  because  of  the  ocular  symptoms 
due  to  the  associated,  if  not  causative  disease  of  the  hypophysis,  and  'to 
neurologists  because  of  the  obscurity  of  its  pathology-  and  the  occurrence 
of  nervous  symptoms. 

The  most  striking  feature  of  the  disease^  that  which  makes  it  pos- 
sible to  diagnose  a  well  developed  case  at  sight,  is  the  alteration  in 
physiognomy  resulting  from  an  overgrowth  of  the  hands  and  feet,  the 
head,  jaws,  lips,  and  tongue  and  a  cervico-dorsal  spinal  curvature.  The 
most  frequent  first  subjective  symptom  is  headache,  dull  or  sharp,  local 
or  general,  paroxysmal  or  lasting.  The  change  in  bulk  is  first  called 
to  the  notice  of  the  patient  by  the  fact  that  he  must  wear  larger  and 
larger  shoes  or  gloves.  Even  before  this,  friends  may  observe  an  inex- 
plicable alteration  in  the  facial  expression. 

Causation. — The  predisposing  causes  are  unknown.  Direct  inheri- 
tance seems  to  play  no  part,  though  a  few  cases  have  been  reported  in 
brothers.  It  is  possible  that  in  them  pathological  incidents  during 
intrauterine  life  acted  as  predisposing  causes  rather  than  heredity. 
Most  frequently  the  cases  are  isolated,  one  in  a  family.  The  influence 
of  race  is  unknown,  but  it  seems  to  be  very  rare  in  the  American  negro, 

*  Archives  of  Pediatrics,  vol.  xx.  p.  428. 


560  THE    EYE    AND    NERVOUS    SYSTEM 

Berkley  alone,  so  far  as  I  know,  having  reported  a  case.  According  to 
the  tables  of  Hinsdale,  males  are  more  predisposed  than  females;  ac- 
cording to  Dana,  the  two  sexes  are  aflFected  with  about  equal  frequency. 
The  period  of  greatest  frequency  of  onset,  so  far  as  that  can  be  deter- 
mined in  a  disease  so  insidious  in  its  development,  is  from  the  twentieth 
to  the  thirtieth  year.  A  few  cases  have  been  reported  in  which  the  onset 
occurred  after  the  seventieth  year  and  recently  there  have  been  pub- 
lished instances  in  which  the  disease  was  alleged  to  be  congenital.  It 
is  by  no  means  certain  that  these  are  genuine  or  that  they  should  not  be 
classed  elsewhere.  They  may  bear  the  same  relation  to  the  acquired 
disease  that  infantile  cretinism  bears  to  myxoedema.  The  relation  of 
giantism  and  acromegaly  is  much  discussed.  They  certainly  are  not 
the  same  thing  and  the  latter  is  not  infrequent  in  the  former. 

The  exciting  causes  are  also  unknown.  The  rule  is  for  the  disease 
to  develop  without  the  previous  occurrence  of  anything  which  could  be 
assumed  to  be  a  cause.  It  may  follow  an  acute  infectious  fever,  rheu- 
matism, trauma,  childbirth,  prolonged  emotional  strain  and,  indeed, 
any  disease.  It  sometimes  occurs  in  imbeciles  and  epileptics.  One 
thing  is  sure,  alcohol  and  syphilis  are  not  exciting  causes.  If  they 
were  it  would  be  much  more  common.  I  shall  discuss  the  relation 
between  disease  of  the  hypophysis  and  acromegaly  under  the  heading, 
morbid  anatomy. 

Symptomatology. — The  usual  picture  is  as  follows:  Some  time 
between  late  adolescence  and  middle  life  the  hands  and  feet  begin  to 
enlarge  and  the  face  to  alter.  At  the  same  time  headache  begins,  is 
much  treated  and  persists.  The  lips  and  eyelids  become  thickened,  the 
ears  enlarged,  the  nose  broadened,  lengthened,  and  the  septum  thick- 
ened. The  mucous  membranes  hypertrophy  and  the  tongue  becomes 
unwieldy  from  its  increased  bulk.  While  these  changes  are  occurring 
in  the  superficial  tissues,  similar  ones  take  place  in  the  bones  of  the 
skull  and  especially  in  the  face.  The  lower  jaw  enlarges  so  much  as 
to  cause  prognathism,  the  line  of  its  circumference  extending  far  beyond 
the  upper  jaw.  The  cheek  bones  become  prominent.  The  voice  grows 
hoarse  and  deep.  The  skull  increases  only  moderately  in  circumfer- 
ence, but  the  fingers  lengthen  and  broaden  and  become  like  spades. 
Visual  disturbances  appear,  contraction  of  the  fields  or  hemianopsia. 
The  patient  becomes  dull,  lethargic,  stupid,  unfit  for  any  work.  A 
true  melancholia  may  appear.  In  the  meanwhile  the  vertebra3  have 
become  affected  and  cervicodorsal  kyphosis  sets  in.  Finally  the 
patient  dies  from  the  disease  of  the  hypophysis,  with  symptoms  of  brain 
tumor  or  from  some  intercurrent  affection.    The  duration  of  the  disease 


Fig.  3. — Acromegaly,  showing  facial  deformity  and  marked  dorso-cervical  kyphosis,  (Case  of  Dr. 
F.  A.  Packard.  American  Journal  of  the  Medical  Sciences,  Jan.-June,  1S92.  For  photograph  of  brain 
from  this  case,  see  chapter  ix,  page  428j. 


ACROMEGALY  561 

is  very  variable,  and  using  this  as  a  means  of  classification  Sternberg 
divides  it  into  three  varieties,  namely,  the  benign,  which  lasts  about 
fifty  years  and  in  which  the  changes  are  very  slight,  the  usual  chronic 
form  having  a  duration  of  from  eight  to  thirty  years,  and  an  acute 
malignant  form,  in  which  death  usually  occurs  within  four  years.  In 
the  last  the  hypophysis  is  always  sarcomatous.  ^Notwithstanding  the 
high  mortality  of  the  disease  and  its  tendency  to  slowly  but  surely 
progress,  in  some  cases  the  pathologic  process  ceases  and  in  that  sense 
the  patient  recovers.     The  lesions  already  produced  of  course  remain. 

Even  at  the  expense  of  a  little  repetition  the  symptoms,  both 
objective  and  subjective,  deserve  individual  study.  The  objective  symp- 
toms are  the  more  important  in  diagnosis.  Most  striking  is  the  change 
in  the  general  appearance  of  the  patient,  caused  by  the  thickening  of 
the  cutaneous,  subcutaneous,  and  in  less  degree,  the  bony  structures  of 
the  hands,  feet  and  skull.  As  a  rule,  the  enlargement  begins  simul- 
taneously in  all  parts,  but  occasionally  one  extremity  is  affected  before 
the  others.  The  fingers  and  toes  enlarge  more  in  circumference  than 
in  length.  They  become  sausage-shaped,  and  the  broadened  hands  spade- 
like. The  disproportion  between  the  much  enlarged  hands  and  the 
normal  arms  and  forearms  is  so  marked  as  to  be  grotesque.  On  the 
palms  and  soles  the  epidermis  and  true  skin  is  much  thickened  and 
pads  form.  The  nails  are  flattened  and  striated  longitudinally.  The 
orbital  ridges  and  the  external  occipital  protuberance  are  marked.  The 
increase  in  the  circumference  of  the  skull  is  largely  due  to  enlargement 
of  the  sinuses,  but  sometimes  the  cranial  bones  are  thickened. 

The  bony  changes  in  the  thorax  are  characteristic.  .  As  stated  above, 
cervicodorsal  kyphosis  is  common, — sometimes  it  is  so  marked  that  the 
ribs  touch  the  brim  of  the  pelvis.  The  sternum  is  thickened  and 
widened,  the  ensiform  being  very  prominent.  The  increased  thickness  of 
the  manubrium  is  important  to  remember,  because  the  percussion  note 
which  it  gives  may  be  supposed  to  be  due  to  a  persisting  thymus  gland 
beneath  it.  Erb  regards  sternal  dulness  as  pointing  strongly  to  the 
presence  of  the  thymus  gland.  This  sign  is  surely  not  infallible.  The 
clavicles  and  ribs  are  massive.  On  account  of  the  changes  in  the  bones 
of  the  thorax  its  shape  is  much  altered.  Its  anteroposterior  diameter 
becomes  proportionally  greater  than  the  lateral.  The  pelvic  bones  and 
the  great  joints  of  the  body  are  not  affected.  Except  in  the  so-called 
giant  type  of  the  disease  the  long  bones  remain  normal.  The  neck  is 
thickened  and  the  abdominal  walls  are  sometimes  pendulous. 

The  ocular  symptoms  are  important.  The  eyeballs  are  often  prom- 
inent and  there  may  be  extreme  exophthalmos,  due  probably  to  an 
36 


562  THE    EYE    AND    NERVOUS    SYSTEM 

increase  of  the  fatty  tissue  behind  the  bulb.  Hinsdale  quotes  Burchard 
as  reporting  one  case  in  which  the  eyeball  itself  was  enlarged.  Rarely 
severe  ocular  neuralgia  occurs.  Nystagmus  is  sometimes  seen.  There 
may  be  partial  or  complete  palsy  of  the  third  and  sixth  nerves.  The 
irides  may  not  react  to  light  at  all  or  hemianopic  iritic  rigidity  may 
be  present.  Some  visual  disturbance  always  exists.  There  may  be  only 
slight  dimness  of  vision  or  absolute  blindness  consequent  upon  optic 
atrophy  due  to  papillitis  caused  by  enlargement  or  tumor  of  the  hypoph- 
ysis. The  form  and  color  fields  may  be  concentrically  contracted  or 
there  may  be  true  hemianopsia.  Disease  of  the  hypophysis  and  possi- 
bly, as  Broca  believes,  bony  changes  in  the  optic  foramina  consequent 
upon  the  overgrowth  of  the  lesser  wing  of  the  sphenoid  bone  may  pro- 
duce bitemporal  hemianopsia, — i.e.,  blindness  in  the  nasal  half  of  each 
retina.  Homonymous  hemianopsia  has  been  described  by  de  Schwein- 
itz,  among  others.  The  type  of  hemianopsia  present  depends  upon  the 
direction  of  the  pressure  upon  the  optic  chiasm  and  optic  nerves.  Some- 
times there  is  total  blindness  in  one  eye  and  hemianopsia  in  the  other. 
The  ophthalmoscopic  picture  is  the  same  as  is  found  in  tumor  of  the 
brain  in  any  region. 

The  larynx  is  enlarged  in  all  directions,  the  mucous  membrane  and 
the  cartilages  thickened,  making  the  voice  deep  and  hoarse.  The 
thyroid  may  be  enlarged  or  diminished,  hypertrophied  or  sclerosed. 
Very  rarely  Graves's  disease  occurs.  Menstruation  in  women  and 
sexual  desire  and  power  in  men  are  apt  to  disappear  early.  At  first 
muscular  power  is  retained  or  even  increased,  but  later  there  is  marked 
weakness.  The  knee-jerk  is  usually  normal.  It  may  on  account  of 
complications  be  absent,  increased,  or  unequal  on  the  two  sides.  Elec- 
tric conductivity  is  increased.  The  urine  frequently  contains  albumin 
and  glucose  and  may  be  enormously  increased  in  quantity.  The  blood 
presents  no  noteworthy  changes.  Disturbances  of  sensibility  do  not 
occur  in  uncomplicated  cases,  but  hysterical  hemiana^sthesia  has  been 
noted.  Extreme  hunger  and  thirst  are  not  uncommon.  There  is  often 
cardiac  enlargement  with  dilatation  causing  paroxysmal  palpitation. 
The  characteristic  mental  state  is  one  of  apathy,  lack  of  energy,  and 
marked  sleepiness.  There  is  almost  always  some  emotional  depression 
which  may  pass  into  a  true  melancholia.  Some  patients  become  simply 
a  little  silly,  mildly  demented,  and  unduly  cheerful  and  good  natured. 

Morbid  Anatomy  and  Pathology. — The  skin,  subcutaneous  tissues,  and 
the  bones  in  the  affected  regions  of  course  always  show  marked  signs 
of  disease.  There  is  a  true  hypertrophy  of  the  skin  and  the  sweat  and 
sebaceous  glands,   of  the   arterial   walls,   and   of  the   sheaths  of  the 


Fig.  5.— Showing  deformity  of  hand.    (Case  of  Dr.  F.  A.  Packard.) 


Fig,  6.— a.  Photograph  of  the  patient  at  twenty-two  years  of  age.    b.  Photograph  at  forty-five  years  of 
age.    (Case  of  Dr.  F.  A.  Packard.) 


Via.  ".—Portion  of  skiapraph  of  left  hand  of  Pr.  Walton's  case  of  acromegaly.    (Actual  size.) 


ACKOMEGALY  563 

cutaneous  nerves.  In  the  bones  the  periosteum  is  thickened,  the  grooves 
of  the  blood-vessels  deepened  and  enlarged,  and  in  the  skull  and  spine 
small  extoses  are  present.  The  bones  are  thickened,  and  since  this  does 
not  involve  the  whole  bone  symmetrically,  deformity  ensues.  The  lower 
jaw  is  always  most  affected.  In  the  spine  the  vertebrae  are  ankylosed 
by  bony  overgrowth.  The  smaller  joints  present  appearances  similar  to 
those  seen  in  arthritis  deformans.  In  a  case  of  Broadbent's,  X-ray  exam- 
ination showed  that  there  was  enlargement  of  the  soft  parts  only,  the 
bones  being  of  normal  size.  This  may  mean,  however,  not  that  disease 
of  the  bone  is  not  an  inherent  part  of  acromegaly,  but  that  in  this  case 
the  skin  became  affected  first,  as  indeed  may  be  the  rule. 

The  organ  most  discussed  in  all  studies  on  acromegaly  is  the  hypoph- 
ysis. It  is  more  frequently  found  diseased  than  any  other  organ  in  the 
body,  and  not  a  few  authorities  hold  that  if  it  be  normal  acromegaly 
cannot  occur.  They  explain  the  few  cases  in  which  normal  hypophyses 
have  been  found  at  necropsy  by  claiming  errors  in  clinical  diagnosis. 
The  following  seem  to  be  the  facts  in  the  matter.  The  hypophysis  is 
diseased  in  the  great  majority  of  cases  if  not  in  all.  It  may  be  hyper- 
trophied  or  the  seat  of  a  tumor.  On  the  other  hand,  disease  of  it  com- 
pletely destroying  its  glandiilar  structure  and,  of  course,  also  its  rudi- 
mentary nervous  structure,  may  occur  without  any  symptom  of  acro- 
megaly appearing.  Such  disease  may  occur  at  any  period  of  life  and  last 
for  quite  a  long  time.  For  example,  a  patient  of  mine  who  had  adiposis 
dolorosa  showed  at  necropsy  a  tumor  as  large  as  a  walnut.  Her  illness 
had  lasted  many  months.  The  kind  of  disease  affecting  the  hypophysis 
seems  to  be  a  matter  of  no  moment  since  hypertrophy  and  tumors  of 
many  kinds  occur.  Some  writers  hold  that  simple  hypertrophy  of  the 
hypophysis  is  only  a  symptom  of  the  disease,  and  not  causative.  Exper- 
imental destruction  of  it  in  lower  animals  does  not  produce  acromegaly. 
This  may,  however,  be  due  to  the  fact  that  death  is  apt  to  follow  very 
quickly,  that  the  hypophysis  may  have  other  functions  in  lower  ani- 
mals than  in  man,  and  that  destruction  of  function  need  not,  indeed 
ought  not,  to  cause  the  same  symptoms  as  perversion  of  function,  such 
as  may  occur  in  a  diseased  but  not  totally  destroyed  organ.  Altogether 
it  is  best  to  confess  ignorance  and  admit  that  the  true  relation  between 
acromegaly  and  hypophyseal  disease  remains  unexplained.  Others  of 
the  so-called  ductless  glands  are  often  found  diseased,  notably  the  thy- 
roid. The  thymus,  which  should  not  be  discoverable  in  an  adult,  is 
often  present  and  the  seat  of  a  tumor.  The  ovaries  and  testicles  are 
often  atrophied.  Slight  and  probably  coincidental  changes  in  the  spinal 
cord,  degeneration  of  the  posterior  and  lateral  tracts  have  been  found. 


564  THE    EYE    AND    NERVOUS    SYSTEM 

The  spinal  and  sympathetic  ganglia  have,  when  examined,  shown  hyper- 
trophy. Syringomyelia  has  been  reported,  but  surely  cannot  have  any 
causative  influence.  A  few  investigators  have  endeavored  to  throw  light 
on  the  pathology  of  acromegaly  by  chemical  studies,  but  so  far  with 
little  success.  Edsall  and  Miller,  for  example,  made  a  very  careful 
study  of  the  urine  in  two  cases,  but  could  only  show  that  there  is  "  a 
growth  of  abnormal  bone  rather  than  a  mere  abnormal  growth  of  bone, 
and  that  there  are  very  marked  abnormalities"  [chemical]  "  in  the  soft 
tissues."  All  explanations  of  acromegaly  heretofore  offered  have  been 
purely  hypothetical,  except  that  of  its  hypophyseal  origin  which,  while 
not  proven,  certainly  has  evidence  in  its  favor. 

Differential  Diagnosis. — A  fully  developed  typical  case  of  acromegaly 
can  be  diagnosed  at  sight.  There  are,  however,  several  diseases  which 
may  in  fact  be  pathologically  nearly  related  to  it  and  which  may  be 
mistaken  for  it  unless  a  careful  examination  be  made.  Myxoedema,  for 
example,  has  a  slight  superficial  resemblance  to  it,  but  the  myxoedema- 
tous  swelling  involving  the  entire  body,  the  local  tumefactions  partic- 
ularly in  the  suprascapular  region,  the  puffed  face  with  obliteration  of 
the  lines  of  expression,  and  the  brittle,  thin  hair  are  points  quite  suflS- 
cient  to  serve  for  diagnosis.  Many  giants  are  acromegalic,  but  in  simple 
giantism  there  is  symmetrical  overgrowth  of  the  entire  body  and  not 
of  the  extremities  alone.  So-called  partial  giantism,  hypertrophy  of 
individual  extremities,  is  recognized  by  its  limitation  to  one  part.  In 
osteitis  deformans  (Paget's  disease)  the  shafts  of  the  long  bones  are 
chiefly  affected,  and  in  the  head  the  bones  of  the  cranium,  not  those  of 
the  face.  In  Paget's  disease  the  face  is  triangular  with  the  base  up,  in 
acromegaly  it  is  egg-shaped.  In  Paget's  disease  also  changes  in  the  soft 
tissues  are  not  present  and  the  symptoms  rarely  appear  before  the 
fiftieth  year.  In  leontiasis  ossea  the  cranial  bones  alone  are  affected. 
Adiposis  dolorosa  is  distinguished  by  the  local  swellings,  the  pain,  the 
absence  of  deformity,  and  of  ocular  symptoms.  The  most  difficult 
differential  diagnosis  of  all  is  that  from  pulmonary  hypertrophic  osteo- 
arthropathy. This  disease  also  was  discovered  by  Marie.  In  it  the 
bones  are  more  affected  than  the  soft  parts.  The  wrists  are  greatly 
deformed  while  the  lower  jaw  escapes.  Kyphosis  is  rare,  scoliosis  fre- 
quent. The  hands  are  paw-shaped.  There  is  always  some  pulmonary 
affection.     X-ray  examination  is  useful  in  diagnosis. 

Treatment. — Nothing  has  been  found  which  influences  the  course  of 
the  disease.  Thyroid  and  hypophyseal  extracts  have  no  effect.  At- 
tempts to  remove  the  tumor  of  the  hypophysis  have  been  followed  by 
death  in  a  short  time. 


180  360 


270 


Obsen-ed  in  1885. 


Left  ( 


,.d  to  tt>e 'MB 'TJ 


Observed  in  1S92. 
Fig.  8 Visual  fields.    (Dr.  F.  A.  Packard's  case  of  acrom^aly.) 


CHAPTER    XV. 

DISEASES   OF    THE    SPINAL    CORD    AND    SPINAL    NERVES. 

By  T.  H.  WEISENBURG,  M.  D. 

MYELITIS. 

Myelitis  is  inflammation  of  the  substance  of  the  spinal  cord.  We 
have  the  acute,  subacute,  and  chronic  forms,  depending  upon  the  mode 
of  the  onset.  \Mien  the  inflammation  involves  the  whole  thickness  of 
the  cord  for  one  or  more  segments,  it  is  called  transverse  myelitis ;  if 
more  extensive  in  continuous  areas,  we  have  the  diffuse  type;  and  if  the 
process  is  at  various  levels,  it  is  called  a  disseminated  myelitis. 

Etiology. — The  exact  cause  of  acute  myelitis  is  not  known,  but  it  is 
believed  to  be  an  acute  infectious  disease  due  to  a  specific  germ.  In 
several  instances  staphylococci  and  streptococci  have  been  found  in 
the  cerebrospinal  fluid,  and  the  pneumococcus  has  been  found  in  a 
case  of  disseminated  myelitis.  Exposure  to  cold  and  wet,  overexer- 
tion, and  the  acute  infectious  diseases,  such  as  smallpox,  diphtheria, 
typhoid  fever,  pneumonia,  and  gonorrhcea,  some  of  the  chronic  blood 
conditions,  and  even  gout,  may  cause  myelitis,  while  syphilis  is  one  of 
the  most  frequent  causes. 

Pathology. — Microscopically,  the  appearance  depends  upon  the 
stage  of  the  disease.  In  the  acute  period  the  blood-vessels  are  dilated 
and  filled  with  red  and  white  corpuscles.  Around  the  vessels  are  found 
leucocytes  and  accumulations  of  round  nuclei,  and  occasionally  hemor- 
rhages are  seen.  The  nerve-cells  are  in  various  stageg  of  disintegration. 
The  axis  cylinders  are  swollen  and  their  myelin  sheaths  destroyed.  The 
neurogliar  fibrils  are  swollen,  and  their  nuclei  are  much  increased  in 
number.  Granular  cells  are  found  in  abundance  in  the  neurogliar  tissue 
and  around  the  blood-vessels. 

Symptoms. — The  symptoms  of  acute  or  subacute  myelitis,  with 
which  we  are  concerned,  depend  upon  the  part  of  the  spinal  cord  affected. 
Usually  the  dorsal  cord  is  most  intensely  involved.  The  symptoms 
may  come  on  rapidly,  sometimes  in  a  few  hours,  or  may  come  on  grad- 
ually, lasting  for  some  weeks  or  months.  There  may  be  a  feeling  of 
malaise,  and  even  a  rise  of  temperature,  and  the  patient  may  complain 
of  a  feeling  of  numbness  or  of  a.  tingling  sensation  in  the  limbs,  some- 
times, also,  of  a  pain  in  the  back.     Following  this,  or  perhaps  without 

565 


56G  THE    EYE    AND    NERVOUS    SYSTEM 

these  pains,  there  is  a  feeling  of  weakness  in  the  extremities,  which  is 
rapidly  followed  by  complete  flaccid  paralysis.  At  times  a  total  paraly- 
sis comes  on  within  a  few  hours.  Sensation  in  all  forms  is  much 
affected,  the  upper  limit  of  the  anaesthesia  depending  upon  the  location 
of  the  lesion.  The  reflexes  may  be  lost  at  first ;  but  in  a  short  time  the 
limbs  will  become  spastic,  the  reflexes,  such  as  the  patellar  and  the 
Achilles  jerks  will  be  much  increased,  and  the  Babinski  phenomenon  is 
obtained.  The  vesical  and  rectal  sphincters  are  involved  from  the 
beginning,  there  being  at  first  retention  of  urine  and  constipation,  fol- 
lowed by  incontinence.  Trophic  disturbances,  such  as  bed-sores,  soon 
appear.  Wasting  of  muscles  and  the  reactions  of  degeneration  are 
obtained,  provided  the  cells  of  the  anterior  horns  supplying  these  parts 
are  destroyed.     Lumbar  myelitis  causes  somewhat  different  symptoms. 

If  the  infection  is  intense,  the  disease  extends  rapidly,  soon  affect- 
ing the  cells  supplying  the  respiratory  muscles  and  causing  death.  If 
the  patient  survives,  the  course  of  the  symptoms  will  depend  upon  the 
severity  of  the  lesion.  Usually  sensation  will  return  before  motion,  and 
the  patient  will  gradually  show  the  symptoms  of  spastic  paraplegia. 

Ocular  Symptoms. — Inflammation  of  the  optic  nerve  accompanying 
myelitis  is  rare.  Steffan  and  Erb,^  in  1876,  first  described  such  an 
occurrence.  Katz,^  in  1896,  collected  twenty-one  cases.  Since  that 
time  seventeen  have  been  recorded,  to  which  the  author  is  able  to  add 
one  case  of  his  own.  This  makes  a  total  of  thirty-nine  cases ;  and  when 
the  large  number  of  instances  of  myelitis  are  considered,  the  rarity  of 
the  affection  is  shown.     (See  Chapter  VIII,  p.  375). 

In  the  majority  of  instances  the  disease  first  manifests  itself  in  a 
rapid  diminution  of  vision,  mostly  in  both  eyes,  but  sometimes  only 
in  one.  Often  some  time  elapses  before  the  second  eye  is  affected, 
which  is  frequently  not  until  the  first  is  on  the  way  to  recovery. 

The  diminution  of  vision  is  usually  rapid.  In  one  case,  after 
twenty-four  hours  there  was  complete  amaurosis.  In  other  instances 
a  few  days,  and  sometimes  a  number  of  months,  elapse  before  there 
is  complete  loss  of  vision.  In  the  author's  case  one  month  passed  before 
there  was  complete  blindness. 

It  is  not  always  necessary  to  have  subjective  symptoms  of  diminu- 
tion of  vision,  for  in  a  number  of  reported  cases  the  patients  did  not 
complain  of  such  phenomena,  and  even  the  ophthalmoscopic  examina- 
tion may  be  negative,  the  diagnosis  of  optic  neuritis  being  made  by  the 
microscope. 

'  Arch.  f.  Psych,  u.  Nen'enkr.,  vol.  x.  Xo.  1. 
'Arch.  f.  Ophthalm.,  vol.  42. 


MYELITIS  567 

Usually  the  symptoms  are  ushered  in  by  pains  in  the  orbit  and  in 
the  frontal  region  of  the  affected  side.  They  are  increased  by  movement 
of  the  eyeball,  and  there  is  sometimes  acute  pain  on  pressure.  These 
pains  have  been  variously  explained.  Some  authors  believe  that  they 
are  due  to  an  inflammation  of  the  optic  nerve  sheaths  or  to  a  periostitis 
of  the  optic  foramen.  They  have  also  been  attributed  to  that  form  of 
inflammation  of  the  optic  nerve  known  as  retrobulbar  neuritis.  Most 
probably  they  are  due  to  the  irritation  of  the  sensory  branch  of  the  fifth 
nerve,  which  supplies  the  optic  sheath.     (See  Chapter  XVIII,  p.  743.) 

In  the  majority  of  instances  the  optic  neuritis  precedes  the  mye- 
litis, sometimes  by  a  few  days,  or  from  three  to  five  months.      The 


Fig.  1. — Case  of  meningo-myelitis  with  bilateral  optic  atrophy  secondary  to  optic  neuritis. 
Position  of  hands  shows  extent  of  power  of  elevation. 

symptoms  may  come  on  together,  or  the  optic  neuritis  may  follow  the 
myelitis,  as  in  the  author's  case,  after  three  months. 

The  condition  of  the  pupils  varies.  Usually  they  are  wider  than 
normal  and  the  reaction  to  light  is  lost.  This  has  been  explained  by 
the  existing  amaurosis.  The  pupils,  however,  may  be  unequal,  or  one 
or  both  may  be  myotic.  If  the  myelitis  involves  the  cervical  cord,  espe- 
cially the  eighth  cervical  and  first  dorsal  segments,  there  may  be  a  con- 
traction of  the  pupil,  narrowing  of  the  palpebral  fissure,  and  enoph- 


568  THE    EYE    AND    NERVOUS    SYSTEM 

thalmus,  due  to  paralysis  of  the  cervical  sympathetic.  Irritation  of 
the  sympathetic  produces  widening  of  the  palpebral  fissure,  exophthal- 
mos, and  dilation  of  the  pupil — as  in  one  case  (Bielchowsky),  in  which 
exophthalmos  and  swelling  of  the  lids  were  seen.  Nystagmus  has  been 
mentioned  but  once,  by  Brissaud  and  Brecy ;  ^  it  being  vertical  in 
direction.  Except  in  one  instance,  in  which  there  was  redness,  nothing 
pathological  has  been  found  in  the  external  part  of  the  eye. 

Disturbances  of  the  fields  of  vision  and  of  color-sense  are  not 
numerous,  because  the  blindness  is  usually  rapid  and  in  all  parts  of  the 
field.  Central  scotoma,  a  concentrically  contracted  field  of  one  eye, 
or  a  quantitative  light  disturbance  in  a  part  of  the  field,  have  been 
described.  Disturbances  of  color-perception  have  been  mentioned  a 
number  of  times,  as  well  in  the  beginning  as  in  the  end  of  the  disease. 
In  one  instance  a  unilateral  color-blindness  was  found  after  the  optic 
neuritis  had  disappeared. 

Involvement  of  the  other  cranial  nerves  is  rare.  Mention  is  made 
of  it  in  only  four  cases.  The  facial  nerve  was  involved  in  one  instance, 
while  in  Another  both  internal  recti  muscles  and  the  seventh  and  twelfth 
nerves  were  implicated.  In  the  other  two  cases  there  was  paralysis  of 
both  internal  and  external  recti  muscles,  ptosis,  and  paresis  of  the  facial 
and  hypoglossal  nerves.  In  several  reported  cases  some  of  the  peripheral 
nerves  were  affected,  such  as  the  median,  sciatic,  and  external  popliteal ; 
microscopical  examination  showed  an  acute  neuritis,  similar  to  the 
inflammation  of  the  cranial  nerves. 

Ophthalmoscopically,  we  usually  find  in  the  beginning  of  the  neu- 
ritis more  or  less  swelling  of  the  papillse,  tortuosity  of  the  vessels,  and 
blurring  of  the  edges  of  the  disk.  This  is  by  no  means  always  the  case, 
for  the  ophthalmoscopical  findings  may  be  negative  all  through  the  dis- 
ease. The  retina  is  usually  not  affected.  Haziness  in  the  region  of  the 
macula  and  an  increasing  pigmentation  of  the  pigment  epithelium  have 
been  described.  ^licroscopical  examination  does  not  explain  the  latter 
condition.  Some  authors  consider  these  changes  as  due  to  a  degenera- 
tion of  the  retina. 

Both  optic  nerves  are  involved  in  the  majority  of  instances.  Two 
cases,  however,  are  recorded  in  which  only  one  of  tlie  nerves  was  dis- 
eased, but  in  one  of  these  cases  there  was  an  overfilling  of  the  vessels 
of  the  opposite  eye. 

After  the  disturbance  of  vision  has  reached  its  highest  stage — 
this  lasting,  on  an  average,  about  two  weeks — there  is  usually  an  im- 

'  Rev.  Neurol.,  Jan.  30,  1904. 


MYELITIS  560 

provement  if  the  case  is  not  fatal ;  and,  rarely,  a  return  of  vision.  Only 
three  eases  are  on  record  in  which  the  return  of  vision  was  complete. 
Usually  complete  blindness  in  both  eyes  results. 

Ophthalmologically,  in  about  half  the  cases  there  is  a  resulting 
white  atrophy  of  the  optic  nerves;  neuroretinal  changes  have  been  ob- 
served in  two  instances,  and  in  three  the  ophthalmological  findings  after 
the  subsidence  of  the  ocular  symptoms  were  completely  normal,  but  in 
none  of  these  three  cases  were  the  symptoms  severe. 

Remissions  of  the  symptoms  have  been  noted  in  five  instances. 
These  occurred  simultaneously  in  the  eye  and  in  the  cord,  and  have 
been  explained  in  various  ways.  Elschnig  *  believes  that  the  patho- 
logical process  in  the  optic  nerve  is  in  the  nature  of  a  primary  interstitial 
neuritis ;  and  that  the  inflamed  interstitial  tissue  exerts  a  mechanical 
pressure  upon  the  nerve-fibres  and  the  vessels,  thereby  interfering  with 
their  function.  Should  the  inflammation  subside,  function  would  be 
restored.  Bielchowsky,  however,  thinks  that  the  process  is  a  primary 
parenchymatous  neuritis ;  and  that  the  remissions  occur  because  the 
toxin  causing  the  disease  is  diminished,  and  the  axis  cylinders  regener- 
ate, thus  restoring  function. 

Of  the  39  reported  cases  of  myelitis  associated  with  optic  nerve 
changes,  20  have  been  with  autopsy.  It  is  striking  that  in  nearly  all 
the  myelitic  process  involved  almost  the  whole  extent  of  the  spinal 
cord.  The  process  in  all  the  cases  was  either  acute  or  subacute,  both 
in  the  spinal  cord  and  in  the  optic  nerves,  and  not  chronic. 

In  most  instances  the  nature  of  the  inflammation  is  that  of  an  acute 
disseminated  myelitis.  Transverse  myelitis  is  of  rare  occurrence.  His- 
tologically, the  myelitis  is  either  in  the  nature  of  an  acute  diffuse  paren- 
chymatous inflammation  or  a  combined  parenchymatous  and  inter- 
stitial process.  Bielchowsky,®  in  a  thorough  microscopical  examination 
of  four  cases,  found  three  distinct  pathological  processes.  In  his  first 
case  the  inflammation  was  of  the  acute  hemorrhagic  type,  affecting  the 
whole  extent  of  the  spinal  cord.  Here  the  inflammation  was  as  much 
interstitial  as  parenchymatous.  In  the  second  case,  the  myelitis  was 
diffuse  and  of  a  primary  parenchymatous  nature,  and  affected  prin- 
cipally the  white  matter.  The  nerve-fibres  were  everywhere  destroyed, 
the  myelin  being  partially  or  completely  broken  up,  and  the  axis- 
cylinders  were  degenerated.  The  lumina  of  the  blood-vessels  were  not 
overfilled,  but  the  walls,  especially  the  adventitia,  were  full  of  granu- 

*  Arch.  f.  Augenheilkunde,  vol.  xxvi. 

"  Myelitis  u.  Sehnervenentziindung,  Berlin,   1901 ;    V.  v.  S.  Karger. 


570  THE    EYE    AND    NERVOUS    SYSTEM 

lar  cells.  The  glia  fibres  seemed  normal  or  perhaps  a  little  swollen,  and 
the  neuroglia  cells  were  increased  in  number.  In  the  meshes  of  the  glia 
tissue,  however,  there  was  an  enormous  number  of  granular  or  fat- 
cells,  these  being  also  very  numerous  around  the  blood-vessels.  A  slight 
proliferation  of  the  endothelial  cells  of  the  intima  was  likewise  present. 
In  the  third  and  fourth  cases  the  inflammation  seemed  to  be  limited 
largely  to  the  posterior  and  lateral  tracts.  Histologically,  the  process 
was  similar  to  that  in  the  second  case.  In  the  majority  of  instances  of 
myelitis  with  optic  neuritis,  the  myelitis  is  such  as  is  reported  in  Biel- 
chowsky's  second  case — notably  so  in  the  cases  recorded  within  recent 
years,  in  which  microscopical  examinations  have  been  made  by  modem 
methods.  Some  authors,  however,  as  Elschnig,  interpret  the  inflamma- 
tion as  being  primarily  interstitial  in  nature. 

The  histological  process  in  the  optic  nerve  is  always  similar  to  that 
in  the  spinal  cord.  There  is  either  an  acute  hemorrhagic  inflammation 
or  a  primary  diffuse  parenchymatous  neuritis.  In  the  latter  case,  how- 
ever, the  inflammation  does  not  present  the  diffuse  areas  seen  in  the 
spinal  cord,  but  a  continuous  lesion.  This  is  due  to  the  merging  of  the 
adjacent  softened  areas. 

In  most  instances  the  optic  nerve  is  involved  throughout  its  whole 
extent,  from  the  papillse  to  the  chiasm  and  through  the  whole  thick- 
ness of  the  nerve.  This  is  not  always  so,  however,  for  there  may  be 
in  the  periphery  of  the  nerve  a  small  band  of  normal  fibres.  The 
chiasm  is  involved  in  most  cases,  and  the  process  may  extend  back  of 
the  chiasm  into  the  optic  tracts.  Secondary  degeneration  may  be  traced 
partially  into  the  optic  tracts,  but  the  primary  optic  centres — viz.,  the 
external  geniculate  body,  the  pulvinar  of  the  optic  thalamus,  and  the 
anterior  corpora  quadrigemina — have  never  been  found  diseased.  The 
fibres  of  Gratiolet  and  the  cuneus  have  also  never  been  found  in  a  dis- 
eased condition. 

A  cross  section  of  the  diseased  optic  nerve  will  always  show  a  widen- 
ing of  the  interfascicular  septa ;  an  increase  in  the  number  of  neu- 
roglia cells ;  a  large  number  of  flattened  granular  cells  in  the  meshes  of 
the  glia  tissue  and  around  and  in  the  blood-vessels ;  and  a  destruction 
of  the  nerve-fibres.  As  a  rule,  the  pial  covering  of  the  optic  nerve  is 
not  altered.  It  has  been  observed  to  be  diseased,  however,  in  two  in- 
stances ;  and  here  it  was  probably  local  in  origin,  for  the  accumulation 
of  nuclei  was  opposite  the  point  of  greatest  inflammation  of  the  optic 
nerve.  The  endothelial  cells  covering  the  subarachnoid  spaces  were  also 
increased  in  these  two  cases. 

If  the  whole  optic  nerve  is  equally  diseased,  it  is  impossible  to 


MYELITIS  571 

determine  whether  the  process  is  of  retrobulbar  origin  or  not.  In  the 
majority  of  instances,  however,  the  optic  neuritis  is  retrobulbar  in 
origin,  for  in  an  examination  of  the  limits  of  the  pathological  process 
— as  in  the  chiasm — recent  changes  may  be  found.  The  inflammation, 
however,  need  not  implicate  both  the  intraorbital  and  intracranial 
portions  of  the  optic  nerve ;  for  in  a  number  of  cases  the  intraorbital 
part  of  the  nerve  was  perfectly  normal, — the  inflammation  beginning  in 
the  central  part  of  the  nerve,  before  its  decussation,  and  involving  the 
chiasm  and  the  optic  tracts  to  a  limited  extent. 

Marked  neuritic  atrophy  of  the  papilla  and  of  the  surrounding  part 
of  the  retina  has  been  described  by  Dalen.'' 

Examination  of  the  other  involved  cranial  nerves  showed  an  intense 
acute  neuritis.  The  inflammation  was  not  secondary  to  a  nuclear  change 
nor  to  an  inflammation  of  the  pia,  and  there  was  no  secondary  degener- 
ation. 

In  two  instances — those  of  Elschnig  and  of  Weil  and  Gallawardin  "^ 
— there  was  a  slight  diffuse  interstitial  encephalitis.  In  no  case  was 
there  basal  meningitis.  In  the  medulla  oblongata,  except  for  the  usual 
ascending  secondary  degenerations,  nothing  abnormal  was  found. 

Relation  between  the  Myelitis  and  the  Optic  Neuritis. — Several 
theories  have  been  advanced  to  explain  the  occurrence  of  optic  neuritis 
with  myelitis.  Wharton  Jones  believed  that  the  optic  neuritis  was 
caused  by  a  circulatory  disturbance,  due  to  involvement  of  the  cervical 
sympathetic.  Allbutt  is  of  the  opinion  that  the  optic  neuritis  is  due 
to  an  ascending  basal  meningitis ;  while  still  other  authors  believe  that 
an  accompanying  intracranial  lesion  causes  the  optic  neuritis. 

From  the  evidence  of  recent  pathological  findings,  all  these  theories 
may  be  dismissed.  In  most  instances  the  optic  neuritis  precedes  the 
myelitis ;  so  the  hypothesis  of  an  optic  neuritis  secondary  to  a  cervical- 
sympathetic  lesion  is  not  tenable.  Again,  there  is  not  the  accompanying 
sympathetic  phenomena ;  nor  does  a  sympathetic  lesion  produce  optic 
neuritis. 

Allbutt's  theory  may  be  readily  dismissed,  for  no  basal  meningitis 
has  been  found  in  any  recorded  case.  So  far  no  secondary  intracranial 
lesion  has  been  observed.  In  only  two  instances  was  there  cerebral 
involvement,  and  in  these  it  was  in  the  nature  of  a  diffuse  encephalitis. 

Taylor  and  Collier  ^  have  recently  attempted  to  show  that  the  optic 
neuritis  is  always  due  in  these  cases  to  a  lesion  of  the  cervical  part  of 

'Arch,  f.  Ophthalm.,  vol.  48. 
'Rev.  Neurol.,  Oct.  31,  1903. 
"Brain,  1901. 


572  THE    EYE    A]!^D    NERVOUS    SYSTEM 

the  spinal  cord ;  and  that  in  every  one  of  the  reported  cases  the  patho- 
logical process  involved  the  cervical  cord.  A  careful  analysis,  however, 
does  not  seem  to  bear  out  this  statement.  In  sixteen  of  the  twenty 
autopsies  the  lesion  did  extend  to  the  cervical  cord ;  but  in  the  majority 
of  these  the  symptoms  seem  to  have  indicated  that  the  pathological 
process  had  begun  in  the  dorsal  cord  and  extended  upwards.  Clinically 
there  was  no  analogy  between  the  appearance  of  the  optic  neuritis  and 
the  myelitis.  In  fact,  as  before  stated,  the  optic  neuritis  generally  pre- 
cedes the  myelitis.  In  support  of  this  theory  it  may  be  said  that  in 
several  reported  cases  the  optic  neuritis  was  most  marked  on  the  side 
corresponding  with  the  greater  involvement  of  the  spinal  cord,  the 
lesion  in  these  cases  being  principally  in  the  cervical  cord.  Taylor  and 
(^)llier  also  believed  that  the  inflammation  must  be  a  partial  one ;  that 
is,  not  involving  the  whole  thickness  of  the  cord. 

In  view  of  the  fact  that  the  histological  process  is  alike  in  the  spinal 
cord  and  in  the  optic  nerve,  we  must  come  to  the  conclusion  that  the 
processes  are  identical  and  due  to  the  same  cause — probably  a  toxin 
that  exerts  its  influence  equally  on  the  spinal  cord  and  on  the  optic  nerve. 
AMiy  this  toxin  should  pick  out  the  optic  nerve  is,  however,  a  question. 
It  may  be  explained  on  the  theory  of  locus  minoris  resistentiw.  The 
optic  nerve  has  the  same  embryological  origin  as  has  the  spinal  cord, 
and  it  may  be  that  the  toxin  that  acts  upon  the  spinal  cord  has  a 
selective  action  upon  the  optic  nerve. 

Prognosis. — As  a  rule,  the  prognosis  in  these  cases  depends  upon  the 
progress  of  the  myelitis,  for  improvement  in  the  spinal-cord  symptoms 
always  indicates  an  improvement  in  those  of  the  optic  nerve. 

Treatment. — Treatment  is  usually  unavailing.  When  a  history  of 
syphilis  is  obtained,  which  is  in  about  one-third  of  the  cases,  a  combined 
antisyphilitic  treatment  is  indicated ;  but,  as  a  rule,  this  is  of  no 
benefit.  The  bed-sores  should  be  carefully  looked  after,  and  the  con- 
tracted limbs  kept  in  good  condition.      Catheterization   is  commonly 

indicated. 

SYRINGOMYELIA. 

As  a  rule,  the  symptom  comi)lex  of  syringomyelia  is  so  typical  that 
the  diagnosis  does  not  offer  much  difficulty.  The  disease  generally 
begins  early  in  life  and  is  chronic  and  progressive.  The  symptoms  may 
be  divided  into  three  forms:  first,  the  atrophy  of  the  muscles  of  the 
upper  limbs;  second,  the  disturbances  of  sensation,  and,  third,  vaso- 
motor and  trophic  phenomena. 

The  atrophy  usually  begins  in  the  small  nuiscles  of  the  hand  and 
may  involve  all  the  muscles  of  one  or  both  upper  limbs,  but  is  not 


SYRINGOMYELIA  573 

uniform.  Fibrillary  twitching  may  be  present  and  electrical  reactions 
of  degeneration  may  be  obtained. 

Sensation  for  touch  is  generally  preserved,  but  pain  and  tempera- 
ture sensations  are  lost.  This  dissociation  of  sensation  may  be  present 
in  one  or  both  upper  limbs  and  part  of  the  trunk,  depending  upon  the 
part  of  the  spinal  cord  involved,  but  it  is  usually  more  marked  on  one 
side. 

Vasomotor  and  trophic  phenomena,  as  cyanosis  of  the  hand,  oedema, 
arthropathies,  and  deformities  of  bone,  are  common. 

The  lower  limbs  may  be  spastic,  due  to  secondary  degeneration  of 
the  pyramidal  fibres,  or  may  be  paralyzed,  due  to  direct  involvement  of 
the  lumbar  cord,  but  this  is  exceptional.  If  the  disease  involves  the 
medulla  oblongata  and  pons,  we  may  have  symptoms  of  cranial  nerve 
palsy,  as  atrophy  of  the  tongue,  disturbances  of  respiration,  laryngeal 
palsy,  deafness,  facial  paralysis,  and  symptoms  of  involvement  of  the 
different  ocular  nerves. 

Pathology. — The  cause  of  this  symptom  complex  is  a  cavity  or  a 
gliomatous  tumor  formation  in  the  substance  of  the  spinal  cord,  which 
generally  involves  its  centre,  the  posterior  columns,  or  the  posterior 
horns.  It  hardly  ever  involves  the  anterior  part  of  the  cord,  and  may 
be  unilateral  throughout  its  whole  extent.  The  cavity  is  usually  largest 
at  the  cervical  cord  and  ends  in  the  lower  thoracic  region,  thus  explain- 
ing the  preponderance  of  symptoms  in  the  upper  limbs.  It  has  been 
thought  that  the  cavity  does  not  extend  higher  than  the  nucleus  of  the 
seventh  nerve  in  the  pons,  but  Spiller  has  observed  an  instance  in 
which  the  cavity  extended  as  high  as  one  cerebral  peduncle.  It  is 
important  to  remember  this  fact,  especially  when  we  consider  the 
cause  of  the  different  ocular  nerve  palsies.  The  cavity  is  filled  during 
life  with  cerebrospinal  fluid.  A  congenital  defect  in  the  formation  of 
the  spinal  cord  is  probably  the  basis  of  the  disease. 

Ocular  Symptoms. — Eye  symptoms  are  uncommon.  We  shall  con- 
sider in  order  involvement  of  the  optic  nerves,  the  ocular  muscles,  and 
the  pupils.  Optic  neuritis  has  been  recorded  by  Saxer,®  BuUard  and 
Thomas,^^  and  by  Thorington  and  myself.^^  In  all  of  these  cases  a 
complicating  internal  hydrocephalus  was  probably  at  fault,  as  was 
show^n  by  the  necropsy  findings  in  the  first  two.  It  is  well  to  remember 
that  internal  hydrocephalus  is  not  an  infrequent  complication  of 
syringomyelia,  and  that  many  of  the  cranial-nerve  palsies  which  may 

'  Beitriig.  z.  path.  Anat.  u.  z.  allgem.  Path.,  vol.  xx.  No.  11. 
"  American  Journal  of  Medical  Science,  1899. 
"Ibid.,  Nov.  1905. 


574  THE    EYE    AND    NERVOUS    SYSTEM 

be  present  in  syringomyelia  are  due  to  the  pressure  of  the  cerebrospinal 
fluid.  This  was  the  case  in  the  above-mentioned  instances,  in  which 
both  external  recti  and  the  facial  nerve  were  diseased.  Hoffmann  sug- 
gests that  a  tumor  situated  in  the  medulla  oblongata,  which  may  be 
the  starting  point  of  a  syringomyelic  cavity,  may  cause  optic  neuritis. 
This  undoubtedly  may  occur  and  it  is  probable  that  in  some  of  the 
cases  of  secondary  optic  atrophy  this  may  be  the  explanation. 

Primary  optic  atrophy  occurs  less  often  in  syringomyelia  than  in 
any  other  spinal-cord  disease.  ^laixncr  (quoted  by  Schlesinger),  in 
thirty-two  cases,  found  optic  atrophy  in  three.     In  one  case  under  my 


Fig.  2.— Case  of  syringomyelia  with  optic  neuritis  and  bilateral  external  rectus  paralysis:  also 
showing  position  of  head.    (Reported  with  Dr.  James  Thorington.) 


(observation  gray  degeneration  of  the  optic  disk  occurred  in  one  eye,  the 
other  fundus  being  fairly  normal.  This  is  unusual,  for,  when  disturb- 
ances do  occur,  they  usually  involve  both  optic  nerves  equally.  It  must 
be  remembered,  however,  that  tabes  dorsalis  rarely  complicates  syringo- 
myelia, and  when  ])rimary  optic  atrophy  is  present  this  disease  should 
be  excluded. 

The  ophthalmoscopic  findings  are  usually  negative.  The  disks  may 
be  pale,  but  always  in  their  whole  extent,  differing  from  the  temporal 
paling  so  often  seen  in  multiple  sclerosis.  The  fundi  in  two  of  my 
cases  were  gray  in  color.  Vision,  as  a  rule,  is  not  interfered  with,  but 
if  disturbances  occur,  both  eyes  are  equally  affected.     Neither  scotoma 


SYRIIs^GOMYELIA  575 

nor  disturbances  of  light  and  color  sense  have  been  recorded  in  this 
disease. 

A  concentric  restriction  of  the  visual  fields,  especially  for  colors,  and 
particularly  for  green,  sometimes  occurs.  Schlesinger  ^^  collected  from 
the  literature  one  hundred  and  thirty  cases,  in  which  this  symptom  was 
present  in  thirty-eight.  The  examinations  were  perimetric.  Of  these, 
twenty-four  had  no  symptoms  of  hysteria.  The  contraction  of  the  visual 
fields  was  not  severe,  and  consisted  in  a  peripheral  defect,  especially  for 
green.  He  concludes  that  in  most  cases  of  syringomyelia  the  visual 
fields  are  normal,  that  the  contraction  of  the  fields  may  be  due  to  a 
concomitant  hysteria,  and  that  in  a  small  number  of  cases  of  syringo- 


FiG.  3.— Case  of  syringomyelia  with  optic  neuritis,  showing  lateral  curvature. 
(Reported  with  Dr.  James  Thorington. ) 

myelia  not  complicated  with  hysteria,  alterations  in  the  visual  fields 
may  occur. 

Other  authors,  however,  do  not  believe  that  in  an  uncomplicated  case 
with  negative  ophthalmoscopic  findings  visual  contractions  can  occur, 
and  ascribe  these  symptoms  to  hysteria.  In  five  cases  under  my  obser- 
vation, in  which  a  perimetric  examination  was  made,  no  contraction  of 
the  visual  fields  was  noticed.  Schlesinger  explains  the  contraction  by 
an  intracranial  complication,  probably  a  hydrocephalus.  If  this  is  so, 
we  might  expect  that  these  symptoms  would  vary  on  different  exami- 

"  Die  Syringomyelie,  Leipzig  u.  Wien,  1902. 


576  THE    EYE    AND    NERVOUS    SYSTEM 

nations,  but  tliis  is  not  mentioned.  In  the  eight  eases  recorded  by  Dejer- 
ine  and  Tuilant/^  who  were  the  first  to  call  attention  to  the  restriction 
of  the  visual  fields,  an  examination  a  year  later  still  denoted  the  same 
condition. 

Paralysis  of  Ocular  Muscles. — Schlesinger,  in  a  study  of  three  hun- 
dred cases  from  the  literature,  found  paralysis  of  the  ocular  muscles 
in  thirty-three,  approximately  in  11  per  cent,  of  the  cases.  It  occurs 
less  often  than  in  multiple  sclerosis  and  tabes  dorsalis.  The  paralysis 
generally  comes  on  early  in  the  disease  and  may  be  transient,  but  later 


Fig.  4.— Case  of  syringomyelia  with  partial  ptosis  of  right  upper  lid. 

in  the  disease  may  become  permanent.  These  transient  paralyses 
resemble  those  of  syphilis  and  tabes. 

The  abducens  nerve  is  more  often  involved  than  the  other  cranial 
nerves,  and  more  often  the  involvement  is  unilateral.  The  oculomotor 
nerve  is  usually  only  partially  involved,  as  shown  by  a  ptosis.  This 
is  present  in  the  patient  whose  photograph  is  given.  The  ptosis  may 
be  slight  and  may  vary  from  day  to  day,  as  in  the  patient  in  the  photo- 
graph, in  whom  the  ptosis  varied  considerably  on  successive  examina- 
tions. This  variability  in  the  amount  of  paralysis,  as  shown  by  the 
abducens  nerve,  seems  to  be  characteristic  of  syringomyelia. 

The  ptosis  which  is  due  to  the  third  nerve  involvement  must  be 
differentiated  from  the  ptosis  due  to  sympathetic  paralysis.     It  is  well 


"Semaine  Med.,  1891,  No.  6. 


SYRINGOMYELIA  677 

known  that  in  sympathetic  paralysis  all  of  the  symptoms  need  not  be 
present,  and  we  may  have  in  both  conditions  only  a  ptosis.  An  appli- 
cation of  cocaine  will,  in  sympathetic  paralysis,  not  dilate  the  pupil. 

If  the  syringomyelic  cavity  extends  high  up  into  the  pons,  we  may 
have  complete  chronic  external  ophthalmoplegia.  Retraction  of  the  ocu- 
lar muscles  appears  late  in  the  disease. 

A  direct  involvement  of  the  ocular  nuclei  or  of  their  intramedullary 
fibres  by  the  cavity  will  explain  the  ocular  palsies,  providing  there  are 
in  these  cases  some  other  symptoms  of  bulbar  paralysis.  If  they  are  not 
present,  an  internal  hydrocephalus  is  probably  the  cause.  The  tran- 
sient ocular  palsies  and  variability  of  the  ptosis  may  be  explained  by 
the  difference  in  pressure  of  the  fluid  in  the  syringomyelic  cavity. 

According  to  Schlesinger,  nystagmus  and  nystagmoid  movements 
of  the  eyeball  in  syringomyelia  occur  in  about  15  per  cent,  of  the  cases. 
Of  three  hundred  and  thirty  cases,  fourteen  had  true  nystagmus  and 
thirty-seven  nystagmoid  movements.  Schlesinger  states  that  the  nys- 
tagmus comes  on  early  in  the  disease,  but  it  may  be  a  late  manifestation. 
The  movements  may  be  horizontal,  vertical,  diagonal,  or  rotatory.  Dis- 
turbance of  vision  in  nystagmus  never  occurs,  but  in  a  number  of  in- 
stances the  fixing  object  was  said  to  be  fluctuating. 

The  nystagmoid  movements  are  far  more  common  than  those  of  true 
nystagmus.     They  are  obtained  only  on  lateral  deviation. 

The  cause  of  the  nystagmus  and  the  nystagmoid  movements  in  this 
disease  are  not  known,  but  various  theories  are  given.  Some  authors 
believe  that  they  are  an  accidental  complication  of  the  disease,  but  it 
may  be  argued  against  this  hypothesis  that  the  nystagmus  comes  on  as 
the  disease  appears.  Schlesinger  believes  that  it  is  due  to  the  pressure 
•of  hydrocephalus,  while  other  authors  believe  that  it  is  congenital. 

In  the  different  diseases  in  which  nystagmus  is  a  symptom,  such  as 
Friedreich's  disease  and  in  syringomyelia,  a  congenital  anomaly  in  the 
formation  of  the  brain  and  spinal  cord  is  thought  to  be  the  cause. 
Multiple  sclerosis  also  is  believed  by  some  to  be  produced  in  the  same 
way.  In  these  diseases  the  symptoms  do  not  appear  until  some  con- 
tributory cause  starts  the  pathological  process.  Nystagmus  appears  at 
the  same  time.  It  is  a  striking  fact  that  in  syringomyelia,  in  the  cases 
in  which  nystagmus  occurs,  there  are  no  disturbances  of  vision,  accom- 
modation, or  in  the  pupillary  reflex,  this  arguing  against  an  acquired 
lesion.  It  is  probable,  therefore,  that  the  cause  of  the  nystagmus  in 
these  diseases  is  some  congenital  maldevelopment,  and  that  this  symp- 
tom does  not  appear  until  the  symptoms  of  involvement  of  the  rest  of 
the  brain  and  spinal  cord  present  themselves. 
37 


578  THE    EYE    AND    NERVOUS    SYSTEM 

Pupillary  Symptoms. — The  pupils  in  syringomyelia  are  usually  not 
affected,  but  myosis  and  differences  in  the  size  of  the  pupils  sometimes 
occur.  In  two  hundred  cases  studied  by  Schlesinger  pupillary  differ- 
ences were  found  in  twenty-four.  The  pupillary  light  reflex  is  not 
often  absent,  and  yet  a  number  of  such  cases  have  been  recorded.  Dejer- 
ine  and  Mirallie  record  an  instance  where  the  light  reflex  was  absent  on 
one  side  only.  It  may  be  assumed  that  when  the  pupillary  light  reflex 
is  disturbed  or  absent,  we  may  have  an  accompanying  tabes  or  general 
paresis. 

Paralysis  of  the  sympathetic  may  be  caused  by  a  cavity  in  the  lower 
portion  of  the  cervical  cord.  Sympatlietic  symptoms  are  generally  uni- 
lateral, but  may  be  bilateral.  Such  symptoms  are  narrowing  of  the 
palpebral  fissure,  myosis,  enophthalmos,  and  disturbances  of  the  secre- 
tions. As  a  rule,  we  do  not  have  all  of  these  symptoms  in  sympathetic 
paralysis,  and  we  may  have  only  myosis  with  ptosis  without  involvement 
of  the  secretory  functions.  These  symptoms  are  caused  by  the  in- 
volvement of  the  sympathetic  fibres  which  ascend  in  the  spinal  cord 
from  the  ciliospinal  centre  in  the  first  dorsal  segment  to  the  medulla 
oblongata. 

Involvement  of  the  Fifth  and  Seventh  Nerves. — The  descending 
sensory  portion  of  the  trigeminus  nerve  is  sometimes  involved  in 
syringomyelia.  This  is  first  manifested  by  paraesthetic  phenomena,  later 
by  pains,  and  finally  by  anaesthesia.  A  disturbance  in  the  secretion  of 
tears  and  of  saliva  has  been  noted  in  a  few  instances.  The  secretion  of 
tears  is  sometimes  increased  and  appears  on  laughing  or  on  irritation 
of  the  nasal  mucous  membranes  on  the  side  opposite  the  anaesthesia. 
The  corneal  and  lachrymal  reflexes  are  lost.  Trophic  disturbances  are 
uncommon.  This  may  be  due  to  an  incomplete  paralysis  of  the  trigemi- 
nus, but  even  in  total  paralysis  trophic  disturbances  may  not  occur. 
This  is  probably  due  to  the  nature  of  the  lesion,  for  it  has  been  shown 
that  an  irritative  condition,  either  in  the  pons  or  in  tlie  Gasserian 
ganglion,  may  produce  a  keratitis.  According  to  Gowers,^*  a  greater 
irritation  is  necessary  to  produce  a  corneal  ulcer  if  the  lesion  involves 
the  fifth-nerve  fibres  within  the  pons  or  the  sensory  root  than  if  the 
lesion  is  at  or  in  front  of  the  Gasserian  ganglion. 

The  facial  nerve  is  sometimes  involved  in  syringomyelia.  The 
paralysis  may  be  transitory.  All  or  only  part  of  the  nerve  may  be 
diseased.  If  the  upper  portion  is  much  involved,  it  causes  a  lagoph- 
thalmos. 


Diseases  of  the  Nervous  System,  vol.  ii.  217. 


SPINAL  CORD  DISEASES  579 


THE    RELATION    OF    OCULAR    SYMPTOMS    TO 
SPINAL-CORD  DISEASES. 

In  the  following  pages  will  be  considered  the  various  ocular  dis- 
turbances occurring  in  conjunction  with  certain  spinal-cord  diseases, 
such  as  primary  lateral  sclerosis,  amyotrophic  lateral  sclerosis,  postero- 
lateral sclerosis,  and  the  subacute  combined  system  degenerations  which 
sometimes  complicate  pernicious  anaemia.  Primary  ascending  unilateral 
paralysis  will  also  be  considered  in  this  connection.  As  a  rule,  ocular 
changes  are  rare,  but  optic-nerve  atrophy  has  been  recorded  in  all  of  these 
diseases,  as  well  as  pupillary  phenomena. 

To  understand  the  relation  of  the  optic-nerve  atrophy  to  the  various 
spinal-cord  diseases,  we  must  first  clearly  understand  the  nature  of 
these  spinal  cord  affections.  The  belief  is  gradually  gaining  ground  that 
such  diseases  as  primary  lateral  sclerosis  and  amyotrophic  lateral  sclerosis 
are  due  to  a  defect  of  vital  endurance,  or,  as  Gowers  has  recently  termed 
it,  to  an  "  abiotrophy."  Both  of  these  diseases  come  on,  as  a  rule,  early 
in  life.  The  optic  nerve  atrophy  appears  simultaneously  with  the  spinal- 
cord  symptoms,  and  both  manifestations  are  uniformly  progressive.  We 
are  led  to  believe,  therefore,  that  we  have  in  the  optic-nerve  atrophy  the 
same  primary  cause — a  lack  of  vital  endurance.  It  is  difficult  to  ex- 
plain, however,  why  the  optic  ner\'e  or  certain  tracts  of  the  spinal  cord 
should  be  picked  out  for  degeneration. 

Lack  of  vital  endurance  may  be  manifested  late  in  life,  as,  for  in- 
stance, in  such  diseases  as  paralysis  agitans.  It  is  possible  that  this 
explains  the  unilateral  slowly  progressive  ascending  paralysis  described 
by  Charles  K.  Mills. 

Such  degenerations  as  are  due  to  pernicious  anaemia  are  undoubtedly 
toxic  in  origin.  The  poison  which  is  generated  by  the  disease  circulates 
in  the  blood  and  acts  upon  the  nervous  structures.  It  may  be  due  to  a 
lack  of  resisting  power  that  the  spinal  cord  and  the  optic  nerves  are 
affected. 

Tabes  dorsalis  is  also  a  disease  which  is  produced  by  a  toxin,  and 
it  is  most  probable  that  posterolateral  sclerosis,  which  comes  on  late  in 
life,  is  due  to  a  similar  cause — a  syphilitic  toxin — and  that  the  cause  of 
the  optic-nerve  atrophy  in  the  two  affections  can  be  similarly  explained. 

It  is  noticeable  that  in  all  diseases  just  mentioned,  the  optic-nerve 
atrophy  is  progressive,  and  that  return  of  function  does  not  occur.  Oph- 
thalmoscopically,  we  find  a  gray  degeneration  of  the  disk.  The  retina, 
as  a  rule,  is  not  affected.  There  is  usually  a  peripheral  restriction  of 
the  fields,  and  there  may  be  a  central  scotoma. 


680  THE    EYE    AND    NERVOUS    SYSTEM 

The  pupillary  phenomena  are  attributed  to  the  disturbance  of  the 
cervical  sympathetic.     This  will  be  later  fully  discussed. 

PRIMARY    DEGENERATION    OF    THE    PYRAMIDAL    TRACTS 
(PRIMARY    LATERAL    SCLEROSIS). 

In  this  affection  both  pyramidal  tracts  are  diseased,  the  nerve  cells 
in  the  anterior  horns  of  the  spinal  cord  being  normal.  Both  lower  limbs 
are  usually  involved,  and  we  have  the  well-kijown  picture  of  lateral 
sclerosis.  The  limbs  are  weak  and  very  spastic.  All  the  tendon  reflexes 
are  increased,  and  walking  is  usually  impossible  because  of  the  weakness 
and  the  contractures.  There  is  no  atrophy  of  the  muscles.  As  the 
disease  progresses,  the  upper  limbs  become  involved  similarly  to  the 
lower. 

As  a  rule,  there  are  no  eye  symptoms;  but,  rarely,  optic  atrophy 
similar  in  character  to  that  seen  in  tabes  is  observed.  Gowers  mentions 
a  case  of  spastic  paraplegia  in  a  boy  of  twenty,  who,  besides  the  spinal- 
cord  symptoms,  had  a  gradually  developing  primary  optic  atrophy. 
The  acuity  of  vision  was  reduced  to  one-tenth.  The  fields  presented  a 
peripheral  restriction.  There  was  a  well-marked  central  scotoma  for  red 
and  green,  which  was  irregular  in  outline,  but  extended  to  an  average 
of  about  ten  degrees  from  the  fixing-point,  and,  in  one  eye,  to  twenty 
degrees. 

The  degeneration  in  the  pyramidal  tracts  and  in  the  optic  nerves 
was  simultaneous  and  progressive  in  character,  and  argued  for  the 
abiotic  nature  of  the  disease.  Again,  the  eye  symptoms  were  similar  to 
those  seen  in  an  ordinary  atrophy  of  spinal  origin.  Optic  neuritis  is 
said  to  have  occurred  in  the  course  of  lateral  sclerosis,  but  an  examina- 
tion of  the  recorded  cases  fails  to  show  clearly  such  an  occurrence. 

AMYOTROPHIC    LATERAL  SCLEROSIS. 

In  this  fonn  of  primary  disease  of  the  motor  system  there  is,  in 
addition  to  the  clinical  picture  of  lateral  sclerosis  just  described,  an 
atrophy  of  the  muscles  of  the  affected  limbs.  This  is  caused  by  the 
involvement  of  the  nerve-cells  of  the  anterior  horns  in  the  spinal  cord. 
The  disease  is  progressive,  and  finally  involves  the  different  nuclei  in 
the  medulla  oblongata  and  pons,  giving,  besides  the  spastic  symptoms, 
bulbar  phenomena,  such  as  difficulty  in  speech  and  deglutition,  drib- 
bling of  saliva,  atrophy  of  the  tongue,  limbs,  and  face,  and  fibrillary 
twitching  of  the  parts  involved. 

If  the  disease  is  limited  to  the  spinal  cord,  eye  symptoms  are 
rare.     When  the  bulb  becomes  involved,  there  may  be  any  of  the  ocular 


POSTEROLATERAL  SCLEROSIS  581 

phenomena  common  in  bulbar,  palsy,  which  will  be  described  in  another 
chapter. 

Irregularity  of  the  pupils,  with  partial  or  total  loss  of  reaction  to 
light,  and  optic  atrophy  have  been  described.  The  pupillary  phenomena 
are  explained  by  the  sympathetic  involvement;  while  the  optic-nerve 
atrophy  is  similar  to  that  seen  in  other  primary  degenerations  of  the 
pyramidal  tracts.  Spiller  mentions  a  weakness  of  one  external  rectus 
muscle  in  one  instance. 

PROGRESSIVE   ASCENDING   UNILATERAL    PARALYSIS. 

This  disease,  which  has  only  recently  been  described  by  Mills,  is 
a  form  of  primary  degeneration  of  one  pyramidal  tract,  ascending  in 
character.  A  lower  or  an  upper  limb  is  first  affected ;  then,  in  the 
course  of  some  time  (in  the  original  case,  eighteen  months),  the  other 
limb  is  involved,  giving  the  picture  of  a  hemiplegia.  A  weakness  of 
the  lower  distribution  of  the  face  is  also  present.  The  affected  limbs 
are  weak  and  spastic,  and  the  reflexes  are  increased. 

In  a  case  subsequently  described  by  Spiller,  in  which  the  eye  exami- 
nation was  made  by  Dr.  Ilansell,  the  report  was  as  follows : 

Right  Eye. — Media,  clear;  fundus,  normal.  A  myopia  of  3  D. 
exists. 

Left  Eye. — Media,  clear ;  arteries,  small ;  veins,  normal.  The  edges 
of  the  disk  are  distinct.  The  nerve-head  is  white,  the  atrophy  probably 
being  spinal  in  origin.     There  is  no  indication  of  previous  neutritis. 

It  is  interesting  to  observe  that  in  this  case  "the  atrophy  of  the  optic 
nerve  corresponded  with  the  primary  degeneration  of  the  pyramidal 
tract  of  the  same  side,  both  being  on  the  left ;  whereas  the  other  optic 
nerve  was  normal. 

POSTEROLATERAL  SCLEROSIS. 

In  this  combined  system  disease  there  is  a  degeneration  of  the  pos- 
terior and  lateral  tracts  of  the  spinal  cord.  Besides  the  increase  of  the 
reflexes,  the  spastic  condition,  and  the  weakness  of  the  limbs  that  are 
common  in  lateral  sclerosis,  there  is  a  spastic-ataxic  gait,  incoordination 
of  the  limbs,  disturbance  of  sensation  for  touch  and  pain,  especially  on 
the  sole  of  the  foot,  in  front  of  the  tibia,  and  on  the  chest  as  is  common 
in  tabes,  and  a  disturbance  in  the  functions  of  the  bladder  and  the 
rectum. 

Ocular  Symptoms. — Rigidity  of  the  pupil  to  light  is  unusual,  but 
Gowers  ^^  has  observed  it  in  two  or  three  cases.    In  one  instance,  accom- 

"  Diseases  of  the  Nervous  System,  vol.  i.  50G. 


582  THE    EYE    AND    NERVOUS    SYSTEM 

modation  was  lost,  the  light  reaction  being  normal.  It  may  be  said  that 
pupillary  changes  occur  in  the  cases  in  which  syphilitic  infection  is 
present.  Optic-nerve  atrophy  is  rare,  although  it  has  been  observed  in 
a  few  cases.  Usually  there  is  no  involvement  of  the  extraocular  muscles, 
although  nystagmus  has  been  recorded. 

I  have  had  under  my  care  a  man  about  thirty  years  of  age,  who, 
besides  the  symptoms  of  posterolateral  sclerosis,  showed,  one  year  after 
the  beginning  of  these  symptoms,  the  following  eye  phenomena : 

The  pupils  were  three  millimetres  in  width.  The  direct  and  con- 
sensual reactions  were  normal.  There  was  incipient  optic-nerve  atrophy. 
Both  optic  nerves  were  conspicuously  pallid  and  slightly  excavated.  No 
gross  fundus  changes  were  observed  in  the  retina  or  the  choroid.  On 
lateral  deviation  to  the  right  or  the  left,  decided  nystagmoid  movements 
were  observed.    The  examination  was  made  by  Dr.  E.  A.  Shumway. 

It  is  possible  that  true  nystagmus  does  not  occur  in  this  disease, 
but  we  have  what  may  be  called  nystagmoid  movements,  if  we  choose 
to  make  such  a  distinction.  In  nystagmus  the  movements  may  be  in 
any  direction  and  to  either  side  of  the  fixing  point,  whereas  in  nystag- 
moid movements  the  oscillations  of  the  eyeball  are  always  horizontal 
and  are  obtained  only  on  lateral  deviation. 

SPINAL-CORD   SYMPTOMS   IN   PERNICIOUS   ANEMIA. 

It  is  well  known  that  we  may  have  changes  in  the  spinal  cord  due  to 
any  chronic  ana'mic  condition,  and  this  has  been  especially  well  recog-. 
nized  in  pernicious  anaemia.  Spinal  symptoms  may  appear  even  before 
the  blood-count  indicates  the  disease,  but  as  a  rule  they  are  mani- 
fested after  the  disease  has  well  advanced,  or  they  may  come  on  simul- 
taneously. 

Pathologically,  there  is  a  diffuse  degeneration  of  the  posterior  and 
lateral  columns,  but  the  degeneration  is  not  limited  to  these  tracts,  and 
extends  beyond  these  areas  to  the  anterior  part  of  the  cord.  The  process 
is  uniform  and  involves  symmetrical  areas. 

Besides  the  usual  symptoms  of  pernicious  anaemia,  as  weakness,  de- 
bility, and  reduction  in  the  amount  of  haemoglobin  and  in  the  red  and 
white  blood-cells,  the  disease  usually  begins  by  a  feeling  of  numbness, 
or  a  pins-and-needles  sensation  in  the  legs,  followed  by  weakness  and 
spasticity.  The  reflexes  are  increased ;  in  a  short  time  the  upper  limbs 
become  similarly  involved,  and  we  have,  besides,  a  considerable  ataxia 
of  all  the  limbs  and  a  disturbance  of  the  bladder  and  rectal  functions. 
Later  there  may  be  complete  loss  of  sensation,  a  flaccid  condition  may 
succeed  the  spasticity,  and  all  the  reflexes  may  be  lost. 


PERNICIOUS  ANEMIA  583 

Ocular  Symptoms. — Eye  symptoms  are  rare.  Towards  the  end  of 
the  affection  the  pupils  have  been  found  unequal,  and  in  one  instance 
there  was  a  slight  bilateral  ptosis  and  a  marked  myosis  due  to  sym- 
pathetic involvement.  In  a  few  instances  reflex  iridoplegia  has  been 
reported.  Xystagmus  has  also  been  observed.  Optic-nerve  atrophy  is 
extremely  rare,  only  a  few  cases  being  on  record. 

I  have  under  my  observation  an  interesting  case  of  this  kind.  The 
patient,  a  man  of  42,  without  any  previous  ailment,  suddenly  had  a 
gastric  hemorrhage.  The  following  day  he  began  to  lose  sight  in  both 
eyes,  and  in  a  week  he  was  completely  blind,  in  which  condition  he  has 
remained.  He  did  not  experience  any  weakness  in  his  limbs  until  one 
day  after  sight  was  completely  lost,  from  which  time  he  gradually 
developed  the  typical  spinal  symptoms  of  the  affection.  The  blood- 
count  showed  the  usual  changes  of  pernicious  anaemia. 

Ophthalmoscopic  examination,  by  Dr.  de  Schweinitz,  made  four 
days  after  the  blindness  became  complete  showed  a  double  optic  neuritis. 
The  pupils  were  dilated  and  were  irresponsive.  There  was  shrinking 
of  the  retinal  arteries  and  overfilling  of  the  veins,  and  there  was  some 
oedema  of  the  nerve-head  and  surrounding  area. 

It  is  hard  to  explain  the  evident  low-grade  optic  neuritis  which  was 
present  in  this  case,  as  shown  by  the  examination,  and  the  relation  it 
bore  to  the  sudden  loss  of  vision.  A  gastric  hemorrhage  may  bring  on 
an  atrophy  of  the  optic  nerve  and  cause  blindness  in  a  few  hours,  but  it 
will  not  produce  optic  neuritis.  Most  probably  there  was  a  low-grade 
optic  neuritis  present  before  the  hemorrhage  came  on,  but  tiiis  did  not 
seriously  affect  the  vision,  and  the  sudden  loss  of  blood  brought  on 
the  rapid  amaurosis.  Optic  neuritis  in  the  course  of  pernicious  ansemia 
may  occur,  but  it  usually  gives  a  picture  of  an  albuminuric  retinitis, 
and  the  low-grade  optic  neuritis  may  have  been  a  preliminary  stage  of 
the  affection.  Ophthalmoscopical  examination  at  the  present,  ten 
months  after  the  onset  of  the  disease,  shows  a  typical  post-neuritic 
atrophy.    There  are  also  nystagmoid  movements  on  lateral  deviation. 

INJURIES    OF    THE    SPINAL   CORD. 

Injuries  to  the  substance  of  the  spinal  cord  are  by  no  means  rare. 
Fracture  and  dislocation  of  the  vertebra?  are  the  most  common  cause. 
Dislocation  of  the  vertebrae  is  most  frequent  in  the  cervical  region,  be- 
tween the  atlas  and  axis  and  between  the  fifth  and  sixth  cervical  ver- 
tebrae. The  spinal  cord  may  be  injured  although  the  vertebrae  are  not 
at  all  harmed.  There  may  be  at  the  moment  of  injury  a  sudden  torsion 
of  the  cord,  which  may  produce  a  hemorrhage,  or  a  softening,  or  both. 


584  THE    EYE    AND    NERVOUS    SYSTEM 

Usually  the  hemorrhage,  or  haematomyelia,  is  in  the  central  part  of  the 
spinal  cord.  Direct  pressure  causes  softening  of  the  affected  parts,  and 
there  may  be  also  hemorrhages  and  areas  of  softening  either  above  or 
below  the  point  of  greatest  pressure.  Gunshot  injuries  or  stab  wounds 
are  not  unusual.  The  spinal  cord  may  be  directly  injured  or  there 
may  be  a  resulting  hemorrhage  and  softening  from  injury  to  the 
vertebra'. 

Symptoms. — The  symptoms  produced  will  depend  entirely  upon  the 
part  of  the  spinal  cord  involved.  If  there  is  a  fracture  or  dislocation, 
there  is  usually  a  deformity  of  that  part,  accompanied  by  rigidity  of  the 
back  and  neck,  and  pain  on  movement.  All  of  the  limbs  are  paralyzed 
if  the  injury  is  above  the  cervical  enlargement ;  if  below  this  part,  only 
the  lower  limbs  are  paretic.  The  reflexes  are  at  first  lost,  and  later 
become  increased.  The  rectal  and  bladder  functions  are  disturbed,  and 
sensation  is  lost,  depending  upon  the  area  of  the  cord  destroyed.  The 
symptoms  of  traumatic  myelitis  are  identical,  except  that  we  have  no 
dislocation  or  fracture  of  the  vertebrae.  In  central  haematomyelia  the 
same  picture  is  presented,  and  we  may  have,  besides,  the  syringomyelic 
disturbance  of  sensation,  and  the  pain  may  be  excessive. 

We  shall  here  also  consider  such  other  diseases  which  produce  symp- 
toms of  compression  or  disturbance  of  the  function  of  the  spinal  cord, 
because  the  ocular  phenomena  are  identical.  Such  diseases  are  tumors 
of  the  vertebrae  and  of  the  spinal  cord,  aneurism  of  the  aorta,  caries, 
carcinoma  of  the  vertebra?  and  the  meninges,  and  pachymeningitis  cervi- 
calis  hypertrophica. 

Tumors  of  the  vertebra?  are  far  more  common  than  tumors  of  the 
meninges  or  of  the  substance  of  the  cord.  Whether  the  tumor  is  extra- 
medullary  or  intramedullary,  the  symptoms  are  usually  identical,  and 
depend  upon  the  part  of  the  cord  involved.  The  pain  in  these  cases  is 
sharper  and  more  persistent  than  in  any  other  spinal  disease,  and  its 
location  depends  upon  the  part  of  the  body  supplied  by  the  involved 
spinal  segment  or  spinal  root. 

An  eroding  aneurism  of  the  descending  aorta,  caries  of  the  vertebra?, 
carcinoma  of  the  vertebra  and  of  the  meninges,  and  pachymeningitis 
will  give  symptoms  of  pressure  upon  the  spinal  cord,  depending  upon 
the  part  involved,  and,  as  these  symptoms  in  the  main  resemble  each 
other,  we  will  not  consider  each  separately.  The  differential  diagnosis 
can  be  readily  made  in  most  instances  by  the  concomitant  symptoms. 

Ocular  Symptoms. — There  is  no  subject  on  which  we  have  less 
accurate  information  than  the  ocular  symptoms  accompanying  injuries 
of  the  spinal  cord.     Most  of  the  literature  consists  of  a  repetition  of 


INJURIES  OF  THE  SPIXAL  CORD  585 

statements  made  by  a  few  authors,  and  these  are  by  no  means  definite. 
Considerable  confusions  exists,  partly  because  the  ocular  symptoms  fol- 
lowing an  injury  of  the  spinal  cord  are  confused  with  the  ocular  mani- 
festations due  to  a  hysterical  or  neurasthenic  condition  following  trauma 
of  the  spine,  the  so-called  traumatic  neurosis. 

In  the  latter  condition  we  may  have  dimness  of  vision,  pain  in  the 
head  and  eyes,  reversion  of  the  color-fields,  and  a  concentric  diminution 
of  the  fields  of  vision,  and  any  of  the  hysterical  phenomena  accom- 
panying the  disease.  This  subject  will  be  fully  discussed  in  another 
chapter. 

The  ocular  phenomena,  when  they  follow  a  true  injury  to  the  spinal 
cord,  may  be  divided  into  three  classes :  first,  changes  in  the  optic  nerve ; 
second,  sympathetic  phenomena  ;  and,  third,  pupillary  changes.  These 
will  be  discussed  in  order. 

Symptoms  of  Involvement  of  the  Optic-Nerve. — Clifford  Allbutt,^^ 
in  1870,  first  called  attention  to  the  changes  in  the  fundus  oculi  in 
injuries  to  the  spinal  cord.  This  author  stated  that  these  phenomena, 
which  consisted  in  a  slight  haziness  of  the  disk  and  a  distention  of  the 
retinal  veins,  did  not  occur  except  in  chronic  cases,  and  only  in  the 
course  of  a  few  weeks  or  months,  and  that  the  higher  the  lesion  the  more 
likely  are  these  symptoms  to  occur.  These  findings  are  somewhat  at 
variance  with  later  reports.  Thorburn  recorded  three  cases  of  injury  to 
the  cervical  cord  with  ophthalmoscopical  changes,  but  in  his  cases  the 
symptoms  appeared  in  the  acute  and  not  in  the  chronic  cases.  Taylor 
and  Collier  ^^  record  two  cases  of  fracture  and  dislocation.  In  one 
case  there  was  a  definite  optic  neuritis  in  one  eye,  apparently  caused  by 
a  piece  of  bone  pressing  upon  the  lower  cervical  cord.  When  this  was 
removed  the  neuritis  rapidly  disappeared. 

The  early  report  of  Allbutt  is  by  no  means  definite,  as  we  are  not 
told  how  often  and  when  the  eye  examinations  were  made.  Again, 
our  knowledge  at  that  early  period,  1870,  was  by  no  means  as  definite 
as  it  is  at  present,  and  we  doubt  somewhat  the  statement  that  changes 
in  the  fundus  occur  only  in  chronic  cases. 

It  is  probable  that  at  times  changes  in  the  disk  occur,  but  these  are 
mild  and  temporary,  and,  therefore,  frequent  and  early  ophthalmo- 
scopical examinations  are  necessary  to  detect  the  condition.  Similar 
changes  have  been  described  in  rare  instances  in  tumors  of  the  cervical 
cord,  as  in  a  case  of  Taylor  and  Collier,  of  an  extramedullary  myxoma 
comprising  the  third  cervical  segment.     Here  there  was  a  double  optic 

''The  Lancet.  Jan.  l.i.  1S70.  "Brain.   1901. 


586  THE    EYE    AND    NERVOUS    SYSTEM 

neuritis.  In  caries  of  the  cervical  vertebrae  we  may  have  slight  changes 
in  the  fundus,  but  in  the  cases  where  there  is  a  definite  optic  neuritis 
an  intracranial  tubercle  probably  is  the  cause. 

It  is  curious  that  no  ocular  disturbances  occur  in  any  case  in  which 
the  lesion  is  below  the  second  dorsal  segment.  A  few  cases  are  on  record 
where  lesions  below  this  area  produced  changes  in  the  fundus,  but  a 
closer  examination  always  showed  involvement  of  the  cervical  cord. 
We  are  led,  therefore,  to  believe  that  in  some  cases  of  injury  to  the 
spinal  cord  we  may  have  a  low-grade  optic  neuritis,  and  that  this  only 
occurs  if  the  cervical  cord  is  involved.  It  is  difficult  to  explain  this. 
We  have  seen  that  optic  neuritis  occurs  in  conjunction  with  myelitis, 
and  this  has  been  explained  by  a  similar  pathological  lesion  in  the  optic 
nerve  and  in  tlie  spinal  cord  due  to  the  same  cause.  But  here  we  have  a 
traumatic  myelitis  of  the  cervical  cord,  as  a  result  of  which  there  are  at 
times  mild  and  sometimes  definite  changes  in  the  optic  nerve.  This  is 
further  shown  in  the  instances  in  which  unilateral  compression  of  the 
cervical  cord  produced  unilateral  changes  in  the  fundus. 

Wharton  Jones  explained  this  relation  by  a  sympathetic  involve- 
ment ;  but  this  is  not  correct,  because  in  lesion  of  the  cervical  sympa- 
thetic we  do  not  have  optic  neuritis.  At  present  no  satisfactory  explana- 
tion can  be  offered ;  further  study — especially  more  accurate  and  fre- 
quent ophthalmoscopic  examinations  in  every  case  of  injury  of  the 
spinal  cord  and  experimental  lesions  on  lower  animals — is  necessary 
to  solve  this  interesting  problem. 

Symptoms  of  Sympathetic  Involvement. — The  cervical  sympathetic 
is  quite  frequently  involved  in  traumatic  lesions  of  the  spinal  cord. 
Our  knowledge  of  the  anatomy  and  physiology'  of  the  sympathetic  sys- 
tem is  by  no  means  exact.  Through  experimental  lesions  upon  lower 
animals  by  Budge  and  others  and  by  clinical  experience,  we  know  that 
in  the  lower  part  of  the  cervical  and  the  upper  part  of  the  dorsal  cord 
there  is  a  so-called  ciliospinal  centre.  This  area  has  been  more  accu- 
rately localized  in  the  eighth  cervical,  and  especially  in  the  first  dorsal 
segment.  The  anterior  roots  of  the  first  dorsal  segment  seem  to  contain 
the  oculopupillary  fibres.  This  has  never  been  better  demonstrated  than 
by  Oppenheim,  who  had  the  opportunity  of  stimulating  with  an  electric 
current  the  exposed  anterior  roots  of  the  first  dorsal  segment  in  a  case 
of  gunshot  injury  to  the  spine.  Oculopupillarj'  symptoms  were  present 
on  stimulating  these  roots,  while  stimulation  of  the  roots  below  had  no 
such  result.     The  eighth  cervical  root  was  not  exposed. 

The  classical  symptoms  of  irritation  of  the  cervical  sympathetic 
are  enlargement  of  the  pupil,  widening  of  the  palpebral  angle,  a  slight 


INJURIES  OF  THE  SPIXAL  CORD 


587 


exophthalmos,  delayed  descent  of  the  upper  lid  on  looking  downwards, 
paleness  of  the  face,  and  increase  of  the  sweat  secretion.  Paralysis  of 
the  cervical  sympathetic  produces  a  small  pupil,  narrowing  of  the  pal- 
pebral angle,  a  slight  enophthalmus,  a  warmth  or  coldness  of  the  face, 
and  hyperidrosis  or  anidrosis.  Intraocular  tension  is  somewhat 
diminished. 


Fig.  5. — Case  of  hemorrhage  or  traumatic  myelitis  in  the  left  eighth  cervical  and  first  dorsal  seg- 
ments, causing  Brown-Scquard  symptoms,  with  sympathetic  paralysis  of  the  left  side,  narrowing  of 
pupil,  narrowing  of  palpebral  fissure,  retraction  of  eyeball,  and  paralysis  of  the  left  hand  and  fore- 
arm. The  photograph  shows  the  patient's  inability  to  firmly  close  the  left  hand.  (Case  of  Dr.  W.  G. 
Spiller,  Polyclinic  Hospital.) 

We,  however,  rarely  see  all  of  these  symptoms  in  a  lesion  of  the 
sympathetic.  This  is  partially  explained  by  the  different  centres  in 
the  spinal  cord  for  the  oculopupillary  and  the  vasomotor,  trophic,  and 
secretory  functions.  C.  Bernard  demonstrated  in  lower  animals  that 
by  cutting  the  first  dorsal  roots  oculopupillary  phenomena  only  were 
produced,  while  cutting  the  sympathetic  fibres  coming  from  the  second 
to  the  fourth  thoracic  vertebrae,  produced  vasomotor  symptoms.  It  is 
generally  thought  that  the  vasomotor  fibres  are  transmitted  from  the 
third  to  the  sixth  dorsal  roots,  inclusive. 

Oppenheim's  case  is  proof  of  this,  for  there  were  no  vasomotor  symp- 


688  THE    EYE    AND    NERVOUS    SYSTEM 

toms  on  irritating  the  first  dorsal  roots.  Kraus  ^*  is  of  the  opinion  that 
there  are  only  oculopupillary  symptoms  if  the  spinal  cord  is  principally 
affected,  but  if  vasomotor  symptoms  predominate,  the  rami  communi- 
cantes  of  the  sympathetic  are  more  involved. 

The  oculopupillary  fibres  leave  the  ciliospinal  centre  by  means  of 
the  rami  communicantes  of  the  first  dorsal  root  and  join  the  inferior 
cervical  sympathetic  ganglion.  Here  these  fibres  are  joined  by  the  vaso- 
motor fibres  from  the  second  to  the  sixth  thoracic  segments,  inclusive. 
By  means  of  the  cerebral  sympathetic,  these  fibres  enter  the  orbit  and 
innervate  the  dilator  pupillae,  the  non-striped  part  of  the  levator  palpe- 
bral superioris,  the  orbital  muscle  of  Miiller,  and  a  small  bundle  of  non- 
stripped  muscle  which  lies  behind  the  globe  of  the  eye  and  bridges 
across  the  sphenomaxillary  fissure  at  the  back  of  the  orbit. 

In  the  majority  of  instances,  the  only  sympathetic  phenomena  ob- 
served in  traumatic  lesions  of  the  cervical  cord  are  a  myosis,  and  some- 
times a  narrowing  of  the  palpebral  fissure.  These  symptoms  are  not 
only  produced  if  the  lesion  is  in  the  eighth  cervical  and  first  dorsal  seg- 
ments, but  as  well  if  the  lesion  is  higher.  We  do  not  know  the  course  of 
the  fibres  of  the  cervical  sympathetic  in  the  upper  cervical  cord,  bulb  and 
the  brain,  but  it  is  probable,  as  Koclier  ^^  thought,  that  these  fibres  run 
together  in  the  eighth  cervical  segment  and  then  diverge  in  their  course 
cerebralwards.  Therefore,  a  lesion  above  the  eighth  cervical  segment 
would  produce  only  partial  symptoms,  as  myosis  and  ptosis,  because 
only  a  number  of  the  sympathetic  fibres  would  be  involved.  A  lesion 
limited  to  one  side  of  the  cord  will  produce  unilateral  sympathetic 
symptoms. 

Gunshot  injuries  of  the  cervical  sympathetic  sometimes  occur.  In 
most  of  these  instances  the  symptoms  produced  are  those  of  paralysis 
of  the  sympathetic  accompanied  by  vasomotor  phenomena.  Tumors  of 
the  substance  of  the  cervical  cord  itself,  and  extramedullary  tumors 
pressing  upon  the  cord  or  upon  the  rami  communicantes  may  produce 
sympathetic  phenomena.  Growths  of  the  glands  of  the  neck,  intra- 
thoracic tumors,  especially  those  of  the  upper  posterior  mediastinum, 
and  aneurisms  of  the  descending  aorta,  subclavian,  common  carotid  and 
vertebral  arteries  have  also  been  known  to  produce  symptoms  of  sym- 
pathetic involvement,  but  these  instances  are  rare. 

Pupillary  Changes. — In  a  number  of  instances  of  injury  to  the  upper 
cervical  cord  the  pupils  have  been  found  irresponsive  to  light,  while  the 
reaction  to  accommodation  and  convergence  was  normal.     The  study  of 

'"Zeitsch.  f.  Klin.  Med.,  1905,  vol.  xviii. 
'»  Die  Liisionen  d.  Riickenm.,  1890.     .Jena. 


INJURIES  OF  THE  SPINAL  CORD  589 

the  cause  of  reflex  iridoplegia  has  very  recently  taken  on  an  additional 
interest,  because  of  the  experiments  of  Bach  and  Meyer.^*'  While  their 
conclusions  have  been  based  upon  experiments  on  lower  animals,  and  as 
yet  clinical  evidence  has  not  substantiated  their  investigations,  still, 
considering  our  meagre  knowledge  of  the  subject,  such  an  important 
contribution  should  be  carefully  considered. 

These  authors  believe  that  in  the  upper  part  of  the  spinal  cord  and 
the  lowest  part  of  the  floor  of  the  fourth  ventricle,  near  the  median  line, 
there  is  an  inhibition  centre  for  the  light  reflex,  and  an  inhibitory  centre 
for  pupillodilation.  Irritation  of  this  part  always  produced  pupillary 
rigidity  and  myosis.  A  right-sided  irritation  produced  reflex  iridoplegia 
upon  the  left  side.  Bach  also  explained  the  myosis  which  always  accom- 
panied the  reflex  iridoplegia  by  a  more  powerful  action  of  the  sphincter 
over  the  weaker  dilator  pupilla',  assuming  that  the  irritation  of  the 
inhibitory  light  reflex  centre  had  aborted  all  other  irritation  in  the 
pupil,  placing  it  in  a  state  of  absolute  rest. 

In  a  series  of  cases  of  tabes  and  general  paresis  Reichardt  ^^  attempts 
to  prove  that  the  reflex  iridoplegia  occurring  in  these  diseases,  and  also 
in  cases  of  fracture  and  dislocation  of  the  upper  cervical  vertebra?,  and 
in  tumors  of  that  region,  are  dependent  upon  the  lesion  in  the  spinal 
cord.  He  examined  cases  with  and  without  failure  of  the  light  reflexes, 
and  in  every  instance  where  there  was  pupillary  rigidity  he  found  a 
degeneration  of  the  endogenous  fibres,  especially  of  the  second  and  third 
cervical  segments,  in  the  area  between  the  posterior  commissure  and  the 
ventral  part  of  the  column  of  Goll,  the  so-called  Bechterew's  inter- 
mediate zone. 

Reichardt  assumes  that  there  are  four  pupillary  centres  in  the  cervi- 
cal cord :  First,  Budge's  centre,  the  so-called  ciliospinal  centre  in  the 
lower  cervical  cord ;  second,  a  pupillodilator  centre  in  the  upper  part 
of  the  cervical  cord ;  third,  an  inhibition  centre  for  the  light  reflex ; 
and  fourth,  a  centre  for  dilation  of  the  pupil  by  irritation  of  the  skin 
covering  the  cervical  region  in  the  fourth  and  fifth  cervical  segments. 
Reichardt  evidently  accepts  Bach  and  Meyer's  experiments  proving  an 
inhibition  centre  for  the  light  reflex  as  conclusive. 

This  author  assumes  that  the  centres  two  and  three,  which  are 
pupillo-dilators  and  pupillo-contractors,  respond  to  all  kinds  of  irrita- 
tion, both  psychical  and  sensory,  thus  causing  a  constant  change  in  the 
pupil.     In  destruction  of  the  endogenous  flbres  of  the  posterior  cord 


^  Arch.  f.  Ophthalm.,  vols.  Iv.  and  Ivi. 

'"Arch.  f.  Psych,  u.  Nervenkr.,  vol.  xxxix.  No.  1. 


590  THE    EYE    AND    NERVOUS    SYSTEM 

there  is  brought  about  an  abnormal  condition  and  a  consequent  failure 
of  the  light  reflex.  An  interruption  of  the  fibres  between  centres  one 
and  three  will  cause  the  same  result,  presuming  that  the  course  of  these 
fibres  is  in  the  posterior  columns. 

Reichardt  does  not  believe  that  a  traumatic  lesion  of  the  upper 
cervical  cord  produces  immediate  pupil  rigidity,  because  the  endogenous 
fibres  would  not  have  time  to  degenerate,  but  there  probably  would  be 
loss  of  the  light  reflex  just  before  death.  In  chronic  cases,  as  in  a  bulbar 
syringomyelia,  there  would  be  destruction  of  these  endogenous  fibres 
and  a  consequent  pupillary  rigidity.  The  endogenous  character  of  the 
lesion  must  be  shown  by  an  absence  of  degeneration  of  the  posterior 
roots  in  the  upper  dorsal  and  cervical  cords.  This  author  also  calls 
attention  to  the  absence  of  pupillary  rigidity  in  traumatic  lesions  of  the 
upper  dorsal  and  lower  cervical  cords,  for  the  lesion  to  produce  this  sign 
must  be  proximal  to  the  fourth  cervical  segment.  A  number  of  cases  of 
fracture  and  dislocation  and  tumors  of  the  cervical  cord,  with  pupillary 
rigidity,  are  quoted  to  prove  the  assertion  of  the  author. 

These  statements  cannot  be  definitely  accepted  without  further  proof. 
It  would  be  well  if  in  all  cases  of  tabes  dorsalis  and  injury  to  the  upper 
part  of  the  spinal  cord,  there  were  a  thorough  microscopical  exami- 
nation made  of  the  uppermost  part  of  the  cervical  cord,  for  it  is  more 
than  probable  that  there  is  some  relation  between  this  region  and  the 
condition  of  the  pupil  and  the  optic  nerve. 

In  a  case  of  dislocation  of  the  third  cervical  vertebrae  with  a  result- 
ing myelitis  of  the  fifth  cervical  segment,  the  pupils  were  noted  to  be 
unequal,  and  a  day  before  death  there  was  no  reaction  of  the  pupils  to 
light.  Examination  of  the  second,  third  and  fourth  cervical  segments 
failed  to  show  any  degeneration  in  any  part  of  the  sections. 

ACUTE   ASCENDING    PARALYSIS    (LANDRY'S    PARALYSIS). 

Considerable  confusion  still  exists  as  to  the  true  nature  of  this  dis- 
order, first .  described  by  Landry  in  1859.  According  to  Landry  the 
symptoms  begin  with  a  diminution  of  power  in  the  lower  limbs,  which 
spreads  rapidly  and  successively  involves  the  muscles  of  the  buttocks, 
loins,  abdomen,  chest,  arms,  and  of  the  neck.  The  limbs  are  flaccid,  the 
reflexes  are  lost  and  there  may  be  some  paresthetic  phenomena,  but  no 
other  sensory  disturbances.  The  bladder  and  rectum  are  never  involved, 
and  the  mind  remains  clear  to  the  end.  The  course  is  rapid  and  may 
be  fatal  in  a  few  days,  either  through  respiratory  or  cardiac  paralysis. 

This  symptom-complex  has  been  modified  to  a  considerable  degree, 
and  while  we  still  recognize  the  rapidly  ascending  form  of  the  paralysis, 


LAXDRY'S  PARALYSIS  591 

other  symptoms  may  be  jDresent.  It  can  be  assumed  that  the  symptoms 
just  described  are  to  be  found  in  the  early  stages  of  the  disorder,  and, 
if  it  be  not  fatal,  we  may  have  added  anaesthesia  of  the  limbs,  abdomen 
and  chest,  pain  on  pressure  over  the  nerve  trunks,  atrophy  of  the  mus- 
cles, reactions  of  degeneration,  and,  sometimes,  paresis  of  the  functions 
of  the  bladder  and  rectum.  If  the  medulla  oblongata  becomes  involved, 
■we  have  added  symptoms  of  bulbar  palsy,  as  difficulty  of  speech  and 
inability  to  swallow. 

The  etiology'  of  the  affection  is  still  obscure.  It  has  been  known  to 
follow  cold,  influenza,  various  infectious  diseases,  and  other  similar 
causes.  The  pathological  findings  in  some  cases  have  been  entirely 
negative,  but  in  most  of  the  recent  autopsies  alterations  in  the  nerve- 
cells  of  the  anterior  horns  of  the  spinal  cord,  and  an  engorgement  of 
the  vessels  of  the  cord  and  the  leptomeninges  have  been  found.  In  some 
instances  diffuse  inflammation  of  the  spinal  cord  has  been  described. 
In  most  cases  a  parenchymatous  degeneration  of  the  peripheral  nerves 
is  present,  and  often  these  were  the  only  pathological  findings,  causing 
some  authors  to  believe  in  the  peripheral  origin  of  the  symptom- 
complex. 

It  is  probable  that  the  disorder  is  due  to  an  acute  intoxication,  and 
the  toxin  may  be  caused  by  various  agents,  and  this  toxin  acts  upon 
the  entire  central  nervous  system.  This  toxic  theory  is  supported  by 
the  uniform  finding  of  an  enlarged  spleen  and  liver,  and  the  febrile 
symptoms  which  usually  usher  in  and  accompany  the  paralysis.  Bac- 
teriologically  a  number  of  bacilli  have  been  found,  but  none  character- 
istic. Buzzard  was  able  to  isolate  a  bacillus  from  the  blood  of  a  patient 
with  Landry's  palsy  which,  when  cultivated  and  injected  into  rabbits, 
caused  the  symptoms  of  an  acute  ascending  paralysis. 

Ocular  Symptoms. — Eye  symptoms  in  Landry's  paralysis  are  un- 
common, because  the  affection  is  usually  fatal  before  the  nuclei  of  the 
eye  muscles  are  involved.  Abducens  paralysis  has  been  noted.  Three 
cases  are  on  record  in  which  ptosis  was  present.  In  the  case  of  Hoff- 
mann ^^  the  patient  had  difficulty  in  articulation  and  in  swallowing, 
and  the  tongue  movements  were  weak.  Both  facial  nerves  were  pa- 
retic, and  there  was  a  right-sided  ptosis.  Bailey  and  Ewing  ^^  recorded 
ptosis  in  another  instance.  Goebel,^^  in  a  brief  report,  mentions  in- 
volvement of  all  the  eye  muscles,  besides  difficulty  in  swallowing,  chew- 
ing, and  talking.     In  these  three  instances  the  autopsy  showed  a  diffuse 

'^  Arch,  f .  Psych.,  vol.  xv. 

=^Xew  York  Medical  Journal,  1896: 

''"Xeurolog.  Centralbl.,  1898,  No.  7. 


692  THE    EYE    AND    NERVOUS    SYSTEM 

myelitic  process  of  the  medulla  oblongata.  In  Goebel's  case,  which 
lasted  for  seventeen  days,  myelitic  degeneration  by  the  method  of 
Marchi  was  found  in  the  area  between  the  oculomotor  nucleus  and  the 
pyramidal  crossing. 

Pupillary  symptoms  are  equally  rare.  Paresis  of  accommodation 
has  been  reported  by  Bernhardt  in  one  instance,  and  unequal  pupils  in 
another.    Slow  reaction  to  light  may  also  be  present. 

In  our  present  knowledge  of  Landry's  symptom-complex  it  is  diffi- 
cult to  give  a  clear  explanation  of  these  ocular  disturbances,  but  it  is 
probable  that  there  is  a  nuclear  or  fascicular  involvement  of  the 
affected  cranial  nerves. 

DISEASES  OF  THE  NERVES. 

In  this  connection  we  shall  consider  only  the  diseases  of  the  brachial 
plexus,  because  in  this  type  of  paralysis  the  rami  communicantes  of 
the  first  dorsal  roots  may  be  involved  and  cause  symptoms  of  sympa- 
thetic paralysis.  Vasomotor  phenomena  are  not  usually  present,  be- 
cause the  probable  course  of  these  fibres  is  through  the  second  to  the 
sixth  thoracic  roots. 

Paralysis  of  the  brachial  plexus  may  be  total  or  partial,  unilateral 
or  bilateral.  If  total,  the  arm  hangs  limply  by  the  side,  no  movements 
being  possible ;  the  muscles  are  atrophic,  electrical  reactions  of  degen- 
eration are  obtained,  and  trophic  phenomena  are  present.  Partial 
brachial  paralysis  may  be  either  of  the  upper  plexus  type,  the  so-called 
Duchenne-Erb  form,  in  which  the  fifth  and  sixth  cervical  roots  or  the 
fibres  in  the  plexus  coming  from  these  roots  are  involved,  or  the 
Klumpke,  or  lower  plexus  type,  in  which  the  eighth  cervical  and  first 
dorsal  roots  are  diseased. 

In  the  upper  plexus  form  the  deltoid,  triceps,  brachialis  anticus,  the 
supinator  longus  and  brevis,  and  the  infraspinatus  muscles  are  diseased. 
It  is  impossible  to  adduct  the  arm,  and  the  forearm  is  extended  and 
pronated.  Sensation,  as  a  rule,  is  not  disturbed.  The  muscles  are 
atrophic,  and  there  may  be  reactions  of  degeneration.  In  the  Klumpke 
paralysis  the  small  muscles  of  the  hand  and  a  number  of  the  muscles  of 
the  forearm,  especially  the  flexors,  are  paralyzed.  Sensory  disturbances 
are  common  in  the  hand  and  forearm,  especially  in  the  ulnar  distri- 
bution. 

These  different  types  of  brachial  plexus  paralyses  are  mostly  trau- 
matic in  origin,  and  may  be  due  to  blows,  gunshot  or  stab  wounds,  frac- 
ture of  the  head  of  the  humerus,  dislocation  of  the  shoulder,  and 
tumors.    The  so-called  obstetrical  and  narcosis  paralyses  are  included  in 


DISEASES  OF  THE   NERVES  593 

the  upper-arm  type.  The  disease  may  also  occur  idiopathically,  the 
cause  probably  being  toxic. 

Ocular  Symptoms. — Oculopupillary  symptoms,  consisting  in  a  nar- 
rowing of  the  pupil  and  of  the  palpebral  fissure,  can  occur  only  if  the 
first  dorsal  roots  are  involved,  either  in  the  intervertebral  foramen,  or 
before  their  separation  from  the  rami  communicantes.  Therefore,  in 
the  lower  arm  type  of  paralysis,  in  which  the  eighth  cervical  and  first 
dorsal  roots  are  diseased,  we  always  have  oculopupillary  symptoms.  It 
is  possible,  however,  to  have  this  type  of  paralysis  without  sympathetic 
involvement,  if  the  fibres  coming  from  these  roots,  in  the  brachial 
plexus,  and  not  the  roots  themselves,  are  diseased.  It  is  difficult,  how- 
ever, to  make  such  a  clinical  differential  diagnosis  because  the  symptoms 
are  identical,  but  we  can  always  assume  that,  if  oculopupillary  symp- 
toms are  present,  the  first  dorsal  root  is  diseased. 

In  involvement  of  the  fifth  and  sixth  cervical  roots  in  the  inter- 
vertebral foramen,  oculopupillary  symptoms  may  occur.  Heubner  ^^^ 
records  three  such  cases.  In  the  first,  there  was  an  osteosarcoma  of  the 
seventh  and  eighth  cervical  vertebrae;  in  the  second,  caries  of  the 
seventh  and  first  dorsal  vertebra^ ;  and,  in  his  third  case,  there  was  a 
tumor  involving  the  same  area.  This  can  be  explained  only  by  the 
accidental  involvement  of  the  rami  communicantes  of  the  first  dorsal 
root,  or  by  involvement  of  the  sympathetic  fibres  in  the  spinal  cord.  In 
disease  of  the  fifth  and  sixth  cervical  roots  outside  of  the  intervertebral 
foramen,  oculopupillary  symptoms  were  noted  in  a  case  by  Oppenheim, 
in  which  a  tumor  of  the  supraclavicular  region  pressed  upon  the  fifth 
and  sixth  cervical  roots,  and  in  another  similar  instance  of  Levy  Dorn's. 
Here  there  must  have  been  an  involvement  of  the  oculopupillary  fibres 
in  the  cervical  sympathetic. 

In  the  Duchenne-Erb  type  of  paralysis,  due  to  a  birth  palsy,  or  the 
paralysis  occurring  in  the  course  of  etherization,  the  traction  upon  the 
arms  may  cause  an  abnormal  stretching  and  tearing  of  the  rami  com- 
municantes of  the  first  dorsal  root,  thus  causing  sympathetic  paralysis 
without  the  first  dorsal  root  itself  being  diseased. 

If  all  of  the  roots  of  the  brachial  plexus  are  diseased,  we  may  have 
oculopupillary  symptoms.  As  a  result  of  gunshot  or  stab  wounds  we 
may  have  forms  of  paralysis  which  do  not  conform  with  any  of  the 
known  types,  with  sympathetic  symptoms.  In  these  cases  the  first  dorsal 
roots  are  either  involved,  or  the  oculopupillary  fibres  in  the  cervical 
sympathetic  are  injured. 

"  Charito  Annalon..  180.5. 
38 


594  THE    EYE    AND    NERVOUS    SYSTEM 

Eulenburg  and  Guttman  do  not  believe  that  sympathetic  phenomena 
accompany  every  brachial  plexus  palsy,  but  are  of  the  opinion  that  in 
these  cases  of  traumatic  neuritis  the  inflammation  spreads  to  the  spinal 
cord  through  the  involved  roots,  causing  a  circumscribed  secondary 
myelitis,  and  that  this  myelitis  occurring  in  the  lowest  part  of  the  cervi- 
cal and  first  dorsal  segments  causes  oculopupillary  symptoms.  While 
this  may  be  true  in  some  instances,  it  is  not  the  rule,  for  experimental 
investigations  have  demonstrated  that  cutting  the  first  dorsal  roots 
will  cause  immediate  oculopupillary  phenomena. 

MULTIPLE  NEURITIS. 

Multiple  neuritis  is  produced  by  a  variety  of  causes,  chronic  alco- 
holism being  the  most  common.  Toxic  disturbances,  such  as  are  pro- 
duced by  the  various  infectious  diseases  and  the  different  metallic 
poisons,  the  chronic  diseases  such  as  tuberculosis  and  malaria,  beriberi, 
leprosy  and  senility,  may  produce  polyneuritis.  The  disease  may  come 
on  without  any  apparent  cause  or  follow  a  cold,  it  probably  being  in 
these  instances  infectious  in  origin. 

The  symptoms  of  multiple  neuritis  may  develop  suddenly  or  sub- 
acutely.  The  patient  first  complains  of  a  feeling  of  numbness  in  his 
feet  or  hands,  or  of  sharp  pains,  these  gradually  increasing  in  severity. 
A  gradual  weakness  in  the  lower  or  the  upper  limbs  then  appears,  but 
sometimes  the  loss  of  power  may  come  on  very  suddenly.  There  may 
be  accompanying  febrile  symptoms,  and  the  patient  may  be  delirious. 
The  muscles  are  flaccid  and  the  tendon  and  skin  reflexes  are  abolished. 
There  is  tenderness  and  pain  on  pressure  over  the  nerve  trunks  and  the 
pains  may  become  very  acute.  Sensation  is  usually  affected,  there  being 
first  hyperesthesia  followed  by  anjEstliesia.  The  paralysis  is  sym- 
metrical and  involves  in  the  lower  limbs  principally  the  peroneal  and 
anterior  tibial  nerves,  causing  bilateral  foot  drop,  and  in  the  upper 
limbs  the  distribution  of  the  radial,  median  and  ulnar  nerves,  caus- 
ing wrist  drop.  The  paralysis  is  degenerative  in  character  and  in  a 
short  time  electrical  reactions  of  degeneration  are  usually  obtained. 
The  bladder  and  rectum  are  hardly  ever  involved,  this  being  an  im- 
portant diagnostic  feature.  Vasomotor  and  trophic  phenomena  are 
sometimes  present. 

At  times  ataxia  of  the  upper  and  lower  limbs  is  present  and  the 
patient  is  unable  to  walk  because  of  this.  This  form  of  multiple 
neuritis  is  sometimes  called  peripheral  neuro-  or  pseudo-tabes  and  it  is 
at  times  impossible  to  distinguish  this  disease  from  tabes  dorsalis.  We 
have  not,  however,  in  the  former  the  chronic  onset,  the  Argyll-Robertson 


MULTIPLE  NEUKITIS  595 

pupil,  the  girdle  sense  and  the  involvement  of  the  bladder  and  rectum, 
this  being,  jjerhaps,  sufficient  to  make  a  different  diagnosis. 

The  cranial  nerves  are  usually  not  affected  in  the  course  of  multiple 
neuritis,  but  instances  of  multiple  neuritis  limited  to  the  cranial  nerves 
have  been  noted.  The  ocular  nerves  are  more  often  involved  than  the 
other  cranial  nerves,  especially  the  sixth  nerve.  The  facial  nerves  are 
sometimes  diseased,  especially  in  conjunction  with  the  sixth  nerve; 
more  rarely  the  trigeminus  and  acoustic  nerves  are  involved.  The  vagus 
is  more  commonly  affected,  especially  in  severe  cases,  causing  an  in- 
crease of  the  pulse  rate,  and  sometimes  death.  The  involvement  of 
the  ocular  nerves  will  later  be  fully  discussed.  The  symptoms  above 
described  are  largely  common  to  all  forms  of  mvyltiple  neuritis;  the 
special  forms  will  be  taken  up  separately  and  the  ocular  symptoms 
accompanying  them  will  be  dwelt  upon. 

Alcoliolic  Multiple  Neuritis. — The  symptoms  above  described  are 
essentially  those  of  alcoholic  multiple  neuritis.  We  sometimes  have 
in  addition  a  peculiar  psychic  involvement  which  consists  of  loss  and 
confusion  of  memory  for  recent  events,  and  in  addition  illusions  and 
hallucinations.     This  is  sometimes  called  Korsakoff's  psychosis. 

Pathologically,  we  have  an  inflammatory  or  parenchymatous  de- 
generation of  the  different  peripheral  nerves,  especially  of  the  muscular 
and  sensory  branches  of  the  skin.  It  is  not  uncommon,  however,  to  find 
in  this  disease  an  alteration  in  the  cells  of  the  anterior  horn  and  of  the 
medulla  oblongata  and  pons.  These  changes  in  the  nerve-cells  consist 
of  a  displacement  of  the  nucleus  to  the  periphery,  and  a  central  chroma- 
tolysis.  Diffuse  and  degenerative  inflammations  with  hemorrhages  have 
also  been  noted,  especially  in  the  region  of  the  gray  matter  of  the  third 
ventricle  and  of  the  aqueduct  of  Sylvius.  This  was  first  described  by 
Wernicke,^^  who  termed  the  disease  acute  hemorrhagic  polioencephalitis 
superior  and  inferior,  depending  whether  the  upper  or  the  lower  cranial 
nuclei  were  involved.  A  degeneration  of  the  anterior  and  posterior  roots 
and  of  the  posterior  and  anterior  horns  has  also  been  described.  These 
changes,  however,  are  not  to  be  confused  with  those  of  multiple  neuritis, 
in  which  we  have  only  alterations  in  the  peripheral  nerves. 

Alterations  in  the  muscles  are  not  uncommon.  These  may  consist 
only  of  a  narrowing  of  the  muscle  fibres,  or  they  may  be  more  severe, 
causing  a  loss  of  striation  of  the  fibres  and  increase  of  the  nuclei. 
Thomsen  ^^  described  in  one  case  sigiilar  alterations  in  an  eye  muscle. 

"Lehrbuch  der  (Jehirnkrankh.,  vol.  ii.  1881. 
"Arch.  f.  Psych.,  1890. 


596  THE    EYE    AND    XERVOUS    SYSTEM 

Ocular  Syinpto)ns. — Ocular  symptoms  are  not  very  rare.  We  shall 
take  up  first  the  alterations  in  the  optic  nerve,  then  the  pupillary 
phenomena,  and  lastly  the  involvement  of  the  motor  cranial  nerves  of 
the  eye. 

Disturbance  of  sight  is  uncommon,  but  when  it  does  occur  it  is 
generally  in  the  nature  of  a  central  scotoma,  especially  for  colors. 
Blindness  has  been  reported  in  a  number  of  instances,  but  in  none  is 
there  an  uncomplicated  history.  In  the  clearest  case,  that  of  Render,^* 
the  ophthalmoscopic  examination  was  not  given,  and  a  number  of  the 
other  cranial  nerves  were  involved.  In  the  other  reported  cases  albumin 
was  always  found  in  the  urine. 

Optic  Nerve. — The  optic  nerve  is  very  seldom  involved  in  alcoholic 
multiple  neuritis.  A  pathological  whitish  paling  of  the  temporal  side 
of  the  disk  has  been  described,  especially  by  Uhthoff,^®  who  examined 
one  thousand  alcoholics  and  found  such  changes  in  139.  He  found, 
by  histological  examination,  a  retrobulbar  neuritis  in  six  of  these  in- 
stances. It  cannot  be  assumed,  however,  that  the  pathological  paling  of 
the  temporal  side  of  the  disk  depends  upon  the  retrobulbar  neuritis,  for 
either  condition  can  occur  without  the  other  being  present.  Optic 
neuritis  is  an  exceedingly  rare  occurrence.  I  have  been  able  to  find  in 
the  literature  only  seven  recorded  cases.  The  neuritis  may  be  well 
marked  in  both  optic  nerves,  or  may  be  limited  to  one.  It  is  usually 
very  mild  in  character,  but  may  be  very  severe.  The  neuritis  comes  on 
generally  late  in  the  disease  and  in  most  instances  disappears  as  the 
disease  improves.  In  all  of  the  recorded  cases,  except  in  one,  a  part  or 
all  of  the  third,  sixth  and  seventh  nerves  were  also  involved. 

I  have  had  under  my  observation  such  a  case  in  the  wards  of  the 
Philadelphia  Hospital.  This  man,  forty  years  of  age,  has  been  drink- 
ing steadily  for  the  past  eighteen  years,  mostly  whiskey,  sometimes  as 
much  as  twenty-five  drinks  a  day.  He  has  had  delirium  tremens  once, 
six  years  ago.  He  smoked  somewhat,  but  denies  venereal  history.  Four 
months  ago  he  first  began  to  have  pain  in  both  eyes  and  complained  of 
photophobia  and  of  a  discharge  from  both  eyes.  Dimness  of  vision  also 
became  apparent  in  both  eyes  about  the  same  time.  The  pain  was 
relieved  somewhat  by  the  wearing  of  blue  glasses,  but  sight  has  grad- 
ually diminished.  He  never  had  diplopia.  Examination  of  the  eyes  by 
Dr.  E.  A.  Shumway  was  as  follows: 

R,  V.  5/30.     L.  V.  5/35.     Suliacute  conjunctivitis  in  both  eyes. 


'The  American  Lancet,  1890. 

•Arch.  f.  Ophthalm.,  vols,  xxxii.  and  xxxiii.  1887. 


MULTIPLE  is^EURITIS  597 

The  pupils  are  equal  and  respond  promptly.  The  fields  are  normal. 
^o  color  scotoma.  Green  is  recognized  badly.  Ophthalmoscopic  ex- 
amination. Right  eye:  Media  clear,  low  grade  optic  neuritis.  Left 
eye:  Similar  low  grade  optic  neuritis,  with  fine  haze  in  the  vitreous, 
posterior  part. 

The  patient  had  dimnution  of  power  in  all  of  his  limbs ;  the  reflexes, 
while  not  lost,  were  much  diminished ;  there  was  pain  on  pressure  over 
the  nerve  trunks  and  spontaneous  shooting  pains  in  the  limbs.  He  has 
had  previous  attacks  of  loss  of  power  in  his  lower  limbs  and  has  com- 
plained of  pain  for  a  long  period.  The  urine  examination  showed  a 
very  slight  trace  of  albumin.  The  neuritic  symptoms  have  improved, 
but  the  optic  neuritis  still  persists,  and  sight  is  no  better. 

In  these  cases  we  must  exclude  the  occurrence  of  a  large  amount 
of  albumin  in  the  urine,  for  a  nephritic  condition  might  readily  cause 
the  optic  neuritis.  In  only  one  of  the  recorded  cases,  that  of  Thomsen,^^ 
was  there  a  pathological  examination.  Here  the  examination  of  the 
optic  nerve  was  not  mentioned,  although  the  author  states  that  the 
examination  of  the  brain  and  the  spinal  cord  was  entirely  negative  and 
that  the  disease  was  limited  to  the  peripheral  nerves. 

It  is  difficult  to  explain  why  the  toxic  process  caused  by  the  alcohol 
should  have  a  selective  action  upon  certain  peripheral  nerves,  and  still 
more  difficult  to  explain  why  it  should  involve  the  optic  nerve,  which 
is  not  really  a  nerve  at  all.  As  a  rule,  intoxications  which  affect 
the  peripheral  nerves  will  not  affect  the  optic  nerve.  From  the  in- 
sufficient number  of  cases  on  record  and  the  absence  of  pathological 
and  experimental  investigations,  we  cannot  assume  that  the  optic 
neuritis  is  caused  by  the  toxin  of  alcohol.  It  is  probable  that  the  optic 
neuritis  which  is  present  may  be  due  to  the  chronic  meningitis  which  is 
present  in  some  alcoholics. 

Pupils. — The  pupils  are  usually  not  affected  in  alcoholic  multiple 
neuritis,  but  a  difference  in  size  is  not  unusual.  I  have  seen  this  in  a 
number  of  instances.  Uhthoff  in  an  examination  of  one  thousand  alco- 
holics found  a  difference  in  the  size  of  the  pupils  in  sixty  of  his  cases. 
In  twenty-five  there  was,  besides,  a  slow  reaction  of  the  pupil  to  light, 
and  in  ten  instances  there  was  a  reflex  pupillary  rigidity,  the  reaction  to 
accommodation  being  nearly  alwa^'S  preserved.  Oppenheim  records 
reflex  iridoplegia  in  both  pupils,  and  in  one  eye  accommodation  was 
also  lost.  Eperon  records  a  similar  instance  without,  however,  loss  of 
accommodation.     Moeli  is  of  the  opinion  that  pupillary  rigidity  is  not 


*"Neurolog.  Centralbl.,  vol.  vi.  No.  1. 


598  THE    EYE    AND    NERVOUS    SYSTEM 

permanent  in  alcoholics,  it  being  transient  in  character.  I  have  also 
observed  this  in  one  instance. 

Ocular  Nerves. — The  eye  muscles  are  not  often  involved.  Unilateral 
external  rectus  palsy  is  the  most  common  form  of  ocular  paralysis,  but 
both  abducens  nerves  may  be  paralyzed.  The  seventh  nerve  is  at  times 
also  involved,  generally  with  the  sixth  nerve.  In  two  patients  recently 
under  my  observation,  external  rectus  palsy  was  present  in  each,  while 
in  one  the  left  seventh  nerve  was  also  slightly  paretic.  In  one  patient, 
hearing  was  for  a  time  diminished.  The  fifth  nerve  is  sometimes  also 
diseased.  Dr.  Spiller  very  kindly  furnished  me  with  the  records  of  a 
case  of  alcoholic  polyneuritis  in  which  the  exit  points  of  the  fifi;h  nerve 
were  tender  to  pressure  and  the  patient  complained  of  a  feeling  of 
numbness  in  his  face.     This  disappeared  in  a  short  time. 

One  or  both  oculomotor  nerves  may  be  involved.  More  often  the 
outer  musculature  of  the  oculomotor  nerve  alone  is  diseased,  causing 
ptosis,  while  the  inner  musculature  remains  free,  iritic  motion  and 
accommodation  not  being  disturbed.  Edinger  explained  this  peculiar 
involvement  by  the  theory  that  the  external  muscles  are  paralyzed  be- 
cause more  strain  is  thrown  upon  them  by  the  poison.  This,  however, 
is  not  a  satisfactory  explanation. 

Uhthoff,  in  his  one  thousand  alcoholics,  found  paralysis  of  the  ocular 
muscles  in  twenty-two  cases ;  in  four  there  was  transient  diplopia,  and 
in  two  bilateral  abducens  paralysis.  The  ocular  palsies  usually  appear 
in  the  height  of  the  disease,  in  about  the  third  or  fourth  week,  and  are 
usually  transient.  In  my  cases,  the  abducens  palsy  came  on  in  the 
second  week  and  lasted  for  about  two  weeks,  and  then  disappeared.  In 
one,  four  weeks  afterwards  the  bilateral  abducens  palsy  reappeared  with 
a  weakness  of  the  left  seventh  nerve.  Besides,  the  woman  had  diffi- 
culty in  swallowing  and  she  had  the  dull,  monotonous  speech  of  bulbar 
palsy.  The  pupils  were  unequal  and  wider  than  normal  and  reacted  to 
light  and  in  accommodation.  The  neuritic  symptoms  were  still  severe. 
In  the  course  of  a  few  weeks  the  bulbar  symptoms  disappeared. 

Pathology. — While  the  number  of  recorded  cases  of  alcoholic  mul- 
tiple neuritis  with  microscopic  examination  is  large,  those  with  in- 
volvement of  the  ocular  nerves  are  by  no  means  common.  Marina  ^* 
recorded  a  case  of  a  man  of  sixty-one  with  involvement  of  the  left 
oculomotor  and  the  right  abducens  and  facial  nerves,  with  the  symp- 
toms of  multiple  neuritis.  A  careful  microscopical  examination  of 
the  peripheral  nerves  and  of  the  brain  and  spinal  cord  gave  entirely 


*'  Ueber  multip.    AugenlUhmung,  Leipzig  u.  Wien,  1896. 


MULTIPLE  NEURITIS 


599 


negative  results.     It  is  well  known,  however,  that  this  can  occur  in 
polyneuritis  of  toxic  origin. 

A  number  of  cases  are  recorded  in  which  the  microscopical  examina- 
tion showed  a  peripheral  degenerative  neuritis  of  the  ocular  nerves, 
without  an  involvement  of  their  nuclei.  Such,  among  others,  are  the 
cases  of  Dammront  and  Mayer,  in  which  there  was  bilateral  external 
ophthalmoplegia,  besides  an  involvement  of  the  seventh,  ninth  and 
twelfth  nerves;  of  Thomsen,  in  which  there  was  bilateral  abducens 
palsy  and  ptosis,  besides  a  nystagmus,  and  a  mild  left  optic  neuritis. 


Fig.  6.— Case  of  alcoholic  multiple  neuritis  with  external  rectus  palsy  and  bilateral  wrist-drop. 
The  external  rectus  palsy  is  not  shown  in  the  photograph. 

In  Thomson's  case  the  only  branch  of  the  oculomotor  nerve  diseased 
was  the  twig  to  the  levator  palpebrae  muscle. 

In  a  later  report,  Thomsen,^  ^  in  a  case  of  alcoholic  polyneuritis  with 
ocular  symptoms,  found  the  peripheral  ocular  nerves  and  their  roots 
and  nuclei  intact,  but  found  hemorrhages  in  the  gray  matter  of  the  third 
ventricle,  and  came  to  the  conclusion  that  the  ocular  palsies  occurring  in 
alcoholic  multiple  neuritis  were  due  to  a  cerebral  lesion,  the  polio- 

'-  Arch,  f.  Psych.,  vol.  xxi.  Case  III. 


600  THE    EYE    AND    NERVOUS    SYSTEM 

encephalitis  hemorrhagica  superior  of  Wernicke.  This  observation  has 
since  been  confirmed  by  others,  as  Boedeker  and  Rennert.  Cases  are 
on  record,  however,  in  which,  besides  this  encephalitis,  the  cranial 
nerves  themselves  were  the  seat  of  a  degenerative  neuritis  (Thomsen 
and  Jacoba?us).  Again,  Gudden '^^  reported  a  case  of  polyneuritis  in 
which  the  encephalopathic  process  involved  the  region  of  the  third 
ventricle  and  the  aqueduct  of  Sylvius  without  producing  any  ocular 
symptoms. 

We  may,  therefore,  have  in  alcoholic  multiple  neuritis:  first,  no 
pathological  changes  either  in  peripheral  ocular  nerves  or  in  their  cen- 
tral connections ;  second,  alterations  only  in  the  peripheral  ocular 
nerves ;  third,  changes  only  in  the  region  of  the  gray  matter  of  the 
third  ventricle  and  the  acpieduct  of  Sylvius ;  fourth,  a  combination  of 
the  degenerative  lesions  of  the  peripheral  ocular  nerves  with  the  enceph- 
alitic  process  of  Wernicke. 

Clinically,  it  is  almost  impossible  to  differentiate  between  these 
pathological  processes.  A  rapid  onset  with  a  bilateral  ophthalmoplegia, 
in  which  both  the  outer  and  the  inner  musculature  are  involved,  with  a 
j)0ssible,  accompanying  optic  neuritis,  may  point  to  an  acute  polioen- 
cephalitis. 

Xystagmus  has  been  observed  repeatedly  in  alcoholic  polyneuritis, 
especially  on  lateral  deviation.  Such  cases  have  been  recorded  by 
Opixjnheim  and  others.  Uhthoff,  in  his  one  thousand  alcoholics,  found 
in  thirteen,  nystagmoid  movements  on  lateral  excursion,  and  in  two 
definite  nystagmus.  Thomsen  in  a  similar  examination  of  a  number 
of  alcoholics  found  nystagmus  in  five.  I  have  observed  similar  nystag- 
moid movements  in  a  number  of  my  cases,  but  always  on  lateral  devia- 
tion. External  rectus  palsy  had  been  present  It  has  seemed  to  me 
that  these  were  nystagmoid  movements,  due  to  the  weakness  of  the 
abducens  nerves  and  not  true  nystagmus.  In  none  of  the  cases  of  poly- 
neuritis was  there  nystagnuis  on  direct  fixation. 

Lead  Multiple  Neuritis. — The  effects  of  lead  on  the  central  ner- 
vous system  are  well  known.  Workers  in  lead,  typesetters  and  others, 
who  come  in  contact  with  this  metal  often  suffer  from  this  disease.  The 
symptoms  of  lead  neuritis  are  generally  preceded  by  lead  colic.  The 
poison  seems  to  have  a  peculiar  affinity  for  the  posterior  interosseous, 
causing  paralysis  of  the  extensors  of  the  hand  and  fingers,  while  the 
supinator  longus  and  triceps  nmscles  are  usually  spared.  The  nerves  of 
the  lower  extremity  are  not,  as  a  rule,  involved,  but  if  they  are,  the 


Arch.  f.  Psych.,  189G. 


MULTIPLE  XEURITIS  601 

peroneal  nerve  is  usually  diseased,  the  tibialis  anticns  nearly  alwav;* 
escaping.  Sensation  is  not  often  disturbed.  Ataxia  hardly  ever  occurs. 
The  muscles  are  atrophic  and  reactions  of  degeneration  are  soon  ob- 
tained. A  blue  line  on  the  gums  is  an  aid  to  the  diagnosis  of  lead 
palsy. 

In  some  instances  lead  poisoning  causes  intracranial  symptoms ; 
this  is  termed  lead  encephalopathy.  Here  we  may  also  have  paralysis 
of  the  cranial  nerves  and  of  the  vocal  cords  and  the  laryngeal  muscles. 
All  of  the  ocular  nerves,  the  third,  fourth  and  sixth,  may  be  involved, 
either  alone  or  in  combination.  Either  optic  neuritis  or  atrophy  may 
occur.  Delirium,  coma,  convulsions,  epileptic  seizures,  hemorrhages 
and  transient  hemiplegia  have  been  observed  in  the  course  of  lead 
encephalopathy.     This  subject  is  discussed  in  another  chapter. 

Pathology. — Pathologically,  the  predilection  of  the  lead  poisons  for 
the  peripheral  nerves  has  been  well  established.  The  neuritis  is  de- 
generative in  character.  Alterations  in  the  cells  of  the  anterior  horn, 
as  described  by  Oppenheim,  Spiller  and  others,  may  occur,  and  the 
anterior  roots  have  also  been  found  diseased.  Goldflam  described 
hemorrhages  in  the  anterior  and  posterior  horns.  The  muscles  are  the 
seat  of  degeneration. 

In  the  literature  there  are  many  reported  cases  of  involvement  of 
the  cranial  nerves  in  the  course  of  lead  multiple  neuritis,  but  in  view 
of  the  scarcity  of  microscopical  examination  and  our  insufficient  knowl- 
edge of  the  patholog}-  of  lead  palsy,  there  is  doubt  regarding  the  exist- 
ence of  a  pure,  peripheral  multiple  neuritis  with  involvement  of  the 
cranial  nerves. 

Partial  paralysis  in  the  distribution  of  the  vago-accessory  and  paraly- 
sis of  the  trigeminus,  facial  and  the  hypoglossus  nerves  have  been  re- 
ported, but  always  with  involvement  of  one  or  more  of  the  other  cranial 
nerves.  A  mononeuritis  of  a  cranial  nerve  is  not  on  record.  A  critical 
analysis  always  shows,  besides,  symptoms  of  cerebral  involvement.  It 
is  striking  also  that  when  facial  paralysis  does  occur  it  is  confined  only 
to  the  lower  distribution,  this  arguing  for  a  central  lesion. 

A  number  of  cases  are  noted  in  which  the  multiple  neuritis  was 
said  to  be  confined  only  to  the  cranial  nerves.  Such  are  the  cases  of 
Pal,^'*  Mannaberg,  and  Chvostek.^^ 

In  Pal's  case  a  typesetter  of  twenty-one  had  for  two  years  attacks 
of  eclampsia,  during  which  she  had  right-sided  abducens  and  facial 


"Ueber  multip.  Neuritis,  Wien.   18D1. 
"Wiener,  klin.  Woch..  189S.  No.  52. 


602  THE    EYE    AND    NERVOUS    SYSTEM 

paralysis.  After  a  severe  attack  of  lead  colic,  disturbances  of  vision 
appeared,  followed  by  optic  neuritis  and  right-sided  abducens  and  facial 
palsy,  with  electrical  reactions  of  degeneration.  Shortly  total  amaurosis 
with  right-sided  ptosis  became  apparent.  There  was  also  ataxia  of  the 
upper  limbs  with  bilateral,  peroneal  palsy  of  the  lower  extremities.  At 
the  necropsy  the  gray  matter,  especially  of  the  occipital  lobes,  the 
internal  capsule  and  the  medulla  oblongata  were  harder  than  normal. 
No  mention  is  made  of  a  microscopic  examination  of  these  parts.  The 
microscopical  examination  of  the  optic  and  the  third,  sixth  and  seventh 
cranial,  the  radial  and  peroneal  nerves  showed  partial  degeneration.  In 
the  spinal  cord  there  was  a  degeneration  of  the  posterior  roots  and  of 
some  of  the  tracts. 

In  the  two  cases  reported  by  Mannaberg  there  was  headache,  dizzi- 
ness, bilateral  optic  neuritis,  right-sided  facial  palsy  and  right-sided 
neuralgia,  limited  to  the  distribution  of  the  fifth  nerve  in  one  case, 
while  in  the  other  there  was  bilateral  neuroretinitis  and  right-sided 
facial  and  abducens  palsy. 

In  Chvostek's  case  there  was  paralysis  of  both  oculomotor,  the  left 
abducens  and  the  right  facial  nerves  and  bilateral  optic  neuritis.  There 
were  also  severe  cerebral  symptoms.  At  the  necropsy  the  brain  sub- 
stance was  extremely  oedematous,  the  convolutions  were  flattened  and 
the  oculomotor  and  abducens  nerves  were  found  degenerated.  This 
degeneration  was  probably  due  to  the  pressure  caused  by  the  oedema. 

In  every  one  of  these  cases  there  were  some  symptoms  of  involve- 
ment of  the  peripheral  nerves,  besides  symptoms  of  cerebral  involvement. 
It  is  probable  that  a  careful  microscopical  examination  of  the  brain 
and  cord  would  have  shown  the  alterations  found  in  lead  encephalop- 
athy. It  is  improbable,  considering  the  nature  of  the  poison  and  its 
widespread  effects  upon  the  cerebrospinal  system,  that  the  cranial  nerves 
should  be  affected  without  involvement  of  the  brain  substance.  We  can 
conclude,  therefore,  that  multiple  neuritis,  limited  to  the  cranial  nerves, 
does  not  occur,  and  that  if  in  the  course  of  a  lead  polyneuritis,  symptoms 
of  involvement  of  the  cranial  nerves  appear,  the  brain  itself  is  diseased. 

Optic  neuritis  alone  has  been  observed  repeatedly  in  the  course  of 
chronic  lead-poisoning.  Goldscheider  ^^  has  called  attention  to  the  in- 
teresting fact  tliat  optic  neuritis  may  occur  alone  in  conditions  which 
lead  to  polyneuritis,  as,  for  instance,  in  the  infectious  diseases  and  in 
the  different  metallic  poisons.  It  is  probable,  however,  that  in  these 
cases  the  optic  neuritis  is  not  a  precursor  of  a  multiple  neuritis,  but 

"  Charity  Annalen,  vol.  xvii. 


MULTIPLE  NEUKITIS  603 

that  it  is  a  manifestation  of  a  cerebral  disease.  As  has  been  previously 
mentioned,  the  optic  nerves  are  not,  as  a  rule,  affected  by  the  same 
toxic  processes  that  are  prone  to  involve  the  peripheral  nerves. 

Changes  in  the  size  of  the  pupils,  with  loss  of  the  light  reflex,  have 
been  observed,  but  these  are  probably  also  encephalic  symptoms. 

Paralysis  of  the  eye  muscles  without  involvement  of  the  other 
cranial  nerves  is  not  very  common  in  lead-polyneuritis.  In  1898  Elsch- 
nig  ^^  collected  sixteen  cases  from  the  literature.  In  these  the  eye 
muscles  were  either  unilaterally  or  bilaterally  involved.  In  four  in- 
stances the  oculomotor  nerve  was  totally  paralyzed.  The  trigeminus  and 
the  abducens  nerves  were  also  involved  in  several  instances.  There 
was,  however,  in  these  cases  no  clear  history,  and  syphilis  could  not 
be  excluded  in  several  instances.  Here  also  we  may  assume  that  these 
paralyses  were  only  a  part  of  the  general  lead  encephalopathy. 

Arsenical  Neuritis. — Arsenic  is  a  frequent  cause  of  polyneuritis, 
but  I  have  been  unable  to  find  any  reference  to  involvement  of  any  of 
the  ocular  muscles.  Optic  neuritis  has  been  reported  by  Higier,  but 
it  is  probable  that  there  was  a  cerebral  involvement  in  his  case. 

Polyneuritis  due  to  other  Metallic  Poisons. — Mercury,  copper, 
phosphorus,  and  carbon  disulphide  have  also  been  known  to  produce 
multiple  neuritis,  but  these  instances  are  rare. 

The  form  of  polyneuritis  due  to  carbon  disulphide  is  extremely  un- 
common and  little  is  known  of  its  pathology,  as  observations  are  lack- 
ing. Workers  in  vulcanized  rubber  are  especially  prone  to  this  disease. 
Mental  excitation  or  depression  with  hysterical  manifestations  precede 
the  neuritis  phenomena,  which  are,  as  a  rule,  of  the  ataxic  form  and 
resemble  greatly  the  alcoholic  type  of  neuritis.  Hysterical  symptoms 
are  so  common  that  some  authors,  as  Marie,  insist  that  the  hysterical 
manifestations  are  among  the  most  prominent  symptoms. 

Ocular  phenomena  are  common  and  consist,  as  a  rule,  of  alterations 
in  the  visual  fields,  especially  for  colors.  Amblyopia  is  a  fairly  con- 
stant symptom.  Scotoma  either  large  or  small  is  found  in  some  cases. 
Pupillary  rigidity  and  even  nystagmus  have  been  recorded.  In  this 
country  within  the  year  1905  Jump  and  Cruice  were  the  first  to  report 
two  such  cases,  one  of  which  I  have  had  the  privilege  of  studying. 
These  cases  showed  marked  hysterical  symptoms.  The  eye  examination 
by  Dr.  Langdon  of  the  first  case  showed  the  optic  disks  to  be  slightly 
congested  with  hypersemia  of  the  retinal  vessels.  The  visual  fields  were 
much  contracted. 

"  Wiener,  med.  Wochen.,  1898,  Nos.  27-29, 


604  THE    EYE    AND    NERVOUS    SYSTEM 

It  is  probable  that  we  have  here  not  a  pure  multiple  neuritis  but  a 
toxic  ])rocess  which  so  influences  the  central  nervous  system  as  to  pro- 
duce the  various  mental,  ocular  and  neuritic  symptoms.  It  must  be 
acknowledged  that  most  of  the  various  manifestations  shown  in  this 
disease  are  hysterical  in  nature;  but  why  should  a  previously  healthy 
individual  who  is  poisoned  by  carbon  disulphide  be  hysterical  only  so 
long  as  the  influence  of  the  poison  lasts  ? 

The  influence  of  toxins,  whether  generated  within  or  without  the 
body,  upon  the  brain  and  spinal  cord  is  becoming  better  recognized. 
It  is  more  than  probable  that  their  influence  is  not  selective  but  gen- 
eral, and  that  we  have  alterations  not  only  in  the  peripheral  nerves, 
but  also  in  the  central  nervous  system.  Such  is  the  ease,  for  instance, 
in  lead  or  alcoholic  poisons  and  in  uraemia. 

Carbon  Monoxide  Neuritis. — Illuminating  gas,  or  carbon  monox- 
ide, may  produce  symptoms  which  resemble  multiple  neuritis.  Involve- 
ment of  the  different  cranial  nerves  is  not  uncommon.  Cases  are 
recorded  in  which  the  third,  fourth,  fifth,  sixth,  and  seventh  nerves  were 
paralyzed  either  wholly  or  in  part.  The  paralysis  persists  only  for  a 
short  time  and  disappears  as  the  general  symptoms  improve.  Blindness 
has  also  been  recorded.  Sibelius  reported  an  interesting  case  of  a  boy 
of  twenty,  who,  after  exposure  to  carbon  monoxide  for  five  minutes, 
became  unconscious.  He  regained  consciousness  the  following  day  when 
it  was  found  that  the  boy  was  blind.  The  pupils  were  large  and  reac- 
tionless  and  the  left  seventh  nerve  was  paretic.  Subsequently  weakness 
of  the  extremities  developed  w'itli  dementia  and  epileptic  seizures.  The 
ocular  symptoms  persisted  until  death,  which  occurred  three  months 
after.  The  microscopical  examination  showed  a  diffuse  encephalitis 
and  a  disease  of  the  blood-vessel  walls,  but  no  thrombosis.  The  amauro- 
sis was  explained  by  a  bilateral  area  of  softening  in  the  occipital  lobe. 

This  case  illustrates  well  that  toxic  processes  do  not  produce  local 
alteration  in  the  peripheral  nerves,  but  that  they  have  a  general  action 
on  the  whole  central  nervous  system. 

Sulphonal  and  Trional  Neuritis. — Since  the  introduction  of  coal- 
tar  derivatives,  poisoning  by  these  drugs  is  becoming  known.  This  is 
especially  so  with  sulphonal.  Erbsloh  recorded  an  instance  of  a  man  of 
forty  who  had  taken  two  grammes  of  sulphonal  daily  for  five  days 
and  developed  symptoms  of  polyneuritis.  Besides,  the  right  pupil  was 
larger  than  the  left,  the  left  abducens  nerve  was  paralyzed  and  he  had 
hallucinations  of  sight.  At  the  necropsy  the  peripheral  nerves  were 
found  degenerated.  Dillingham  also  records  ptosis,  which  lasted  about 
ten  days. 


MULTIPLE  NEURITIS  605 

Diphtheritic  Paralysis. — Approximately  about  one-quarter  of  the 
total  number  of  cases  of  diplitlieria  are  followed  by  paralysis.  It  is 
more  liable  to  follow  a  severe  attack,  although  paralysis  has  been  known 
to  follow  a  simple  sore  throat  or  diphtheritic  inflammation  elsewhere 
in  the  body.  The  older  the  person  the  greater  is  the  tendency  to 
paralysis.     (See  Chapter  V,  p.  231.) 

As  a  rule,  the  paralysis  does  not  appear  until  the  diphtheria  has 
entirely  disappeared,  in  the  third  or  fourth  week  and  sometimes  later, 
although  in  rare  instances  it  may  occur  in  the  height  of  the  disease. 
The  symptoms  of  diphtheritic  paralysis  will  not  here  be  discussed,  and 
we  shall  only  consider  the  ocular  phenomena. 

Ocular  Symptoms. — Loss  of  accommodation  with  impairment  of 
vision  for  near  objects,  due  to  paralysis  of  the  ciliary  muscles,  is  the 
most  frequent  form  of  paralysis,  next  to  that  of  the  palate.  It  generally 
follows  involvement  of  the  latter  or  appears  at  the  same  time  in  about 
the  third  or  fourth  Aveek.  It  is  the  most  common  of  the  ocular  palsies. 
The  ciliary  involvement  may  be  so  slight  that  its  presence  may  not  be 
detected,  especially  in  children.  The  patient  may  not  be  able  to  thread 
a  needle  or  read,  but  may  follow  the  finger. 

The  interference  with  vision  will  depend  upon  the  normal  refracting 
power  of  the  eyeball.  In  a  normal  eye  near  vision  will  be  interfered 
with ;  in  hypermetropia  this  will  be  even  more  marked,  while  in  a 
myopic  eye  there  may  be  very  little  or  no  appreciable  disturbance  of 
vision  for  near  objects,  for  accommodation  is  unnecessary. 

The  pupils,  as  a  rule,  are  of  normal  size  and  the  light  reflex  is  never 
lost,  although  it  may  be  sluggish.  Slight  contraction  of  the  pupils  in 
accommodation  may  be  present,  even  though  accommodation  is  entirely 
lost,  and  the  former  is  rarely  absent.  Loss  of  accommodation  generally 
comes  on  in  two  or  three  days  and  lasts  two  or  three  weeks  and 
then  disappears.  It  involves  both  eyes,  but  may  affect  one  before  the 
other.     Ophthalmoscopic  examination  is  always  negative. 

Paralysis  of  the  ocular  muscles  is  uncommon.  Statistics  upon  this 
point  vary,  some  authors  giving  as  much  as  twenty-five  per  cent,  of 
ocular  palsies  following  diphtheria.  The  external  and  internal  recti 
muscles  are  most  frequently  involved.  This  involvement  may  be  uni- 
lateral, but  is  commonly  bilateral  and  follows  the  loss  of  accommoda- 
tion. The  pals}^  may  be  so  slight  as  not  to  be  noticed,  or  may  be  fluc- 
tuating in  character.     The  paralysis  is  usually  of  short  duration. 

The  oculomotor  nerve,  either  wholly  or  in  part,  is  rarely  involved. 
Ptosis  is  mentioned  only  in  a  few  instances.  Uhthoff  records  in  a  boy 
of  ten   double  external  ophthalmoplegia,  without  involvement  of  the 


606  THE    EYE    AND    NEKVOUS    SYSTEM 

pupils,  with  a  mild  ptosis.  This  lasted  a  month.  Mendel  and  Gowers 
mention  two  cases  in  which  almost  all  of  the  muscles  supplied  by  the 
oculomotor  nerves  were  affected.  In  Gowers'  case  there  was  also  con- 
centric contraction  of  the  visual  fields  and,  according  to  him,  this  is  not 
so  infrequent  in  diphtheritic  palsy,  for  it  generally  escapes  attention, 
due  to  the  presence  of  the  cycloplegia. 


Fig.  7.— Case  of  diphtheria,  with  bilateral  external  rectus  palsy,  developing  two  weeks  after  the 
subsidence  of  diphtheritic  symptoms,  and  lasting  only  two  weeks.  (Case  from  the  nervous  dispensary 
of  the  Philadelphia  Polyclinic.) 

The  prognosis,  so  far  as  ocular  palsies  are  concerned,  is  good.  Only 
in  very  rare  instances  does  the  paralysis  persist.  Gowers  mentions  two 
cases  in  which  there  was  a  slow,  progressive  degeneration  of  certain 
cranial  nerves  occurring  some  time  after  the  ordinary  palsies  appear. 
In  one  case,  besides  weakness  of  the  lips,  tongue  and  pharynx,  there  was 
almost  complete  internal  arid  external  ophthalmoplegia  and  primary 
atrophy  of  the  optic  nerves.  In  his  second  case  sight  began  to  fail  six 
months  after  the  diphtheria  and  was  almost  lost  at  the  end  of  the  year. 
Two  and  a  half  years  after  the  primary  disease,  there  was  gray  atrophy 
of  the  optic  disks,  left  internal  ophthalmoplegia,  and  a  weakness  of 
some  of  the  movements  of  both  eyeballs,  especially  upwards.  These 
changes  were  stationary-. 

Harris  ^*  has  recently  recorded  three  cases  in  which  bulbar  symp- 

**  Brain,  vol.  xxvi. 


MULTIPLE  NEURITIS  607 

toms  followed  a  simple  sore  throat.  In  all  of  these  cases  the  progress 
of  the  paralysis  was  typical  of  post-diphtheritic  palsy.  Besides  the 
bulbar  symptoms,  the  patient  could  not  close  the  eyes  properly,  or  early 
in  the  morning  could  not  open  the  eyes.  The  symptoms  were  sug- 
gestive of  myasthenia  gravis,  but  Harris  believes  that  they  were  diph- 
theritic in  origin. 

Pathology. — The  pathology  of  diphtheritic  paralysis  is  still  very 
much  in  doubt,  because  of  the  varied  findings.  As  a  rule,  in  the 
majority  of  cases  the  changes  are  confined  to  a  degeneration  of  the 
peripheral  nerves,  especially  in  their  distal  parts.  Usually,  the  neuritis 
is  degenerative,  but  may  be  interstitial  in  character.  These  changes, 
however,  are  by  no  means  found  in  every  case.  Gaucher  records  a  case 
in  which  the  brain,  spinal  cord,  and  peripheral  nerves  were  entirely 
normal,  while  Hochhaus  notes  an  instance  in  which  the  alterations  were 
limited  entirely  to  the  muscles.  Changes  in  the  cells  of  the  anterior 
horns  of  the  spinal  cord,  hemorrhages  in  the  gray  substances  of  the 
spinal  cord,  increase  of  the  neuroglia  cells,  and  degeneration  of  the 
anterior  and  posterior  roots  have  also  been  found. 

Changes  in  the  bulbar  nuclei  with  a  degenerative  neuritis  of  the 
cranial  nerves  have  been  found.  Such  cases  have  been  recorded  by 
Mendel,  P.  Meyer,  and  others.  In  Meyer's  case  there  was  a  disap- 
pearance of  the  axis  cylinders,  changes  in  the  myelin  and  increase 
of  the  nuclei  of  the  endoneurium  of  the  third  nerve.  In  one  case  there 
were  minute  hemorrhages  in  the  walls  of  the  sixth  nerve. 

Thus,  from  the  pathology  we  cannot  come  to  a  definite  conclusion 
whether  the  cause  of  diphtheritic  palsy  is  central,  peripheral,  or  both. 
We  have,  however,  a  definite  disturbance  of  function  which  is  caused  by 
a  poison  circulating  in  the  blood.  It  is  only  rational  to  believe  that  in 
such  a  toxic  process  we  may  expect  alterations  in  all  parts  of  the  anat- 
omy. This  is  borne  out  by  the  pathological  findings.  It  is  well  known 
that  certain  poisons  have  a  peculiar  predilection  for  certain  parts  of 
the  nervous  system ;  in  this  case  it  is  shown  by  the  loss  of  accommoda- 
tion. Wilbrand  and  Saenger  believe  that  the  cycloplegia  is  due  to  a 
nuclear  or  central  lesion.  It  is  difficult  to  believe,  however,  that  the 
diphtheritic  poison  could  involve  only  that  portion  of  the  oculomotor 
nucleus  which  is  concerned  in  innervating  the  ciliary  muscles.  It  is 
more  probable  that  we  have  here  a  peculiar  peripheral  process  which 
involves  principally  the  nerve-fibres,  and  secondarily  the  muscles. 

Multiple  Neuritis  due  to  Infectious  Diseases. — Such  infectious  dis- 
eases as  erysipelas,  typhoid  fever,  pneumonia,  measles,  scarlet  fever, 
gonorrhoea,  influenza  and  rheumatism  may  cause  multiple  neuritis,  but 


008  THE    EYE    AND    NERVOUS    SYSTEM 

these  instances  are  necessarily  rare.  Eye  symptoms  are  exceedingly 
uncommon.  Fuchs,  however,  records  mild  grade  optic  neuritis  with 
paling  on  the  temporal  side  of  the  disc  in  a  typhoid  neuritis.  Micro- 
scopic examination  showed  a  retrobulbar  neuritis.  It  is  probable  that 
the  infection  did  not  directly  cause  the  optic  neuritis. 

Influenza,  or  la  grippe,  may  cause  various  nervous  symptoms,  among 
which  is  a  mononeuritis  of  the  different  cranial  nerves.  Such  are  the 
cases  recorded  by  Pfliiger  of  optic  neuritis,  by  Wilbrand  and  Saenger, 
Uhthoff  and  others,  of  paralysis  of  the  different  ocular  muscles.  Wil- 
brand and  Saenger  ^^  reviewed  the  literature  and  collected  the  following 
cases  of  ocular  paralysis  occurring  after  influenza.  In  thirteen  cases 
the  third  nerve  was  involved;  in  fourteen  the  abducens,  and  in  nine 
the  fourth.  In  twelve  cases  there  was  accommodation  paralysis,  and 
in  one  the  iris  was  paralyzed.  Ptosis  occurred  eight  times.  In  five  it  was 
bilateral,  in  three  imilateral.  It  is  probable  that  in  influenza  we  have 
a  toxic  process  which  acts  directly  upon  the  different  cranial  nerves, 
causing  a  temporary  paralysis.  Hemorrhagic  neuroretinitis  was  re- 
corded by  V.  Ley  den.    The  prognosis,  as  a  rule,  is  good. 

Malarial  Neuritis. — Tropical  malaria  may  cause  polyneuritis,  but 
this  disease  is  rare  in  this  country.  I  have  seen  a  number  of  such  cases 
among  soldiers  in  the  tropics.  Mononeuritis  of  the  cerebral  nerves  has 
been  recorded,  and  even  optic  neuritis  has  been  mentioned.  In  one 
instance  bilateral  abducens  palsy  was  noted  by  Strachan. 

Beriberi  or  Kakke  Neuritis. — This  disease  is  rare  in  this  country, 
although  it  is  prevalent  in  the  sea-coast  cities  of  our  Southern  States. 
It  is  quite  common  in  tropical  countries.  The  etiology  of  beriberi  is 
still  in  doubt,  but  it  is  probably  caused  by  soil  infection  and  not  by  a 
rice  diet,  although  this  may  be  a  contributory  cause.  There  are  three 
principal  forms — the  atrophic,  dropsical  and  mixed,  these  probably 
being  different  stages  of  the  disease.  Besides  the  ordinary  symptoms  of 
multiple  neuritis  there  is  great  debility.  Ataxia  is  almost  always 
present.  Dropsical  effusions  in  nearly  all  of  the  serous  cavities  may 
be  present. 

Eye  symptoms  occur,  but  they  have  been  insufficiently  studied. 
Kessler  examined  the  eye  grounds  in  sixty  cases  and  found  a  narrowing 
of  the  retinal  arteries,  w^hitish  paling  of  the  papillae  and,  in  a  large 
number  of  cases,  the  edges  of  the  disk  were  blurred.  It  is  probable, 
however,  that  these  changes  in  the  disk  are  due  to  the  general  nutri- 
tional disturbance  which  is  extreme  and  not  to  the  multiple  neuritis. 


'  Die  Xeurologie  des  Auges,  vol.  i.  248. 


MULTIPLE  NEUKITIS  609 

Disturbances  of  vision,  even  complete  blindness,  may  occur.  I  have 
seen  this  in  a  number  of  cases  among  the  Filipinos,  but  this  generally 
disappears  if  the  patient  improves.  The  cerebral  nerves  are  sometimes 
involved,  as  the  abducens,  facial  and  hypoglossus. 

Leprous  Neuritis. — Both  in  the  nodular  and  the  neuritic  types  of 
leprosy  we  have  involvement  of  the  eyeball.  In  the  nodular  form  the 
leproraata  may  invade  the  conjunctiva,  the  cornea,  the  anterior  chamber 
of  the  eyeball,  the  iris,  or  ciliary  body,  or  may  originate  in  these  parts. 
These  lepromata  ulcerate  and  finally  destroy  sight  in  both  eyes. 

In  the  neuritic  type,  owing  to  the  atrophy  of  the  facial  muscles,  it 
may  be  impossible  to  close  the  eyes,  and  there  may  be  eversion  of  the 
lower  eyelid.  The  upper  eyelids  may  droop.  At  first  there  is  a  con- 
junctivitis ^vith  lachrymation,  but  later  in  the  disease  there  will  be 
ulceration  of  the  cornea,  and  finally  complete  destruction  of  the  eyeball. 
It  is  only  late  in  the  disease  that  such  alterations  in  the  eyeball  may 
be  expected.    I  have  seen  a  large  number  of  such  cases. 

Tuberculous  Neuritis. — This  form  of  multiple  neuritis  is  rare 
and  eye  symptoms  are  recorded  only  in  very  few  cases.  Among  these 
Fuchs  records  optic  neuritis  and  nystagmus,  and  Rosenheim  pupillary 
differences.  The  vagus,  facial  and  phrenic  nerves  have  been  affected, 
but  not  the  ocular  muscles. 

Syphilitic  Multiple  Neuritis. — According  to  Oppenheim,  there  is 
no  doubt  of  the  existence  of  a  multiple  neuritis  due  to  the  syphilo- 
toxic  poison,  but  its  occurrence  is  doubted  by  many.  Cases  are  recorded 
in  which  all  of  the  cranial  nerves  with  the  exception  of  the  first  were 
involved  either  wholly  or  in  part.  Rudinger  reported  a  very  interesting 
case  of  polyneuritis  limited  to  the  cranial  nerves  which  he  considered 
as  syphilitic  in  origin.  In  his  case  a  woman,  after  exposure  to  cold, 
began  to  have  paresthesia  over  the  right  side  of  the  face  and  tenderness 
over  the  exit  points  of  the  right  fifth  nerve.  This  was  followed  by  a 
paralysis  with  atrophy  of  the  muscles  of  mastication  of  the  right  side, 
and  electrical  reactions  of  degeneration.  There  was  also  subluxation  of 
the  lower  jaw  and  disturbance  of  taste  in  the  anterior  two-thirds  of  the 
tongue,  and  a  right-sided  keratitis  neuroparalytica.  iNTeuralgia  of  the 
cornea  preceded  the  keratitis.  The  tear  secretion  in  the  right  eye  was 
not  disturbed,  neither  was  the  sense  of  smell.  The  facial  nerve  was 
paralyzed  on  the  opposite  side  and  the  acoustic  on  the  same  side.  There 
were  no  signs  of  a  basal  meningitis  or  tumor,  but  there  was  a  history  of 
syphilis. 

Keuritis  of  one  facial  nerva  has  also  been  reported  as  occurring  in 
syphilis.  It  is  difficult  to  distinguish,  however,  these  forms  from  a 
39 


610  THE    EYE    AND    XERVOUS    SYSTEM 

syphilitic  meningitis  and  the  occurrence  of  a  syphilitic  neuritis  alone 
may  be  well  doubted. 

Senile  Polyneuritis. — This  form  of  neuritis  is  characterized  by 
the  chronicity  of  its  onset,  the  absence  of  sensory  disturbances  and  of 
any  apparent  cause.     This  disease  is  rare.     Schlesinger  mentions  a  case 


Fig.  8.— Arteriosclerotic  neuritis,  with  paralysis  of  both  external  recti,  and  of  the  superior  and 
inferior  oblique  muscles  of  the  right  eye.    (Patient  of  Dr.  C.  A.  Oliver. ) 

in  which  there  was  a  transient  diplopia  and  sensory  disturbances  in  the 
distribution  of  one  fifth  nerve. 

Von  Frankl-Hochwart  records  an  interesting  case  of  this  kind.  A 
woman  of  sixty-seven,  four  years  after  a  stroke  of  paralysis,  noticed  a 
dimness  of  vision  in  the  right  eye  which  was  followed  in  half  an  hour 
by  diplopia.  A  few  hours  later  the  patient  could  not  open  the  right  eye, 
but  closure  was  normal.  The  eyeball  was  deviated  outwards,  the  pupil- 
lary' light  reaction  was  normal,  and  there  were  no  changes  in  the  fundus. 
The  patient  died.  The  microscopical  examination  showed  some  disin- 
tegration of  the  nerve-fibres  of  the  right  third  nerve,  with  great  increase 
of  round  cells  between  the  nerve-bundles.  The  vessels  in  the  connective 
sheath  of  the  nerve  were  engorged.  The  author  considered  it  a  case  of 
arteriosclerotic  multiple  neuritis  in  which,  however,  only  one  nerve 
was  involved. 

Franceschi  also  recorded  an  instance  in  which  there  was  paresis  of 


MULTIPLE  NEURITIS  611 

the  third,  sixth  and  seventh  nerves  of  one  side.  Microscopically  diffuse 
areas  of  softening  were  found  all  through  the  brain.  There  was  a  paren- 
chymatous neuritis  of  the  involved  cranial  nerves.  In  this  case  there 
was  a  sclerotic  condition  of  the  arteries,  this  being  the  primary  cause  of 
senile  neuritis. 

With  Dr.  Oliver  I  have  observed  an  interesting  case  of  arterio- 
sclerotic neuritis  in  a  man  of  sixty-nine.  The  patient  had  been  per- 
fectly well  up  to  four  years  ago  when  he  first  complained  of  diplopia. 
Since  then  he  has  complained  of  occasional  headache  and  of  a  numb 
feeling  in  his  extremities.  The  tendon  reflexes  were  not  disturbed. 
The  pupils  were  equal  and  reacted  slowly  to  light  and  in  accommodation. 
The  optic  nerves  were  normal.  There  was  paralysis  of  both  external 
recti  and  of  the  superior  and  inferior  oblique  muscles  of  the  right  eye. 
His  arteries  were  markedly  sclerotic.     There  was  no  history  of  syphilis. 

There  can  be  no  doubt  of  the  occasional  occurrence  of  ocular  palsies 
due  to  the  neuritis  caused  by  the  pressure  of  the  sclerosed  basal  arteries. 
This  is  perhaps  not  sufficiently  recognized  as  a  causal  factor.  (See 
chapter  by  Dr.  Hirsch.)  Pathologically,  this  condition  is  sometimes 
observed.  Recently  I  found  both  optic  nerves  much  degenerated,  and 
of  crescent  shape,  due  to  the  pressure  of  the  sclerosed  anterior  cerebral 
arteries. 

Carcinomatous  Multiple  Neuritis. — This  form  of  polyneuritis 
may  be  due  first  to  an  autointoxication,  second  to  a  carcinomatous  infil- 
tration of  the  sheaths  of  the  peripheral  nerves,  and,  third,  to  an  acute 
general  carcinomatous  infiltration  of  the  peripheral  nerve  endings. 

The  character  of  the  multiple  neuritis  due  to  carcinomatous  intox- 
ication does  not  differ  from  the  other  forms  of  polyneuritis  due  to  toxic 
causes.  In  a  case  of  Miura's  there  was  a  slight  optic  neuritis  with  an 
absolute  central  scotoma.  There  was  also  nystagmus  on  lateral  and 
upward  deviation  and  diplopia.  These  symptoms  appeared  several 
weeks  before  death  and  persisted.  The  microscopical  examination  by 
Uhthoff  failed  to  show  any  cause  for  the  optic  neuritis.  The  various 
peripheral  nerves,  as  well  as  the  orbital  parts  of  the  third  and  sixth 
nerves,  showed  degeneration.  The  spinal  cord  and  the  different  cranial 
nuclei  were  normal.  The  slight  swelling  of  the  papillae  was  attributed 
to  the  broadening  of  the  nerve-fibres  in  the  upper  layers. 

Dr.  Spiller  and  I  have  recorded  a  remarkable  case  of  cerebral  in- 
volvement due  to  a  cancer.  A  woman  who  had  a  recurrent  carcinoma  of 
the  breast  five  weeks  before  death  complained  of  failing  vision  in  both 
eyes,  more  marked  in  the  left.  -The  right  pupil  was  smaller  than  the 
left  and  responded  to  light  and  in  accommodation,  but  the  left  pupil  did 


612 


THE    EYE    AND    NERVOUS    SYSTEM 


not.  There  was  some  exophthalmos  of  the  right  eye.  The  external 
rectus  muscle  of  each  side  was  paralyzed  and  there  was,  besides,  a 
paralysis  of  the  left  superior  rectus  muscle,  and  ptosis  on  the  same  side. 
Unfortimately  no  ophthalmoscopic  examination  was  made.  An  exami- 
nation several  days  before  death  showed  an  almost  complete  internal  and 
external  ophthalmoplegia  of  the  left  eye,  and  a  distinct,  but  not  equal, 
weakness  of  the  left  seventh  nerve.  There  was  some  headache  and 
dizziness,  weakness  of  the  limbs  and  absent  knee-jerks. 


Fig.  9.— Case  of  carcinomatous  polyneuritis,    niustration  shows  marked  congestion  of  the  blood- 
vessels and  disease  of  the  oculomotor  nuclei. 

The  macroscopical  examination  did  not  show  anything.  Micro- 
scopical examination  showed  the  optic  nerves  to  be  normal.  Some  of 
the  axis  cylinders  of  both  third  nerves,  of  the  right  fourth,  of  the  right 
sixth,  and  of  both  seventh  nerves,  were  distinctly  swollen,  but  this  was 
especially  marked  in  the  right  sixth  nerve.  The  left  fourth  nerve  was 
normal.  The  left  sixth  nerve  was  not  obtained.  Some  of  the  nerve 
cells  of  both  oculomotor  nuclei  were  much  diseased,  and  the  nucleus  of 
each  abducens  nerve  was  intensely  diseased.  The  other  cranial  nuclei, 
as  well  as  the  cortical  cells,  were  normal.  The  blood-vessels  all  through 
the  brain  were  intensely  congested, — this  being  especially  marked  in 
the  cerebral  peduncles  and  the  posterior  part  of  the  pons. 

This  case  illustrates  well  the  diffuse  action  of  the  carcinoma  toxin. 
It  is  also  noticeable  that  the  pathological  findings  are  not  commensurate 
with  the  symptoms,  for  microscopical  examination  may  denote  a  de- 


MULTIPLE  NEURITIS  613 

generation  of  a  cranial  nerve,  the  clinical  symptoms  of  which  may  have 
been  entirely  absent  and  vice  versa. 

In  the  second  form  of  polyneuritis,  that  due  to  an  infiltration  of 
cancer-cells  in  the  nerve  sheaths,  we  may  have,  as  in  the  case  of  Saenger, 
an  infiltration  of  cancer-cells  in  the  pia  covering  the  nerve-fibres  at  their 
exit  from  the  base  of  the  brain.  Saenger  also  found  an  infiltration  of 
cancer-cells  in  the  pial  covering  of  the  cortex. 

Raymond  has  very  recently  called  attention  to  a  third  form  of 
cancerous  polyneuritis,  the  acute,  general,  miliary  form.  The  symp- 
tom complex  is  similar  to  the  other  forms  of  polyneuritis.  Macroscopi- 
cally  no  changes  can  be  found  centrally  or  in  the  peripheral  nerves,  but 
microscopic  examination  shows  multiple  lesions  in  the  finest  intramus- 
cular nerve  endings,  these  being  due  to  the  compression  by  the  innu- 
merable cancerous  emboli  in  the  muscles,  a  true  carcinomatous  neuro- 
myositis. No  instance  is  recorded,  however,  in  which  the  cranial  nerves 
were  diseased. 

Diabetic  Neuritis. — It  is  recognized  that  diabetes  may  cause  a  dis- 
ease of  the  peripheral  nerves.  It  is,  however,  improbable  that  the 
occasional  involvement  of  a  cranial  nerve  is  part  of  a  peripheral  neuritis, 
for  when  such  symptoms  occur,  we  probably  have  a  general  toxic  process 
due  to  the  diabetic  poison.     This  subject  is  discussed  in  another  chapter. 

Primary  Neurotic  Atrophy. — Because  of  our  lack  of  sufiicient 
pathological  evidence,  confusion  still  exists  as  to  the  cause  of  this  disease. 
It  occurs  in  families  and  is  distinguished  by  atrophy,  developing  in  the 
distal  portions  of  the  extremities.  The  reflexes  are  absent  and  there  are 
few  sensory  disturbances.  Pathologically,  in  the  few  cases  recorded, 
alterations  have  been  found  in  the  peripheral  nerves  and  muscles,  and 
the  nerve-cells  of  the  anterior  horns  of  the  spinal  cord. 

Optic  atrophy  accompanying  this  disease  has  been  recorded  only  by 
Ballet,  Rose,  Vizioli,  and  A.  Gordon.  In  a  case  under  my  observation 
in  the  Philadelphia  Hospital  the  patient  gave  a  distinct  history  of 
paralysis  of  the  left  external  rectus  and  the  left  superior  oblique  muscles. 
The  diplopia  persisted  for  some  time,  but  has  disappeared.  There  was 
no  history  of  syphilis. 

It  is  difiicult  to  explain  the  occurrence  of  the  optic  atrophy  and  the 
involvement  of  the  ocular  muscles.  It  is  probable,  however,  that  we 
have  here  conditions  foreign  to  the  disease  itself. 


CHAPTER    XVI. 
NEUROSES  AKD  PSYCHOSES. 

By  G.  E.  de  SCHWEINITZ,  A.  M.,  M.  D. 

OCULAR    MANIFESTATIONS    OF    HYSTERIA. 

The  investigation  of  the  disturbances  of  visual  sensation  to  which 
hysterics  are  subject  has  acquired  much  importance,  and  the  ocular 
stigmata  of  hysteria,  as  Parinaud  has  said,  constitute  a  symptomatology 
which  is  distinct  and  in  some  phases  unvarying.  The  following  some- 
what artificial  subdivisions  of  the  subject  are  convenient: 

1.  Complete  anaesthesia  of  the  visual  sense,  or  hysterical  amaurosis. 

2.  Incomplete  ana?sthesia  of  the  visual  sense,  or  hysterical  ambly- 
opia. 

3.  Defective  and  painful  visual  sensation,  or  hysterical  asthenopia. 

4.  Anomalies  of  accommodation,  or  hysterical  cyclospasm  and  cyclo- 
plegia. 

5.  Anomalies  of  the  iris  movements,  or  hysterical  pupil  phenomena. 

6.  Anomalies  of  the  eyelid  and  eye-muscle  movements,  or  hysterical 
contractures,  pseudopalsies,  and  palsies  of  the  palpebral  and  bulbar 
musculature. 

7.  Previously  unclassified  hysterical  ocular  phenomena. 

I.    COMPLETE    ANESTHESIA    OF    THE    VISUAL 
SENSE,  OR  HYSTERICAL  AMAUROSIS. 

Hysterical  Amaurosis.^ — This  term  is  applied  to  a  condition  char- 
acterized by  complete  abeyance  of  visual  sensation.  It  occurs  both  aa 
a  unilateral  and  a  bilateral  affection. 

(a)  Bilateral  Hysterical  Amaurosis. — The  literature  has  been  collected 
and  analyzed  by  Cruchet,^  Kron,^  and  Kernels.^  The  last  author  suc- 
ceeded in  gathering  34  cases,  and  since  the  publication  of  his  thesis  a 
number  of  additional  examples  have  been  reported, — for  instance,  by 

'  For  the  hi.storical  side  of  this  question  and  for  the  earlier  obsen'ations  the 
reader  is  referred  to  Gilles  de  la  Tourette's  TraitC*  Clinique  et  Therapeutique  de 
rHyst^rie,  1891,  p.  321;  also  to  P.  Pansier's  Les  Manifestations  Oculaires  de 
I'HystCrique,  Paris,  1892. 

*Archiv.  de  Neurologic,  2d  Series,  xii.,  1901,  p.  177. 

*  Neurologisches  Centralblatt,  xxi.  1902,  p.  584. 

*  Etude  sur  la  C6cit6  hysttrique  ou  Amaurose  Hyst6rique  totale  et  bilat6rale, 
Bordeaux,  1902. 

614 


NEUROSES  AND  PSYCHOSES  615 

Vetter,  McReynolds,  Dupuy-Dutemps,  Le  Roux,^  Hirsch,®  and  Ca- 
bannesJ 

In  most  instances  the  loss  of  sight  has  been  absolute,  or,  form-sense 
and  color-sense  being  lost,  the  sensation  of  bright  light  is  still  retained, 
— that  is,  the  patient  has  subjective  sensations  of  light.  The  onset  may 
be  sudden  or  gradual.  Although  the  conditions  necessary  to  create 
double  hysterical  blindness  usually  follow  an  hysterical  attack  and  the 
affection  has  been  described  by  Binswanger  as  an  interparoxysmal, 
morbid  state,  this  is  not  necessarily  the  case,  as  the  amaurosis  has  been 
encountered  without  any  connection  with  an  attack, — for  example,  after 
a  surgical  operation,  or,  again,  as  in  Leroux's  patient,  without  any 
preceding  hysterical  phenomena.  Usually  both  eyes  are  attacked  simul- 
taneously ;  sometimes  one  eye  may  be  affected  later  than  the  other, — for 
instance,  after  a  few  hours,  or  even  after  some  months.  More  women 
than  men  have  been  affected  (4  men  to  22  women,  according  to  Kron). 
The  age  in  women  has  varied  from  10  to  26  years;  in.  men  from  22 
to  37  years.  The  pupils  may  be  dilated  or  contracted.  Generally  they 
react  to  the  influence  of  light;  sometimes  only  a  feeble  contraction 
follows  the  light  stimulus ;  occasionally  the  pupils  are  dilated  and  insen- 
sitive to  light  (Kernels).  Accompanying  the  amaurosis  the  following 
symptoms  have  been  observed  in  some  of  the  cases:  Lagophthalmos, 
ptosis,  spasm  of  the  orbicularis,  anaesthesia  of  the  conjunctiva,  nystag- 
mus, conjugate  deviation,  and  convergent  strabismus. 

To  establish  the  diagnosis  of  hysterical  amaurosis  there  must  be 
excluded:  Ocular  disease  sufficiently  great  to  produce  blindness,  con- 
genital amblyopia  with  high  grades  of  refractive  error,  and  particularly 
certain  forms  of  so-called  retrobulbar  neuritis.  (For  further  discussion 
of  this,  see  page  638).  Evidently  complete  blindness,  in  the  absence 
of  ophthalmoscopic  signs  of  optic  nerve  disease,  associated  with  normal 
pupil  reaction  to  light  impulses,  would  strongly  suggest,  if  it  did  not 
prove,  the  presence  of  the  hysterical  taint,  but  because  in  rare  instances, 
temporarily  at  least,  the  pupils  under  these  conditions  fail  to  react  to 
light,  the  absence  of  the  pupil  reflex  does  not  necessarily  exclude 
hysteria. 

All  cases  on  record  have  recovered  vision  sooner  or  later,  and  there- 
fore the  prognosis  quoad  visum  is  good.  Indeed,  usually  when  the 
amaurosis  disappears,  it  does  so,  as  Pitres  remarks,  without  leaving 


'  Archives  d'Ophthalmologie,  December,  1903,  p.  801. 

•  Prag.  med.  Wochenschr.,  1904,  xii.  288. 

'  Gaz.  hebd.  d.  sc.  m6d  de  Bordeaux,  1905,  xxvi.  3. 


616  THE    EYE    AND    NERVOUS    SYSTEM 

a  trace.  The  duration  of  the  blindness,  however,  has  varied  consid- 
erably. Following  Kron's  suggestion,  the  cases  may  be  divided  into: 
(a)  The  transitory  forms,  lasting  from  a  few  hours  to  several  days;  (&) 
the  short-enduring  forms,  lasting  from  two  to  six  weeks;  and  (c)  the 
long-enduring  forms,  lasting  from  four  months  to  ten  years.  To  these 
should  be  added  the  intermittent  varieties  described  by  Koenigstein,  in 
which  the  blindness  appears  at  certain  deiinite  hours  of  each  day,  and 
lasts  for  a  time  and  then  disappears.  According  to  Kernels,  if  the 
blindness  has  appeared  suddenly,  it  will  disappear  suddenly ;  if  it  has 
arisen  during  a  crisis,  it  will  be  during  a  crisis  that  it  disappears ;  if 
it  is  without  a  crisis  that  the  blindness  has  appeared  suddenly,  it  will 
be  equally  sudden  and  without  a  crisis  that  the  patient  will  recover 
his  sight;  if  it  has  made  its  appearance  gradually,  its  disappearance 
will  be  a  gradual  one.  As  Kernels  himself  remarks,  these  rules  meet 
with  exceptions.  The  blindness  may  be  exceedingly  stubborn  and  last, 
as  has  already  been  noted,  for  years,  even  for  ten  years,  as  in  Harlan's 
well-known  case.  Before  the  entire  disappearance  of  the  blindness, 
there  may  be  a  stage  of  monocular  amaurosis,  as  in  McHeynolds's  case, 
with  recurrences,  and  occasionally  a  period  of  temporary  hemianopsia. 
(See  also  page  636.)  The  first  color  seen  by  the  patient  on  recovery 
from  the  blindness  is  red.     (Cruchet.) 

Certain  writers  are  sceptical  in  regard  to  the  long-enduring  forms  of 
hysterical  blindness, — that  is  to  say,  sceptical  in  regard  to  the  hysteri- 
cal nature  of  the  affection.  For  example,  B.  Sachs  ^  writes :  "  I  would 
prefer  not  to  accept  as  a  fact  the  occurrence  of  complete  hysterical 
amaurosis  of  months'  duration  until  more  convincing  cases  shall  have 
been  brought  forward."  Similar  doubts  were  entertained  by  Mauthner, 
and  are  implied  by  Schmidt-Rimpler.®  I  have  never  examined  a  patient 
with  bilateral  hysterical  amaurosis,  except  as  it  occurs  in  the  most  tem- 
porary way  during  an  attack,  and  can  reply  to  these  criticisms  only  by 
saying  that  some  of  the  cases  appear  to  me  to  be  above  suspicion, — for 
example,  Mendel's  case,  which  Sachs  himself  quotes. 

(6)  Unilateral  Hysterical  Amaurosis. — Unilateral  hysterical  amau- 
rosis, in  which  there  is  complete  abeyance  of  the  visual  sensation  of 
one  eye,  it  is  usually  stated,  is  a  more  commonly  observed  phenomenon 
than  the  bilateral  variety  of  the  affection.  According  to  Kron,  however, 
more  instances  of  the  double  than  of  the  single  amaurosis  are  on  record, 
— 26  compared  with  23.    Doubtless  the  explanation  of  this  is  that  all,  or 

*  Journal  of  Nervous  and  Mental  Diseases,  xxvi.  1899,  p.  347. 

•  Die  Erkrankunfj^en  des  Auges  im  Zusammenhang  mil  anderen  Krankheiten, 
Wien,  1898,  p.  256. 


KEUROSES  AND  PSYCHOSES  617 

nearly  all,  of  the  cases  of  double  amaurosis  have  been  recorded,  while 
only  a  comparatively  few  unilateral  instances  have  been  published. 
Either  eye  may  be  affected,  and,  according  to  Briquet,  one  eye  is  not 
more  frequently  selected  than  its  fellow,  but  in  Kron's  collection  the 
right  eye  was  blind  13  times  and  the  left  9  times.  Females  are  more 
liable  than  males,  in  the  proportion  of  2  to  1.  The  affection  is  not  con- 
fined to  adults,  and  pure  examples  have  been  observed  in  children, — for 
instance,  by  Veasey  in  a  child  of  11.  I  have  recorded  one  case  in  a 
negro  child  of  11  and  seen  another  in  a  girl  of  14.  The  difficulty  of 
diagnosis  in  very  young  children  is  apparent. 

Unilateral  hysterical  amaurosis  may  arise  suddenly  as  the  result 
of  emotional  excitement,  fright,  trifling  injury,  menstrual  pain,  etc., 
or  occur  as  the  termination  of  an  hysterical  attack,  and  return  with 
the  ending  of  the  second  attack,  constituting,  like  the  bilateral  affection, 
the  interparoxysmal  morbid  ocular  condition,  or,  according  to  Bins- 
wanger,  it  may  exist  for  a  long  time  without  any  connection  with 
attacks  or  emotional  disturbance.  The  period  of  blindness,  like  the 
bilateral  variety,  may  be  brief,  or  last  for  years.  One  of  Pitres's 
patients,  a  child  aged  10,  was  blind  four  years.  In  true  unilateral 
hysterical  amaurosis  the  opposite  eye  possesses  normal  vision  and  visual 
field,  or  even  a  visual  field  more  extended  than  that  which  is  ordinarily 
considered  normal  (Parinaud,  Morax).  More  often  the  opposite  eye 
exhibits  defective  visual  acuity,  contracted  visual  field,  etc.,  symptoms 
which  will  presently  be  elaborated  (page  621),  Under  these  circum- 
stances there  is  really  bilateral  disturbance  of  the  visual  sensations, 
— that  is,  blindnes,  or  amaurosis,  on  one  side,  and  reduced  vision,  or 
amblyopia,  on  the  other.  Associated  with  the  amaurosis  there  may 
be  other  disturbances  of  sensation — anaesthesia  of  the  skin,  perhaps 
confined  to  one  side, — that  is  hemianaisthesia,  anaesthesia  of  the 
mucosa  of  the  pharynx,  of  the  conjunctiva,  and  of  the  cornea.  The 
other  ocular  conditions  previously  noted  with  the  bilateral  form  of  the 
affection  may  also  exist.  While  the  amaurotic  eye  is  likely  to  be  on 
the  side  of  the  greater  cutaneous  anaesthesia,  this  rule  is  not  an  inva- 
riable one.     (See  Page  113.) 

The  Effect  of  Binocular  Fixation  on  Monocular  Amaurosis. — 
Schweigger,  Bernheim,  Pitres,  Janet,  and  other  observers  have  noticed 
and  described  the  disappearance  of  a  monocular  amaurosis  during 
binocular  fixation,  and  we  are  especially  indebted  to  Parinaud  ^^  for  a 


"The  Ocular  Manifestations  of  Hysteria,  System  of  Diseases  of  the  Eye,  edited 
by  Norris  and  Oliver,  vol.  iv.  1900,  p.  741;  and  Annales  d'Oculistique,  cxxiv.  1900, 
p.  17. 


618  THE    EYE    AND    NERVOUS    SYSTEM 

study  of  this  subject.  Briefly,  some  hysterics  completely  amaurotic  in 
one  eye  lose  this  blindness  during  binocular  fixation,  as  may  be  proved 
by  ordinary  prismatic,  stereoscopic,  or  other  tests.  Thus,  if  before  the 
admittedly  sound  eye  of  a  patient  completely  hysterically  amaurotic 
in  the  other  eye  a  prism  is  placed  through  which  a  test-object  is  re- 
garded, and  two  images  are  seen,  there  is  evidence  that  the  blind  eye 
has  been  associated  with  the  other  eye  in  the  act  of  binocular  fixation. 
For  example,  if  previous  examination  has  shown  that  the  right  eye  is 
amaurotic  in  monocular  fixation,  while  with  a  prism  base  down  before 
the  left  eye  the  patient  sees  two  images,  and  on  closing  the  right  eye 
the  lower  image  disappears,  it  is  evident  that  the  blind  eye  has  par- 
ticipated in  the  act  of  binocular  vision. 

According  to  Parinaud,  as  well  as  other  observers, — for  example, , 
Gilles  de  la  Tourette, — the  re-establishment  of  vision  in  the  amaurotic 
eye  at  the  moment  of  binocular  fixation  is  concerned  only  with  the 
central  perception.  The  peripheral  portion  of  the  visual  field  remains 
abolished,  both  in  monocular  and  binocular  vision.  Sometimes  experi- 
ments fail  to  elicit  the  reappearance  of  sight  in  the  amaurotic  eye  in 
the  act  of  binocular  vision,  either  because  the  defective  sight  is  too 
great,  or  perhaps  because  the  patient  never  possessed,  or  possessed  only 
in  imperfect  degree,  binocular  vision. 

Cruchet  ^  ^  describes  an  amaurotic  hysterical  patient  who  did  not 
have  binocular  vision,  and  who  with  all  the  tests  answered  as  if  she 
was  really  blind  in  one  eye.  According  to  him  the  amaurosis  should 
be  considered  in  two  stages, — the  first  one,  in  which  there  is  complete 
abolition  of  binocular  vision,  and  the  second  one,  more  frequently 
observed  than  the  other,  in  which  there  is  intermittent  and  generally 
perverted  conservation  of  binocular  vision.  He  states  that  in  cases 
of  unilateral  hysterical  blindness,  monocular  vision  exclusively  by  the 
good  eye  is  the  rule;  but  at  certain  moments  and  under  certain  condi- 
tions the  monocular  vision  of  the  sound  eye  becomes  monocular  vision 
of  the  amblyopic  eye, — that  is,  there  is  alternation.  Again,  monocular 
vision  may  seem  to  become  binocular,  but  only  apparently.  Really  it 
is  simultaneous  vision, — that  is,  fusion  of  the  images  does  not  take 
place.  Sometimes,  the  monocular  vision  becomes  binocular  vision,  with 
fusion  of  certain  images  and  not  of  others;  the  binocular  vision,  to 
use  the  expression  of  Antonelli,^^  is  dissociated.  Finally,  at  certain 
moments  and  under  certain  conditions  monocular  vision  really  becomes 


"  Archiv  de  Neurologic,  2d  Series,  xii.  1901,  p.  177. 
"Archives  d'Ophthalmologie,  xvii.  1897,  p.  218. 


NEUROSES  AND  PSYCHOSES  619 

binocular  with  complete  fusion  of  all  the  images,  a  condition  which 
Cruchet  regards  as  essentially  temporary  in  character. 

Concerning  the  Nature  of  Hysterical  Amaurosis. — It  may  be 
stated  in  the  light  of  our  knowledge  that  hysterical  blindness,  such 
as  has  been  described  in  the  preceding  paragraphs,  is  not  due  to 
any  failure  of  the  retina  to  appreciate  the  images  of  external  objects, 
but  to  a  functional  disturbance  of  cortical  centres.  A  patient  with 
monocular  amaurosis  of  hysterical  nature  sees  with  the  blind  eye,  as 
may  be  determined  by  the  ordinary  tests  which  are  used  to  detect  pre- 
tended blindness. 

Again,  Binet  and  Janet  have  shown  that  if  before  the  eye  of  an 
amaurotic  hysteric  some  object  is  held  and  the  patient  is  afterwards 
hypnotized,  he  will  be  able  to  tell  that  which  was  previously  exhibited 
and  which  he  regarded  with  an  apparently  blind  eye.  Evidently  the 
patient  really  saw  the  object.  As  Prince  puts  it,  "  the  hysterically 
blind  see,  but  their  visual  sensations  are  not  in  connection  with  their 
dominating  waking  consciousness.  There  would  seem  to  be  a  shutting 
off  from  the  field  of  consciousness  of  certain  perceptions  which  form  a 
group  of  segmented  sensations,  more  or  less  by  themselves,  and  which 
by  artificial  means, — for  example,  the  hypnotic  state, — would  as  memo- 
ries be  brought  into  relation  with  the  remainder  of  the  conscious  field." 

Because  hysterics  apparently  blind  in  one  eye  may  by  tests  ordi- 
narily applied  to  malingerers  be  proven  to  see  with  this  eye,  they  have 
been  characterized  as  cheats ;  but,  as  Pitres  points  out,  the  uniformity 
of  answers  given  by  the  subjects  of  unilateral  hysterical  amaurosis 
cannot  be  reconciled  with  the  theory  of  deception  in  the  gross  sense  of 
this  term. 

According  to  George  C.  Harlan,  patients  with  hysterical  blindness 
may  be  divided  into  three  classes:  those  who  deliberately  simulate  the 
blindness,  those  who  see  unconsciously  but  are  not  capable  of  conscious 
vision,  and  those  who  are  really  transiently  absolutely  blind. 

Binswanger  ^^  quotes  Binet's  conclusion  that  the  disturbances  which 
lie  at  the  bottom  of  hysterical  anaesthesia,  no  matter  what  its  nature  may 
be,  must  have  their  seat  in  the  cerebral  cortex,  because  in  order  that  a 
peripheral  stimulus  may  produce  not  only  movements  but  also  concepts, 
it  must  have  reached  the  centres  in  which  concepts  are  formed, — that  is, 
the  highest  composite  centres  of  the  cerebrum.  Bernheim  believes  that 
an  amblyopic  hysterical  subject  with  the  aid  of  imagination  uncon- 


"  Specielle   Pathologie   und   Therapie.     H.   Nothnagel,   xii.   Band,   i.   Halfte,  ii. 
Abth.,  Die  Hysteric,  von  Prof.  Dr.  Otto  Binswanger,  Wien,  1904,  p.  979. 


620  THE    EYE    AND    NERVOUS    SYSTEM 

sciously  neutralizes  the  visual  image  which  has  been  perceived  in  a 
normal  manner,  although,  as  has  been  shown,  and  as  Pitres  points  out, 
the  patient  does  not  neutralize  binocular  but  apparently  only  monocular 
perceptions.  Janet,  however,  from  his  experiments  leans  to  the  theory 
of  Bernheim,  and  thinks  that  it  demonstrates  the  psychic  or  cortical 
nature  of  this  sensory  disturbance,  while  Charcot  rejects  Bemheim's 
explanation  that  hysterical  blindness  is  exclusively  a  psychic  phe- 
nomenon,— that  is,  an  illusion  of  the  mind  and  not  a  true  sensory 
anaesthesia. 

Keferring  again  to  the  interesting  fact  that  in  a  certain  number  of 
instances,  at  least,  monocular  hysterical  amaurosis  disappears  in  binocu- 
lar fixation,  we  find  that  Parinaud  ^*  dismisses  Bernheim's  theory  that 
such  phenomena  represent  a  blindness  of  the  imagination  due  to  the 
destruction  of  the  image  by  a  psychic  agent,  with  the  curt  expression 
that  "  he  does  not  understand  such  language,"  and  thinks  that  the 
interpretation  of  hysterical  visual  symptoms  depends  upon  the  belief 
in  the  existence  of  certain  relations  between  the  retina  and  the  visual 
centres  in  monocular  and  binocular  sight,  as  well  as  definite  and  distinct 
relations  in  central  and  in  peripheral  vision.  He  goes  on  to  say,  "  If 
we  admit  the  crossed  relation  between  each  eye  and  the  opposite  hemi- 
sphere in  monocular  vision,  the  left  eye  will  become  amaurotic  when  the 
right  side  of  the  brain  is  affected ;  but  if  in  binocular  vision  both  eyes 
are  connected  with  a  single  hemisphere,  it  seems  clear  that  if  that 
hemisphere  be  the  left  and  healthier  one,  the  left  and  amaurotic  eye 
will  be  able  to  see." 

Charcot,  quoted  by  Binswanger,  has  demonstrated  that  hemianaes- 
thetic  patients  with  monocular  color  blindness  are  able  to  identify  the 
color  accurately  during  binocular  vision,  even  with  the  achromatoptic 
eye,  and  therefore  favors  the  view  that  there  are  functional  disturbances 
in  the  intracerebral  centres  for  monocular  vision,  those  for  binocular 
vision  being  uninjured.  Similar  observations  have  been  made  by  Pari- 
naud.   I  shall  return  to  this  subject  in  a  later  paragraph. 

While  there  is  no  proof  of  it,  so  far  as  I  am  aware,  it  is  conceivable 
that  some  toxin  is  liberated  during  an  hysterical  attack  and  the  retina 
poisoned,  or  perhaps  the  cortical  centres,  by  this  toxin,  producing, 
according  to  its  virulence,  a  temporary,  an  enduring,  or  a  long-continued 
blindness,  exactly  as  this  occurs  in  uraemia,  in  diabetes,  and  in  certain 
intestinal  fermentations.  It  would  be  confessedly  difficult  to  reconcile 
this  theory  with  imilateral  amblyopia,  unless  one  were  willing  to  admit 


"  System  of  Diseases  of  the  Eye,  edited  by  Norris  and  Oliver,  iv.  p.  743. 


NEUROSES  AND  PSYCHOSES  621 

a  selective  action  of  tlie  toxin  upon  one  retina,  exactly  as  in  very  rare 
toxic  blindness, — for  example,  quinine  blindness, — is  unilateral. 

II.  INCOMPLETE    AN-ffiSTHESIA    OF    THE    VISUAL    SENSE,  OR 
HYSTERICAL    AMBLYOPIA. 

This  term  is  applied  to  a  number  of  different  visual  disturbances,  of 
which  the  most  important  are:  reduction  of  visual  acuity,  contraction 
of  the  visual  field,  disturbance  of  the  color  and  light  sense,  and  anoma- 
lies of  accommodation.  In  contradistinction  to  the  affections  which 
have  been  described  in  the  preceding  paragraphs  and  which  represent 
a  total  loss  of  visual  sense,  either  unilateral  or  bilateral,  these  are  incom- 
plete disturbances  of  sight.  They  represent,  in  the  language  of  Bins- 
wanger,  the  incomplete  anaesthesias ;  "  they  are  polymorphous  and 
composite  in  character,  both  as  regards  their  extent  and  as  regards  the 
special  variety  of  the  visual  disturbance." 

(1)  Reduction  of  Visual  Acuity. — By  this  is  meant  a  failure  of  the 
affected  eye,  or  eyes,  to  obtain  full  visual  acuity  in  the  absence  of 
refractive  defects  and  pathological  changes,  as  not  infrequently  occurs  in 
hysteric  patients. 

(2)  Disturbances  of  the  Visual  Field  in  Hysteria. — (a)  Visual 
Field  for  White. — Many  of  the  records  of  the  visual  field  examinations 
in  hysterics  and  neurasthenics  are  untrustworthy  because  no  definite 
information  is  given  in  regard  to  the  light,  the  size  of  the  test-object, 
character  of  the  refraction,  the  length  of  time  employed  in  taking  the 
visual  field,  the  distance  of  the  examined  eye  from  the  fixation  point, 
etc.  Unfortunately,  there  has  been  no  uniform  method,  and  hence  the 
comparison  between  the  results  of  different  observers  is  often  difficult 
and  probably  accounts  for  the  discrepancy  in  statement.  Thus,  one 
observer,  in  taking  the  visual  field,  moves  the  test-object  from  the 
periphery  towards  the  centre  and  waits  until  the  patient  distinctly  sees 
the  object,  and  then  records  its  position  on  the  perimeter  arc;  while 
another  evidently  has  recorded  the  points  at  which  the  patient  first  be- 
comes conscious  of  some  object, — for  example,  a  square  of  white  or 
colored  paper  on  the  perimeter  arc.  There  is  almost  a  uniform  absence 
of  statement  in  regard  to  the  patient's  refraction  under  these  condi- 
tions, and  very  rarely  have  control  examinations  been  made  with  the 
carnpimeter,  or,  except  in  recent  times,  with  the  visual  field  taken  at 
varying  fixation  distances.  (For  a  further  consideration  of  this  sub- 
ject, see  Chapter  IV.) 

Just  as  a  certain  number  of  hysterics  have  normal  acuity  of  vision, 
they  may  also  have  a  normal  visual  field  for  white.     Pansier  ^^  states 

"Les  Manifestations  oculaires  de  I'Hystgrie,  Paris,   1892,  p.  17. 


622  THE    EYE    AXD    KERVOUS    SYSTEM 

that  altlioiigh  the  visual  field  for  white  may  be  normal,  he  has  found  a 
true  enlargement  of  the  color  field,  with  or  without  inversion  of  the 
color  circles,  in  all  non-amblyopic  hysterics  which  he  has  examined. 
On  the  other  hand,  Frankl-Hochwart  and  A.  Topolanski  ^°  maintain 
that  in  hysteria  with  normal  sensation  there  is  usually  no  contraction 
of  the  visual  field,  and  von  Reuss  ^^  has  also  noted  that  hysteria  may 
exist  without  any  changes  in  the  fields  of  vision.  John  K.  Mitchell  and 
myself  ^  *  have  observed  a  completely  normal  character  of  the  visual 
field  in  a  certain  number  of  hysterics,  or  simply  contraction  of  the  color 
fields  with  unaffected  form  fields. 

The  essential  characteristic  of  the  visual  field  in  hysteria  is  concen- 
tric contraction,  if  this  is  measured  with  a  white  test-object  on  a 
black  background.  It  represents,  to  use  the  language  of  Parinaud,  "  an 
insensibility  of  a  portion  of  the  field  to  white  light,  and  this  insensi- 
bility develops  from  the  circumference  to  the  centre  in  such  a  way  that  a 
graphic  drawing  of  the  contraction  would  be  represented  by  an  almost 
circular  line."  Xaturally,  as  Parinaud  further  maintains,  concentric 
must  not  be  taken  in  a  too  literal  sense  of  the  term,  because,  although 
quite  noticeable  on  the  temporal  side  where  the  field  is  normally  largest, 
it  may  be  most  restricted  on  the  nasal  side  or  above.  Furthermore,  this 
visual  field  should  manifest  its  contraction  at  the  very  beginning  of  the 
measurement,  as  von  Reuss  ^®  insists,  and  not  be  called  into  existence  by 
fatigue  during  examination. 

The  amount  of  the  reduction  of  the  volume  of  the  visual  field  varies 
from  slight  contraction,  most  noticeable  on  the  temporal  side,  to  such  an 
extreme  restriction  tbat  the  most  peripheral  circle  is  just  beyond  the 
fixing  point.  Binswanger  is  willing  to  apply  the  term  "  pronounced" 
only  to  those  cases  in  which  the  normal  visual  field  is  reduced  fully 
one-half.  Should  the  patient  have  possessed  originally  a  minimal  or 
small  physiological  field,  slight  reduction,  if  bilateral,  would  be  difficult 
to  determine  unless  one  should  find,  as  Parinaud  suggests,  a  concomitant 
hysterical  disturbance  of  accommodation.  (See  page  28.)  Bins- 
wanger has  never  observed  "  the  gravest  cases,  in  which  the  visual  field 
is  reduced  to  the  fixation  point,"  and  he  therefore  differs  from  Parinaud, 
Gilles  de  la  Tourette,  and  Janet.  I  have  on  a  number  of  occasions  seen 
extreme  reduction  in  the  size  of  the  visual  field  (see  Fig.  1),  and  can 
in  this  respect  confirm  entirely  the  observations  of  the  French  observers 
concerning  which  Binswanger  is  sceptical. 


"  Deutschmann's  Beitriige  zur  Augenheilkunde,  ii.  1895,  p.  46. 
"  Das  Gesichtsfeld  bei  functionellen  Nervenleiden,  Wien,  1892. 
"Journal  of  Nervous  and  Mental  Diseases,  January,  1894. 
"  Loc.   cit.,   p.   52. 


LEUKOSES  a:n'd  psychoses 


623 


The  hysterical  field  contraction  is  of  long  endurance,  lasting  for 
days,  months,  and  even  years,  and  the  variations  in  size  which  it  under- 
goes, except  those  which  are  later  recorded,  are  comparatively  unimpor- 
tant until  recovery  occurs,  when  the  visual  field  again  may  assume  its 
normal  size. 

The  behavior  of  the  contracted  visual  field,  when  it  is  measured  with 
the  point  of  fixation  placed  at  varying  distances  from  the  observed  eye, 


Bight 

Fig.  1. — Grand  hysteria,  scattered  anaesthesia.     Woman,  aged  26;  patient  of  Dr.  J.  K.  Mitchell.     Con- 
tracted tubular  fields  ;  partial  reversal  right ;  all  color  sense  absent  in  left  field. 


is  of  importance.  Schmidt-Rimpler  ^^  maintains  that  each  perimetri- 
cally  recorded  concentric  contraction  of  the  visual  field  should  also  be 
investigated  with  a  campimeter  stationed  at  varying  distances  from 
the  patient,  and  WoUenberg,  quoted  by  Binswanger,  found  under  these 
conditions  that  in  the  majority  of  cases,  although  not  always,  as  the 
distance  between  the  patient  and  the  blackboard  is  increased,  the  size 
of  the  visual  field  is  enlarged.  On  the  other  hand,  Richard  Greeff  ^^ 
states  that  the  contracted  visual  field  maintains  the  same  size,  no  matter 
at  what  distance  the  point  of  fixation  of  the  examined  eye  is  placed, — in 
other  words,  that  the  field  has  a  tubular  character;  the  hysteric  has,  to 
use  the  term  which  Greeff  has  given,  a  "  rohrenformiges  Gesichtsfeld." 
The  examination  is  made  as  follows:  The  visual  field  is  measured,  as 
usual,  with  a  perimeter,  and  if  it  is  found  contracted  in  decided  degree, 


^  Deutsch.  med.  Wochenschr.,  1892,  Nr.  24. 

"  Berlin  klin.  Wochenschr.,  May  26,  1902,  p.  496. 


624 


THE    EYE    AND    NERVOUS    SYSTEM 


the  point  of  fixation  for  the  examined  eye  is  removed  one  metre  distant 
and  the  measurements  repeated.  Instead  of  an  increase  in  the  width  of 
the  field,  it  may  remain  just  as  narrow  as  it  was  prior  to  the  lengthening 
of  the  fixation  distance,  and  the  same  is  true  if  this  is  still  further 
increased, — for  example,  to  two,  three,  or  five  metres.  That  this  observa- 
tion is  entirely  correct  I  am  able  to  confirm  as  the  result  of  many 
examinations.     (See  Fig.  2.) 

According  to  Greeff,  the  cause  of  the  tubular  field  is  connected  with 
the  underlying  nature  of  the  disease;  that  is,  in  hysterical  patients  a 
definite  conception  frequently  dominates  the  sensorium,  and  this  applies 
also  to  the  visual  field.    Von  Hoesslin  is  unwilling  to  admit  that  symp- 


Left 


Right 


Fig.  2.— Hysterical  girl.   Patient  of  Dr.  J.  William  White.    Typical  tubular  contracted  field ;  no 
change  in  size  at  30  Cm.,  1,  2,  3,  and  4  metres. 


toms  which  are  physically  impossible  are  genuine  signs  of  disease,  and 
he  regards  the  person  who  exhibits  the  tubular  field  phenomenon  as  a 
malingerer,  and  if  hysteria  is  present,  then  the  subject  is  a  hysterical 
malingerer.  Arnlieim  and  other  observers  are  not  in  accord  with  von 
Hoesslin  in  this  contention,  and  I  agree  with  them  and  with  Wilbrand, 
that  "  we  have  no  right  to  cast  suspicion  of  malingering  on  a  patient 
unless  we  are  able  to  convict  him  or  her  of  malingering." 

Although  the  contraction  of  the  visual  field  may  be  extreme,  visual 
acuity  may  be  normal ;  on  the  other  hand  it  may  be  reduced.  Inasmuch 
as  in  many  cases  the  peripheral  vision  is  completely  abolished,  producing 
typical  tubular  vision,  if  central  sight  is  affected  then  amblyopia  may 
give  place  to  amaurosis  and  the  condition  of  complete  hysterical  blind- 


NEUROSES  AND  PSYCHOSES  625 

ness.  It  should  be  remembered,  however,  that  such  amaurosis  need  not 
have — indeed,  does  not  usually  have — concentric  visual  field  contraction 
as  its  prodrome,  but  appears  suddenly. 

The  contraction  may  be  unilateral  (  8  times  among  79  cases  examined 
by  Parinaud)  or  bilateral,  generally  the  latter.  Usually,  as  Fere,  Pari- 
naud,  Landolt,  Wilbrand,  Saenger,  and  others  have  shown,  it  is  asso- 
ciated with  disturbances  of  cutaneous  anaesthesia,  being,  when  these  are 
present,  in  the  language  of  von  Frankl-Hochwart  and  Topolanski,  a 
fairly  constant  phenomenon.  In  bilateral  cases  the  restriction  of  the  vis- 
ual field  is  usually  greater  on  the  hemianaesthetic  side,  or  upon  that  side 
which  exhibits  the  greater  surface  or  degree  of  anaesthesia ;  rarely  the 
opposite  eye  is  the  more  affected.  Unilateral  contraction  of  the  visual 
field  commonly  appears  on  the  hemianaesthetic  side.  It  should  be  re- 
membered, however,  that  the  presence  of  cutaneous  anaesthesia  does  not 
necessarily  imply  the  presence  of  restricted  visual  fields,  nor  is  it  neces- 
sary that  they  should  appear,  as  J.  K.  Mitchell  and  myself  have  shown, 
when  the  general  symptoms  of  hysteria  are  of  the  highest  grade. 

According  to  Gilles  de  la  Tourette,^^  concentric  narrowing  of  the 
visual  field  is  the  most  frequent  ocular  stigma  in  hysterical  subjects, 
and  Dana  says  that  it  is  the  commonest  of  all  forms  of  anaesthesia. 
Pitres  remarks  that  it  is  one  of  the  most  frequent  sensory  disturbances 
of  the  great  neurosis  and  one  of  the  best  stigmata  of  hysteria.  Bins- 
wanger,^^  on  the  other  hand,  writes,  "  The  scepticism  with  which  we 
regard  the  statements  of  Charcot's  school  (Galezowski,  Parinaud,  Lan- 
dolt, Fere,  and  others)  increases  in  direct  proportion  to  the  growth  of 
our  experience  in  this  subject ;  for  these  investigators  attribute  too  much 
importance  to  the  concentric  contraction  of  the  visual  field  in  hysteria." 
The  value  of  this  symptom  seems  to  me  exceedingly  great,  although  J. 
K.  Mitchell  and  myself  were  unable  to  find  it  as  frequently  among  the 
hysterics  whom  we  examined  as  would  appear  to  be  the  case  in  the 
French  clinics. 

It  has  already  been  pointed  out  that  the  contracted  visual  field  is 
one  that  appears  at  once  and  not  as  the  result  of  repeated  examination, 
and  that  in  a  certain  sense  it  is  a.  permanent  stigma,  or  perhaps,  more 
accurately,  an  enduring  one,  differing  in  this  respect  from  the  one 
which  will  later  be  described  in  connection  with  neurasthenic  cases.  It 
is,  however,  subject  to  certain  variations,  which  must  now  be  mentioned. 
They  are  these : 


"  Traite  Clinique  et  Therapeutique  de  I'Hystgrie,  1891. 
»  Loc.  cit.,  p.  209. 
40 


626  THE    EYE    AND    NERVOUS    SYSTEM 

In  unilateral  visual  field  contraction  the  narrowed  field  may  be  made 
to  disappear  by  irritating  the  skin,  and  to  develop  in  the  healthy  eye; 
in  short,  a  transfer  takes  place.  It  may  be  transferred  to  the  other 
eye  by  suggestion.  Also,  as  Parinaud  has  shown,  if  the  unaffected 
eye  be  opened  and  the  visual  field  previously  contracted  remeasured,  it 
may  increase  its  extent.  Referring  to  this  fact,  Janet  ^^  suggests  that 
under  these  circumstances  the  sensations  of  the  sound  eye  bring  into 
personal  perception  by  a  pre-established  association  those  of  the  neg- 
lected eye.  The  extent  of  the  field  is  furthermore  modified  by  spherical 
glasses,  by  tinted  glasses,  and  by  the  action  of  mydriatics,  as  has  been 
well  shown  by  Parinaud,  Forster,  and  as  I  have  frequently  demon- 
strated. A  visual  field  contracted  for  a  white  test-object  may  reach 
greater  or  even  normal  extent  if  investigated  with  a  bright  electric  light.* 

The  visual  field  may  l>e  diminished  in  size  by  mental  condition,  by 
physical  depression,  preoccupation,  emotions,  fixed  ideas;  in  general 
terms,  as  Janet  puts  it,  in  a  great  number  of  hysterics  the  eifort  of 
visual  attention  contracts  in  a  very  notable  way  their  visual  fields. 
The  variations  in  perimetric  examinations  are  not  necessarily  depen- 
dent upon  simple  retinal  fatigue,  but,  as  Schmidt-Rimpler,  Schultze, 
and  others  have  shown,  may  depend  upon  a  diminution  of  the  cortical, 
psychical  functions,  and  be  due  to  damage  of  the  power  of  concentration. 

Binswanger  emphasizes  the  importance  of  the  psychic  condition  at 
the  time  of  examination  and  its  effect  on  the  visual  field,  and  insists 
that  patients  should  be  methodically  investigated  at  different  times 
with  due  regard,  not  only  to  their  power  of  concentration,  but  also  to 
their  emotional  state,  and  to  the  presence  of  definite  disturbances  of  the 
intellect,  and,  finally,  to  the  relation  of  their  condition  to  antecedent 
hysterical  attacks. 

It  has  been  the  custom  of  some  authors  to  classify  with  hysterical 
fields  "  the  shifting  type,^''  originally  described  by  Forster  in  cases  of 
anaesthesia  of  the  retina,  in  which  the  visual  fields  differ  according  as 
the  examination  is  conducted  from  the  temporal  to  the  nasal,  or  from 
the  nasal  to  the  temporal  side,  the  contraction  being  pronounced  on  the 
nasal  side  in  the  former  and  on  the  temporal  side  in  the  latter ;  the 
exhaustion  type  of  Wilbrand,  in  which  the  test-object  is  moved  from 
the  temporal  to  the  nasal  and  from  the  nasal  to  the  temporal  side 
several  times  in  succession,  across  the  entire  width  of  the  perimeter, 
indeed,  as  often  as  the  field  continues  to  diminish ;   unstable  concentric 

**  The  Mental  State  of  Hysterics.  Translated  by  C.  R.  Corson,  G.  P.  Putnam  4 
Sons,  1901,  p.  69. 


NEUROSES  AND  PSYCHOSES 


627 


limitation, — that  is,  a  field  which  is  constantly  changing  during  the 
examination ;  the  exJiaustion-spiral  type,  in  which  the  tracing  of  the 
visual  field  appears  coiled  like  a  watch-spring  in  consequence  of  its 
limits  becoming  concentrically  smaller;  and  finally,  the  recuperation- 
extension  type,  in  which  the  restricted  field  may  extend  during  rest,  or 
by  a  strong  effort  of  will.  These  five  types,  classified  and  elaborated  by 
von  Reuss,^^  do  not,  however,  belong  strictly  to  pure  hysterical  cases, 
and  will  be  considered  in  more  detail  in  the  section  devoted  to  neuras- 
thenia. 

One  form  of  field,  however,  von  Reuss  feels  should  be  classified 
with  those  which  are  more  strictly  hysterical  in  type,  namely,  the 
so-called  "  oscillating  -field,''^  described  by  Wilbrand  and  O.  Koenig,  in 


Fig.  3.— Left  field.    Oscillating  field.    (After  Wilbrand. ) 

which  the  object  disappears  and  reappears  several  times  in  the  same 
meridian,  and  in  which  a  similar  oscillation  applies  to  color.  This 
phenomenon  has  been  ascribed  to  rapid  change  in  the  sensitiveness  of 
the  retina.     (Fig.  3.) 

As  Wilbrand  has  shown,  if  the  areas  in  the  visual  field  in  which  the 
test-object  disappears  are  studied  in  association  with  one  another,  they 
assume  a  zonular  form, — in  other  words,  an  annular  scotoma  results. 
Such  scotomas  as  hysterical  manifestations  have  been  described  by  a 


^''  Loc.  cit.     See  also  a  good  review  of  von  Reuss's  book  by  J.  J.  Evans,  Ophthal- 
mic RevieAv,  xxi.  1902,  p.  339. 


628 


THE    EYE    AND    NERVOUS    SYSTEM 


number  of  observers, — for  example,  Ziehen  and  Casey  A.  Wood.  (Fig. 
4.)  The  latter  author,  although  unwilling  to  rely  upon  this  sign  alone 
in  the  diagnosis  of  hysteria,  has  observed  it  when  other  ocular  indiea- 


Bight 
Fig.  4.— Annular  scotoma  in  hysteria;  moderate  contraction  of  form  field;  no  reveraal  of  color  lines. 

tions  of  this  neurosis  were  wanting.  Von  Reuss  maintains  that  there 
may  be  a  double-ring  scotoma,  a  fragmentary  ring  scotoma,  or  that  the 
oscillating  field  may  be  associated  with  central  scotoma,  and,  as  in  a 
case  of  Wilbrand's,   with   scintillating  scotoma,   or  temporary   hemi- 


i05°      90"       Ta 
Fig.  5.— Central  scotoma  in  hysteria. 

anopsia.  A  certain  degree  of  stability  characterizes  these  scotomas 
which  distinguishes  them  from  exhaustion  cases.  According  to  Wil- 
brand,  a  central  exhaustion  scotoma  is  rare.  I  have  observed  an  abso- 
lute central  scotoma  in  a  hysterical  woman  which  lasted  for  weeks 
(Fig.  5). 


NEUROSES  AND  PSYCHOSES 


629 


(6)  Visual  Field  for  Colors. — Under  this  heading  must  be  considered 
concentric  contraction  of  the  color  fields,  dyschromatopsia  and  acroma- 
topsia,  and  certain  rare  conditions  to  which  the  term  hyperchromatopsia 
may  be  given. 

Jnst  as  there  may  be  a  narrowing  of  the  visual  field  due  to  an  insen- 
sibility to  white  light,  and  Avhich,  in  the  language  of  Parinaud,  consti- 
tutes the  fundamental  condition  of  this  type  of  hysterical  amblyopia, 
so  also  there  may  be  an  insensibility  to  colors,  which  begins  at  the 
periphery  of  the  visual  field  and  occasions  a  contraction  of  the  color 
field.  The  color  circles  may  be  found  within  the  narrowed  circle  for 
white  exactly  in  their  physiological  order, — that  is,  from  without  in- 


FiG.  6.— Right  hysterical  hemiaiuesthesia ;  patient  of  Dr.  Wharton  Sinkler.  Partial  left  anaesthesia 
reversal  of  color  lines*  outer  circle,  white,  next  inner,  red,  then  blue  and  green ;  form  field  not  con- 
tracted, R.  E. ;  moderate  contraction,  L.  E. 

ward,  blue,  yellow,  red,  green,  and  violet, — the  entire  restricted  field  is 
really  a  much  reduced  normal  visual  field. 

Sometimes,  as  Nonne,  J.  K.  Mitchell,  myself,  and  others  have  shown, 
the  restriction  is  confined  to  the  color  fields,  while  the  white  field 
remains  unaffected  (Fig.  6,  right).  As  long  as  the  color  fields,  even 
though  restricted,  retain  their  normal  physiological  relation  one  to  the 
other,  such  restriction,  as  Parinaud  insists,  does  not  necessarily  indicate 
dyschromatopsia,  but  might  depend  on  individual  differences  found  in 
the  normal  state,  or  on  the  light  and  the  objects  employed,  and  upon  the 
narrowing  due  to  insensitiveness  to  white  light. 

Under  normal  circumstances  the  color  fields  when  tested  with  satu- 
rated colors  in  good  illumination  occur  from  the  periphery  to  the  centre 
in  the  following  order:  blue,  yellow,  red,  green,  violet.  Now  in  hys- 
terical patients  a  somewhat  characteristic  variation  is  that  the  red  field  is 


630 


THE    EYE    AND    NERVOUS    SYSTEM 


the  last  to  be  affected,  with  the  result  that  its  extent  may  equal  that  of 
blue,  so  that  these  color  lines  coincide  or  cross  one  another,  or  it  may 
exceed  it  and  become  the  most  peripheral  of  the  color  circles. 

This  is  the  so-called  inversion  of  the  color  field,  and  represents  much 
more  certainly,  again  to  quote  Parinaud,  the  presence  of  dyschromatop- 
sia  than  a  mere  limitation  of  the  extent  of  the  visual  field  for  colors. 
More  than  this,  the  red  field  may  exceed  in  extent  not  only  the  blue  field 
as  described,  but  that  for  white,  and  become  not  merely  the  peripheral 
color  circle,  but  the  peripheral  circle  for  the  entire  field. 

Binswanger  states  that  in  his  clinic  he  has  observed  cases  in  which 
the  visual  field  for  red  was  extraordinarily  diminished,  so  that  it  was 
exceeded  by  the  visual  field  for  green ;  similar  observations  have  been 
recorded  by  v.  Hochwart  and  Topolanski  and  Wilder,  and  Pansier  has 


Left 


Fig.  7.— Hystero-catalepsy ;  woman  aged  20;  patient  of  Dr.  S.  Weir  Mitchell.  Typical  concentric 
contraction  and  reversal  of  blue  and  red  lines.  In  some  meridians  the  red  field  exceeds  the  white 
field. 

seen  the  green  circle  become  the  most  peripheral  one.  Such  exceptions, 
however,  do  not  destroy  the  main  fact,  that  hysterical  subjects  have  a 
predilection  for  red,  constituting  a  characteristic  of  this  type  of  dys- 
chromatopsia  (Fig.  7). 

Coming  to  the  question  of  partial  abolition  of  the  color-sense,  as 
Landolt  has  pointed  out,  this  may  be  confined  to  the  most  central  por- 
tions of  the  visual  field, — that  is,  the  hysterical  patients  lose  first  the 
power  of  distinguishing  violet  and  green,  while  they  retain  their  power 
of  perceiving  the  other  colors.  Gradually,  it  may  be,  the  ability  to  recog- 
nize red  and  orange  disappears,  while  the  power  to  perceive  yellow  and 
blue  persists ;  or  the  perception  of  red,  instead  of  that  of  blue,  is  the 
last  to  disappear.     Therefore  Pitres  has  arranged  two  series  giving  the 


NEUROSES  AND  PSYCHOSES  631 

order  in  which  the  power  of  recognizing  the  different  colors  is  lost  in 
hysterical  dyschromatopsia :  (1)  violet,  green,  blue,  yellow,  red,  and 
(2)  violet,  green,  red,  yellow,  blue.  In  contrast  to  this,  it  may  be 
pointed  out,  as  Pitres  has  done,  that  in  the  dyschromatopsia  of  certain  or- 
ganic conditions, — for  example,  chronic  retrobulbar  neuritis,  or  so-called 
toxic  amblyopia,  and  tabetic  atrophy  of  the  optic  nerve, — the  perception 
of  red  is  first  lost.  While  most  observers  agree  in  stating  that  violet  or 
gi'een  is  the  first  color  to  be  lost  in  hysterical  cases,  Striimpell  believes 
that  a  definite  order  of  disappearance,  such  as  has  been  quoted,  is 
entirely  too  schematic.  J.  K.  Mitchell  and  myself  noted  long  ago  that 
we  were  not  so  apt  to  find  in  American  patients  the  dyschromatopsia 
which  has  been  described,  although  we  did  find  the  inversion  of  the 
color  field  to  which  full  reference  has  been  made,  and  we  also  noted 
that  the  perception  of  green  was  more  often  disturbed  than  that  of  the 
other  colors  (violet  was  not  used  in  our  tests),  and  in  this  respect  the 
observations  coincide  with  those  of  Landolt. 

Parinaud  ^'^  has  noted  the  following  anomalous  conditions :  A  color 
scotoma  having  all  the  characteristics  of  that  seen  in  toxic  amblyopia ; 
a  central  defect  with  added  peripheral  contraction  resulting  in  an  annu- 
lar color  field  in  the  shape  of  an  intermediary  zone  between  the  central 
circle  and  the  peripheral  zone ;  not  only  an  enlargement  of  the  red  field 
exceeding  that  for  white,  but  a  similar  enlargement  for  other  colors 
(which  Pansier  maintains  is  characteristic,  page  9),  which  were  either 
perceived  as  such  or  as  gray  with  an  intensity  greater  than  that  for 
white.  I  have  recently  studied  with  A.  G.  Thomson  an  hysterical 
boy  with  a  central  color  scotoma  in  his  field  exactly  like  that  which  is 
caused  by  the  abuse  of  tobacco  and  alcohol. 

Hysterical  dyschromatopsia,  or  disturbance  of  color-sense  mani- 
fested by  inversion  of  color  lines  or  loss  of  certain  color  perceptions, — 
for  example,  violet  and  green, — may  give  rise  to  or  be  replaced  by  hys- 
terical achromatopsia,  a  word  for  which  we  are  indebted  to  Galezowski. 
In  this  condition,  first  described  by  Briquet,  the  patient  is  unable  to 
distinguish  any  color,  although  he  recognizes  the  outlines  and  shapes  of 
objects.  Everything  appears,  as  has  been  well  said,  like  a  sketch  in 
India  ink.  To  the  total  achromatoptic  the  colors  have  a  certain  luminous 
intensity,  to  which  brief  reference  has  been  made  by  Obarrio.^'^  Inves- 
tigation of  an  entirely  color-blind  intelligent  female  patient  demon- 


-^  System  of  Diseases  of  the  Eye,  edited  by  Norris  and  Olivei-,  vol.  iv.  1900,  p. 
736. 

"  Bull,  de  la  Soc.  Fr.  d'Ophth.,  vol.  xvii.  p.  453. 


632  THE    EYE    AND    NERVOUS    SYSTEM 

strated  that  each  color  matched  a  certain  gray.  The  patient  quickly 
recognized  the  equivalent  gray  tones,  indeed,  more  quickly  than  a  normal 
individual. 

Dyschromatopsia  and  achromatopsia  are  apt  to  be  associated  with 
cutaneous  and  mucous-membrane  anaesthesia.  Fere  maintains  that  those 
hysterical  patients  who  have  neither  narrowing  of  the  visual  field  nor 
achromatopsia  have  normal  sensibility  of  the  conjunctiva ;  those  who 
have  achromatopsia  and  concentric  contraction  of  the  visual  field  have 
complete  conjunctival  anaesthesia,  and  those  who  have  achromatopsia 
and  practically  abolished  visual  fields  have  not  only  conjunctival  but 
also  corneal  anaesthesia.  To  these  observations  Gilles  de  la  Tourette 
ascribes  great  importance,  while  Binswanger  ^®  is  unable  to  confirm  the 
existence  of  such  regular  interrelation  between  the  sensory  disturbances 
of  the  cornea  and  of  the  coverings  of  the  eye  on  the  one  hand  and  defects 
of  optical  sensation  on  the  other. 

(c)  Light-Sense  and  Color-Sense. — In  addition  to  the  concentric  con- 
traction of  the  visual  field  in  hysterical  patients,  diminution  of  the 
light-  and  color-sense  may  be  present,  as  has  been  pointed  out  by  Pari- 
naud,  Wolffberg,  and  particularly  by  von  Frankl-Hochwart  and  Topo- 
lanski.  According  to  the  last-named  authors,  sometimes,  although  the 
visual  field  and  light-sense  may  be  normal,  whether  it  be  accompanied  by 
disturbances  of  cutaneous  sensibility  or  not,  the  power  of  perception  of 
one  or  other  color — for  example,  blue — may  be  defective,  and  Parinaud 
states  that  wherever  he  has  found  dyschromatopsia  it  has  been  accom- 
panied by  modification  of  the  normal  white  perception.  He  has  also 
observed  patients  who,  examined  with  the  photometer,  could  recognize 
white  light  only  when  it  was  extremely  intense,  but  recognized  nothing 
when  this  intensity  was  lowered,  and  yet  who  had  a  luminous  impression 
when  the  previously  unperceived  white  surface  was  viewed  through  a 
saturated  red  glass. 

The  investigation  of  the  color-sense  and  light-sense  in  hysterical 
patients  is  important,  and  has  not  received  as  much  attention  as  it 
deserves,  because  if,  as  Binswanger  points  out,  there  should  be  an  agree- 
ment, as  there  was  in  von  Frankl-Hochwart  and  Topolanski's  examina- 
tions, between  the  restriction  of  the  visual  fields  and  the  reduction  of 
the  light-sense  and  color-sense,  strong  evidence  would  be  present  that 
there  was  no  simulation,  which  from  the  perimeter  findings  alone 
might  be  suspected.  Binswanger  states  that  Wilbrand  was  unable  with 
the  aid  of  Forster's  photometer  to  determine  anomalies  in  the  light- 

»Loc.  cit..  p.  212. 


NEUROSES  AND  PSYCHOSES  633 

sense  in  hysterical  amblyopia.     The  investigations  of  von  Frankl-Hoch- 
wart  and  Topolanski  were  made  with  the  Chibret  instrument. 

(d)  Hyperchromatopsia  has  been  referred  to  and  the  true  enlarge- 
ment of  the  color  fields,  to  which  Pansier  ascribes  special  importance,  de- 
scribed. J.  K.  Mitchell  and  myself  have  observed  an  exaggerated 
appreciation  of  colors  with  corresponding  enlargement  of  the  color 
fields  in  hysterical  hyperaesthesia.  This  observation  has  been  confirmed 
by  Frankl-Hochwart  and  Topolanski  (Fig.  8).  Finally,  Binswanger 
has  found  in  cases  of  cutaneous  hyperaesthesia  and  hyperalgia  marked 
enlargement  of  the  visual  fields  for  red  and  green,  again  confirming  the 
observation  which  Mitchell  and  I  made  many  years  ago.  In  hysterical 
enlargement  of  the  color  fietds,  the  blue  may  exceed  all  others  and  pass 


Fig.  8.— Hysterical  hyperaesthesia.     Patient  of  Dr.  S.  Weir  Mitchell.     Enlargement  of  color  fields 
and  partial  reversal  of  red  and  blue  lines. 

beyond  the  white,  or  there  may  be  inversion  of  the  wddened  color  circles 
with  red  as  the  most  peripheral  one ;  rarely  this  position  is  taken  by 
green. 

Concerning  the  Nature  of  Hysterical  Visual  Field  Contraction 
and  Color-Sense  Disturbance. — Although  hysterics  may  have  extreme 
contraction  of  the  visual  field,  they  are  not  inconvenienced  by  this 
apparent  limitation  of  their  peripheral  vision,  as  is  a  patient,  for 
example,  with  pigmentary  retinitis.  In  a  word,  their  orientation  is 
usually  perfect.  In  explanation  of  this  it  has  been  suggested  that  the 
peripheral  visual  impressions,  although  unperceived,  bring  about  in 
the  muscular  apparatus,  to  use  the  language  of  Parinaud,  those  in- 
stinctive and  unconscious  movements  by  means  of  which  we  find  our  way 
through  the  external  world.  Groenouw  states  that  hysterical  patients 
with  greatly  contracted  fields  find  no  difficulty  in  getting  about  because 


634  THE    EYE    AND    NERVOUS    SYSTEM 

the  periphery  of  the  retina  still  retains  its  power  of  perceiving  large  and 
bright  objects.  Wilbrand  explains  the  phenomenon  by  assuming  that  the 
irritability  of  the  retinal  periphery  is  not  lost,  and  that  the  insufficiency 
of  the  retina  is  not  so  great  when  the  patient  is  moving  about  as  it  is 
during  steady  fixation.  To  a  certain  extent  what  follows  has  been  dis- 
cussed under  hysterical  amaurosis.      (Page  G19.)     n, 

Von  Graefe  believed  that  hysterical  amblyopia  represented  an  affec- 
tion of  the  outer  layers  of  the  retina  and  could,  in  general  terms,  be 
described  as  a  disturbance  of  the  peripheral  optical  apparatus.  Wil- 
brand has  stated  that  in  hysteria  certain  alterations  exist  in  the  visual 
substance  of  the  rods  and  cones,  and  that  where  there  was  peripheral 
contraction  of  the  visual  field  with  retained  central  vision,  these  altera- 
tions were  limited  to  the  peripheral  retina,  but  that  where  the  ambly- 
opia was  greater,  or  true  amaurosis  appeared,  they  extended  and  the 
entire  retina  became  involved.  This  theory  seemed  to  be  supported  by 
perimetric  examinations  and  the  various  displacement-  and  exhaustion- 
types  of  the  field  of  vision  which  result  from  them,  the  indication  being 
that  the  retina  is  non-resistant  to  fatigue.  (See  also,  page  683.)  Bins- 
wanger  maintains,  although  he  gives  full  place  to  the  theories  which 
have  been  briefly  outlined,  that  the  hysterical  contracted  field  is  the 
result  of  a  cortico-sensory  fatigue,  and  of  a  disturbance  of  the  faculty  of 
concentration. 

Pierre  Janet  ^®  regards  achromatopsia  as  consistent  with  the  belief 
that  the  retina  is  sound,  but  that  the  visual  defect  is  further  back  in  the 
perceptive  centre.  Charcot  also  maintained  that  achromatopsia,  and 
therefore  dyschromatopsia,  are  cerebral  phenomena.  It  has  been  proved 
that  a  hysterical  achromatoptic  viewang  red  on  a  white  ground  will  see 
only  a  gray  image,  but  after  awhile  will  obtain  a  consecutive  green 
image.  In  other  words,  when  the  green  vibration  reaches  the  per- 
ceptive centre,  it  is  not  correctly  recognized,  but  nevertheless  acts 
because  added  to  the  red  vibration  it  gives  the  perception  of  white  and 
produces  the  consecutive  green  image.^*^  Parinaud,  investigating  the 
color  contrasts  upon  hysterical  patients,  found  that  the  sensation  pro- 
duced by  a  color  that  is  not  perceived  can  be  developed  provided  the 
complementary  color  inducing  it  is  normally  seen.  The  converse  of 
this,  however,  is  not  true. 

Simulation  by  the  Hysterical  Visual  Field  and  Hysterical  Ambly- 
opia of  Organic  Defects. —  (a)  Hysterical  Hemianopsia. — There  is  much 


™The  Mental  States  of  Hysterics,  p.  25. 
^  Pierre  Janet,  loc.  cit. 


KEUROSES  AXD  PSYCHOSES  .         635 

difference  of  opinion  whether  hemianopsia  occurs  as  an  ocular  stigma  of 
hysteria,  and  we  have  statements  varying  from  those  of  Parinaud,  who 
declares  that  there  is  one  variety  of  visual  insensibility  that  hysteria 
appears  to  be  incapable  of  producing,  and  that  is  hemianopsia,  to  the 
assertion  of  Rosenthal,^ ^  that  in  all  cases  of  hysterical  amblyopia  which 
he  has  investigated  temporal  hemianopsia  was  present. 

Gowers  ^^  says  that  of  a  large  number  of  cases  of  hemianopsia  which 
have  come  under  his  observation  one  only  may  have  been  of  hysterical 
origin.  He  further  states,^^  "  In  the  hemianaesthesia  of  hysteria  we 
have  evidence  of  arrested  action  of  all  the  higher  special  sense-centres  in 
one  hemisphere.  In  this  association  we  never  meet  with  the  hemianopsia 
that  results  from  disease  of  the  lower  centre  in  the  posterior  part  of  the 
hemisphere.  Instead,  we  have  crossed  amblyopia,  as  it  is  termed; 
peripheral  vision  is  lost  in  both  eyes,  central  vision  persists  in  a  small 
area  in  the  opposite  eye,  and  in  a  larger  area  in  the  eye  of  the  same 
side.    Xo  trace  is  found  of  any  relation  to  the  half  fields." 

Hemianopsia  has  been  reported  by  Westphal,  Galezowski,  Svynos, 
Bonnefoy,  M.  W.  Zimmerman,  Henry  Lloyd,  Dejerine,  Koenig,  and  a 
number  of  other  observers.^^ 

Dejerine  and  Vialet,^^  referring  especially  to  neurasthenia  and  trau- 
matic neurosis,  describe  a  special  form  of  hemianopsia  which  they  think 
constitutes  an  ocular  symptom  of  the  same  order  as  restriction  of  the 
visual  field,  and  which  is  distinguished  from  the  hemianopsia  of  organic 
origin  by  the  variability  of  the  limits  of  the  preserved  half  field,  and 
which  may  last  for  years.  It  is  difficult  to  resist  the  belief  that  this 
represents  rather  an  unusual  and  irregular  contraction  of  the  visual 
field,  with  a  much  greater  restriction  on  one  side  than  on  the  other, 
than  a  true  hemianopsia,  and,  as  far  as  I  am  able  to  judge  from  an 
examination  of  the  literature,  hemianopsia  as  an  enduring  ocular  symp- 
tom of  hysteria,  in  the  same  sense  as  concentric  contraction  of  the  visual 
field,  does  not  exist. 

On  the  other  hand,  it  must  be  admitted  that  as  a  temporary  phe- 
nomenon it  has  been  observed  a  good  many  times  by  those  who  are 

^Wiener  med.  Presse,  Nr.  23,  1879,  pi  736.  In  Gilles  de  la  Tourette's  book 
Rosenthal  is  quoted  as  having  written  a  letter  to  Charcot  in  which  he  appears  to 
retract  the  statement  just  quoted,  and  says  that  hysterical  patients  exhibit  only 
amblyopia  and  concentric  contraction  of  the  visual  field,  and  not  hemianopsia. 

*"  Diseases  of  the  Nervous  System,  American  edition,  1888,  p.  584. 

*•  Clinical  Lectures,  Second  Series,  Subjective  Sensations,  1904,  p.  11. 

^Consult  Wilfred  Harris,  Brain,  1897,  p.  308;  also  Koenig,  Rec.  d'Oph., 
3  Series.  22.  1900.  p.  397. 

'^  Annales  d'Oculistique,  1894,  tome  cxii. 


636 


THE    EYE    AND    NERVOUS    SYSTEM 


competent  to  judge,  and  has  appeared  either  as  a  bitemporal  defect,  a 
homonymous  lateral  defect,  or  even,  it  would  seem,  as  a  binasal  obscura- 
tion. Furthermore,  it  would  seem  from  the  observations  of  Pierre 
Janet,'"'®  who  reports  a  case  of  transitory  hemianopsia  which  occurred 
in  a  woman  the  subject  of  grand  hysteria,  and  which  he  believed  was  not 
brought  about  by  suggestion,  that  it  is  possible  there  may  always  be  a 
period  of  hemianopsia  when  the  ordinary  hysterical  amaurosis  is  grad- 
ually recovering.  Indeed,  such  an  observation  was  made  by  Galezowski 
and  Daguenet  long  before  the  publication  of  Janet's  cases.  These 
observers  found  that  hysterical  amblyopia  gradually  recovered,  and  that 
between  the  period  of  complete  recovery  and  the  period  of  complete 
amaurosis  there  was  a  stage  of  hemianopsia,  and  sometimes,  instead  of 


Left 


Right 


Fig.  9.— Hysterical  girl.    Patient  of  Drs.  J.  K.  Mitchell.and  G.  E.  de  Schweinitz.    Islands  of  anaesthesia. 
Contracted  fields  and  inversion  of  color  lines  on  left  side  where  the  red  exceeds  the  white  field. 

this,  a  transitory  central  scotoma.  A  similar  phenomenon  was  present 
in  a  patient  whose  clinical  history  has  been  reported  by  Hirsch  in 
Germany,  the  defect  exliibiting  itself  in  a  bitemporal  form. 

Whether  it  is  true  that  in  place  of  a  homonymous  lateral  or  bitem- 
poral hemianopsia,  as  Wilfred  Harris  has  described  in  his  Cambridge 
thesis,  binasal  hemianopsia  may  occur  I  do  not  know.  John  K.  Mitchell 
and  myself  published  one  case  in  which,  somewhat  incautiously,  we 
made  use  of  the  expression  that  the  visual  fields  had  the  characters  of 
binasal  hemianopsia,  and  since  then  this  case  has  appeared  in  literature 
as  an  example  of  hysterical  binasal  hemianopsia.  It  should  be  re- 
garded, I  feel  sure,  not  in  this  light,  but  as  an  irregular  form  of  con- 


"  La  Presse  Medicale,  1899,  p.  243. 


NEUEOSES  AND  PSYCHOSES  637 

traction  with  a  greater  contraction  of  the  field  on  the  nasal  side. 
(Fig.  9.) 

According  to  Gilles  de  la  Tourette,  ophthalmic  migraine  may  be 
accompanied  by  hemianopsia  when  it  is  of  hysterical  origin  but  it  is 
always  temporary  and  may  be  regarded  as  an  exaggeration  of  the  re- 
striction of  the  visual  field.  On  the  other  hand,  Babinski  ^^  doubts 
whether  a  true  relationship  between  hysteria  and  an  ophthalmic  mi- 
graine has  been  established,  and  believes  that  the  migraine  when  asso- 
ciated with  the  hysteria  has  an  independent  origin,  making  the  point 
that  between  hysteria  and  hemianopsia  there  is  no  marked  relationship, 
while,  as  he  expresses  it,  the  relationship  between  hemianopsia  and 
ophthalmic  migraine  is  like  that  of  parent  to  child. 

(&)  Insular  Sclerosis  and  Hysteria. — Inasmuch  as  the  symptoms  of 
hysteria  may  precede  the  onset  of  insular  sclerosis  or  occur  during  its 
course,  this  disease  may  be  mistaken  for  hysteria.  Normally,  in  the 
visual  field  between  the  outer  limit  of  white  perception  and  the  outer 
limit  of  color  perception,  colors  appear  light  gray,  white,  or  black,  and 
according  to  Head  ^*  the  same  phenomenon  obtains  in  the  field  of  the 
hysterical  type  of  disseminated  sclerosis,  but  is  not  present  in  the  true 
hysteric  field.  Referring  to  the  blurred  vision,  or  amblyopia,  of  insular 
sclerosis,  Buzzard  points  out  that  the  optic  nerve  is  diseased,  or  even 
atrophied,  in  this  disease  and  normal  in  hysteria.  Other  ocular  signs 
suggestive  of  sclerosis  are  temporary  paresis  of  an  ocular  muscle,  and 
especially  nystagmus.     (See  also,  Chapter  XI,  p.  469.) 

Kampherstein  ^^  states  that  regular  functional  concentric  contrac- 
tion of  the  visual  field  as  it  occurs  in  hysterical  amblyopia  has  not 
occurred  in  his  or  in  Liibber's  investigation  of  cases  of  multiple  sclerosis. 
According  to  Ulithoff,"*"  although  the  occasional  association  of  visual 
field  contraction  with  insular  sclerosis  may  be  explained  by  a  coexisting 
hysteria,  it  must  be  remembered  that  in  the  former  this  condition  may 
obtain,  even  in  the  absence  of  ophthalmoscopic  change,  and  be  none  the 
less  occasioned  by  lesion  of  the  opticus.  Therefore  the  diagnosis  of  pure 
functional  disturbance  of  the  visual  field  under  these  conditions  is 
difficult. 

(c)  Syringomyelia  and  Hysteria. — Concentric  contraction  of  the  visual 
field  for  all  colors  has  been  reported  in  syringomyelia  by  Dejerine  and 

"Archiv  de  Neurologic,  xx.  1890,  p.  305. 

"Quoted  by  Buzzard,  British  Medical  Journal,  Oct.  7,  1893. 
"Archiv  f.  Augenheilk.,  Bd.  xlix.  Erstes  Heft,  1903,  p.  41. 

*•  Graefe-Saemische,  Handbuch  der  gesammten  Augenheilkunde,  Lief.  68-71, 
1904,  p.  352. 


CSS  THE    EYE    AND    NERVOUS    SYSTEM 

Tuilant,  but  Gilles  de  la  Tourette  agrees  with  Brianceau,  whom  he 
quotes,  that  in  the  majority  of  cases  of  this  affection  the  visual  field  is 
normal,  and  that  when  it  is  contracted  it  is  necessary  to  search  elsewhere 
than  in  the  disease  itself  for  this  phenomenon.  Hysteria,  which  may 
accompany  the  affection,  is  accordingly  given  in  this  respect  the  etio- 
logical role,  in  the  absence  of  ophthalmoscopic  lesions.     (Chapter  XV.) 

(d)  Exophthalmic  Goitre  and  Hysteria. — Concentric  contraction  of  the 
visual  field  has  been  observed  in  exophthalmic  goitre,  but  in  the  expe- 
rience of  Gilles  de  la  Tourette,  under  these  circumstances  hysteria  has 
always  been  associated  with  the  malady.  Personally,  I  have  examined  a 
great  many  cases  of  exophthalmic  goitre,  and  in  the  absence  of  ocular 
lesions  have  not  found  notable  alterations  in  the  visual  field,  certainly 
nothing  similar  to  those  which  have  been  described  as  more  or  less 
characteristic  of  hysteria.     (See  Chapter  XIX.) 

(e)  Epilepsy  and  Hysteria. — It  is  well  known  that  there  may  be  con- 
traction of  the  visual  field  after  epileptic  attacks,  and  not  only  a  mere 
contraction  of  the  visual  field  but  an  amblyopia,  and  therefore  in  these 
respects  an  analog}-  between  this  disease  and  hysteria  is  recognized. 
Furthermore,  repeated  epileptic  attacks  might  lead  to  an  apparent  per- 
manent contraction  of  the  visual  field  or,  to  speak  more  accurately,  a 
temporary  amblyopia  which  would  rapidly  subside  after  one  attack 
might  persist  longer  should  the  attacks  be  many  times  repeated,  Pari- 
naud,'**  after  quoting  the  observations  of  Oppenheim  and  Thomsen,  that 
contraction  of  the  visual  field  is  frequently  seen  in  epilepsy,  records 
Hitler's  examinations  in  Charcot's  clinic,  where  he  found  in  87  cases  of 
epilei)sy  a  permanent  restriction  of  the  visual  field  in  only  three.  There- 
fore, as  there  is  an  occasional  more  than  temporary  amblyopia  of  this 
character,  it  is  necessary  that  the  differential  diagnosis  of  epilepsy  and 
hysteria  from  the  visual  stand-point  should  not  rest  alone  upon  the  phe- 
nomena connected  with  the  field  of  vision.  The  other  ocular  symptoms 
of  hysteria  should  serve  to  establish  the  diagnosis.  Finally,  there  is  no 
reason  why  there  should  not  be  an  association  of  epilepsy  with  hysteria, 
an  association  which  has  not  infrequently  been  observed.  (Chap.  XVII.) 

(/")  Retrobulbar  Neuritis  and  Hysteria. — It  will  at  once  be  apparent 
that  sudden  amaurosis  of  hysteria  and  the  rapid  loss  of  vision  which 
accompanies  retrobulbar  neuritis  on  superficial  examination  bear  a 
marked  similarity,  and  there  is  no  doubt  that  the  one  has  occasionally 
been  mistaken  for  the  other.  Naturally,  the  reaction  of  the  pupil  gives 
important  information,  and  if  there  is  blindness  with  preservation  of 


"  System  of  Diseases  of  the  Eye,  edited  by  Norris  and  Oliver,  vol.  iv.  p.  763. 


NEUROSES  AND  PSYCHOSES  639 

pupillary  contraction  to  light  stimulus,  retrobulbar  neuritis  may  be 
excluded.  Sometimes,  however,  as  we  shall  later  see,  the  pupil  does  not 
react  to  light,  temporarily  at  least,  in  hysterical  cases,  and  therefore  on 
this  point  alone  a  differential  diagnosis  could  not  be  established,  and  the 
other  symptoms  which  have  been  described  in  connection  with  hysterical 
amaurosis  would  almost  certainly  lead  to  a  proper  diagnosis, — for  ex- 
ample, the  disappearance  of  monocular  blindness  in  binocular  fixation, 
aiuusthesia  of  the  conjunctiva,  and  contraction  of  the  visual  field  of 
the  opposite  eye  and  inversion  of  the  color  lines.  Symptoms  of  hys- 
teria not  associated  with  the  eye  would  add  most  important  diagnostic 
information. 

The  method  in  Avhich  the  blindness  occurs  in  retrobulbar  neuritis 
is  usually  characteristic,  inasmuch  as  the  obscuration  of  vision  starts 
in  the  centre  of  the  field  and  progresses  in  from  one  to  eight  days  to 
complete  or  nearly  complete  blindness;  furthermore,  there  is  the 
comparativeh'  rapid  replacement  of  negative  ophthalmoscopic  signs 
with  alterations  in  the  fundus  oculi,  and  pain  is  evident  on  movement  of 
the  eyeball  and  when  it  is  pressed  backward  into  the  orbit. 

Some  authors — for  example,  Bregmann  ^^ — suggest  that  just  as  retro- 
bulbar neuritis  may  depend  upon  a  primary  disturbance  of  metabolism, 
so  also  may  hysteria,  the  unknown  toxin  working  on  the  one  hand  on 
the  optic  nerve  and  on  the  other  on  the  brain,  and  think  it  also  possible 
that  the  optic  nerve  disturbances  may  depend  upon  vasomotor  changes 
in  the  opticus.  Hysterical  oedema  is  well  known,  and  therefore  it  is 
possible  that  an  cedema  might  arise  in  the  optic  nerve  which  would 
bring  about  a  condition  of  elevated  pressure  in  the  optic-fibres,  espe- 
cially in  the  neighborhood  of  the  optic  foramen. 

Not  only  has  restriction  of  the  visual  field  similar  to  that  seen  in 
hysteria  been  found  in  the  diseases  which  have  been  quoted  in  the  pre- 
ceding paragraphs,  but  in  many  other  organic  conditions, — for  example, 
brain  tumor,  lesions  of  the  posterior  portion  of  the  internal  capsule 
(Charcot),  tabes  dorsalis,  Friedreich's  ataxia,  and,  finally,  in  certain 
toxaemias,  notably  those  produced  by  lead,  alcohol,  mercury,  and  bi- 
sulphide of  carbon.  Indeed,  for  a  long  time,  particularly  in  France, 
certain  toxic  agents  have  been  regarded  as  capable  of  originating  hys- 
teria, and  the  whole  subject  has  been  well  elaborated  by  Goinon.*^ 
Sometimes  not  only  is  there  contraction  of  the  visual  field,  but  reversal  of 
the  color  lines,  as  in  Nieden's  case  of  nitrobenzol  poisoning.^* 


"  Zeitschrift  f.  Augenheilk.,  vol.,v.  1901,  p.  391. 

"  Les  Agents  provocateurs  de  I'Hyst^rie,  Paris,  These,  1889. 

"  Consult  The  Toxic  Amblyopias,  by  G.  E.  de  Schweinitz,  pp.  130,  231. 


G40  THE    EYE    AND    NERVOUS    SYSTEM 


HI.   DEFECTIVE   AND    PAINFUL   VISUAL   SENSATION, 
OR  HYSTERICAL  ASTHENOPIA. 

Hysterical  Asthenopia — Thus  far  the  visual  phenomena  of  hys- 
teria have  l)een  representatives  of  various  types  of  antesthesia  of  the 
visual  sense,  or,  in  other  words,  a  diminished  or  abolished  sensibility  to 
visual  impressions.  In  contrast,  a  number  of  patients  of  the  hysterical 
type  are  afflicted  with  markedly  painful  visual  sensations,  notably  dread 
of  light,  lachrymation,  blepharospasm,  neuralgic  pains  which  appear  in 
the  neighborhood  of  the  ocular  globe  and  radiate  to  the  temporal  and 
zygomatic  regions,  and  imperfect  eye  endurance.  To  this  set  of  symp- 
toms the  term  hypercesthesia  of  the  retina  has  sometimes  been  applied. 
They  represent  the  condition  which  Forster  described  as  hysterical 
kopiopia,  Bonders  as  painful  accommodation,  Nagel  as  hypercpsihesia  of 
the  ciliary  muscle,  and  Schenkl  as  hysterical  ocular  pain,  but  which,  in 
general  terms,  may  be  classified  under  the  name  attached  to  the  begin- 
ning of  this  paragraph, — namely,  painful  visual  sensations,  or  hysterical 
asthenopia. 

Most  frequently  encountered  in  women,  and  not  rarely  among  chil- 
dren, it  is  probable  that  the  disorders  of  accommodation  and  of  con- 
vergence which  will  be  described  in  subsequent  pages  are  always  present, 
and  are  responsible  for  the  photojihobia  and  other  symptoms  so  com- 
monly supposed  to  indicate  hyperspsthesia  and  irritation  of  the  retina. 
Any  effort  of  vision,  particularly  at  close  work,  is  quickly  followed  by 
ocular  pain,  brow  neuralgia,  and  diffuse  headache,  often  with  micropsia 
and  obscuration  of  vision,  and  quickly  by  fatigue.  The  intolerance  of 
light,  whether  it  be  diffuse  daylight  or  artificial  light,  is  relieved  by 
tinted  glasses,  to  which  the  patients  naturally  turn. 

Schwarz  l^elieves  that  to  hysterical  weakness  of  sight  with  reduction 
of  visual  acuity,  differentiated  from  pronounced  hysterical  amblyopia, 
should  be  accredited  all  the  cases  which  were  formerly  called  "  anaes- 
thesia and  hypenesthesia  of  the  retina,"  "  amblyopia  of  the  visual  field," 
and  "  nervous  astheniopia,"  but  includes  with  the  symptomatology  con- 
centric contraction  of  the  visual  field,  inversion  of  the  color  lines,  dis- 
turbance of  the  light-  and  color-sense,  and  sometimes  central  scotoma. ' 
It  would  seem,  however,  that  a  large  majority  of  these  cases  present 
only  the  symptoms  before  detailed, — namely,  painful  visual  sensations, 
lack  of  eye  endurance,  photophobia,  chromatophobia,  etc.  We  have  in 
these  cases,  according  to  Binswanger,  to  deal  with  hyperalgesia  mani- 
festing itself  through  light  impressions,  and  ho  believes  that  these  hyper- 
algesic  conditions  form  part  of  a  general  hyperalgesia,  most  pronounced 


NEUROSES  AND  PSYCHOSES  641 

in  certain  forms  of  hystero-neurasthenia,  I  shall,  when  discussing  neu- 
rasthenia, refer  to  this  subject  again,  as  the  best  types  of  nervous  asthe- 
nopia of  this  particular  variety  are  seen  in  the  neurasthenic  rather  than 
the  hysteric  patient.     (See  Page  672.) 

Hysterical  Ophthalmic  Migraine. — At  this  point  reference  should 
be  made  to  the  association  of  ophthalmic  migraine  and  hysteria,  consti- 
tuting the  so-called  "  hysterical  ophthalmic  migraine"  described  by  a 
number  of  authors.^  ^  The  well-known  symptoms  of  ophthalmic  mi- 
graine— paroxysmal  headache,  hemicranial  in  type  and  sometimes  pro- 
ceeding to  the  opposite  side,  ushered  in  by  visual  phenomena,  the  most 
important  of  which  are  various  types  of  expanding  and  scintillating 
scotomas  and  hemianopsia,  the  attack  terminating  in  nausea  and  vomit- 
ing— occur  in  hysteria.  According  to  Pansier,  the  migraine  may  con- 
stitute the  aura  of  the  hysterical  attack,  or  be  contemporarieous  with  it, 
or  be  accompanied  with  symptoms  which  are  undeniably  hysterical,  or, 
finally,  be  brought  into  existence  by  pressure  on  the  migrainous  points 
analogous  to  the  hysterogenetic  zone,  or  by  suggestion.  Wliether  hemia- 
nopsia may  accompany  hysterical  ophthalmic  migraine  has  been  espe- 
cially studied  by  Babinski  and  Parinaud,  and  Gilles  de  la  Tourette,  as 
before  stated  (compare  page  637),  concludes  that  if  it  is  present  it  exists 
only  as  a  temporary  phenomenon,  or,  more  accurately,  seems  to  be  a 
temporary  exaggeration  of  the  concentric  restriction  of  the  visual  field. 
(Compare  Chapter  XVII.) 

IV.  ANOMALIES  OF  ACCOMMODATION,  OR  HYSTER- 
ICAL  CYCLOSPASM  AND  CYCLOPLEGIA. 

It  is  generally  conceded  that  the  most  frequent  ocular  manifestations 
of  hysteria  are  those  concerned  with  anomalies  of  accommodation,  par- 
ticularly in  the  form  of 

(1)  Hysterical  Accommodation-Spasm,  or  Cyclospasm. — This 
condition  may  be  associated  with  amblyopia  or  occur  independently  of  it, 
and  is  seen  with  particular  frequency  in  young  subjects.  It  may  be 
partial  or  total,  unilateral  or  bilateral,  and  has  been  particularly  studied 
by  Parinaud.^^  According  to  this  investigator,  hysterical  spasm  of 
accommodation  is  similar  to  that  produced  by  eserin,  and  has  as  its 
chief  characteristic  a  displacement  towards  each  other  of  the  punctum 

*^  Consult  Charcot,  Legon  de  Mardi,  1887-1888,  p.  101 ;  Babinski,  Archives  de 
Neurologie,  November,  1890;  and  Fink,  Des  Rapports  de  la  Migraine  ophthalmique 
avec  I'Hystgrie,  Th&se,  Paris,  1891. 

"Annales  d'OcuIistique,  tome  Ixxix.  1878,  p.  218;    ibid.,  tome  xcvi.  1886,  p.  47; 
see  also  System  of  Diseases  of  the  Eye,  edited  by  Norris  and  Oliver,  vol.  iv.  p.  747, 
"  The  Ocular  Manifestations  of  Hysteria,"  by  Parinaud. 
41. 


642  THE    EYE    AND    NERVOUS    SYSTEM 

proximum  and  the  pundum  remotum, — that  is  to  say,  there  is  a  diminu- 
tion of  accommodation  at  both  ends  of  the  line.  It  will  be  observed 
that  such  phenomena  are  precisely  opposite  to  those  which  occur  in 
paralysis  of  accommodation,  where  the  punctum  proximum  approaches 
the  punctum  remotum,  and  when  it  is  coincident  with  it  there  is  com- 
plete abeyance  of  the  function  of  accommodation.  In  the  hysterical 
patient,  as  long  as  p  and  r  do  not  coincide,  there  is  some  range  of  accom- 
modation ;  when  by  virtue  of  the  continuance  of  the  displacement  of  r 
towards  p  a  point  is  reached  where  fusion  takes  place,  accommodation 
ceases,  and  the  patient  can  see  only  at  a  fixed  point.     (See   Page   37.) 

From  this  description  of  Parinaud's  it  will  be  seen  that  the  recession 
of  p  brings  about  a  state  of  affairs  resembling  paresis  of  accommodation, 
while  the  advance  of  r  creates  an  apparent  myopia.  The  former  condi- 
tion would  be  neutralized  by  a  convex  glass,  the  latter  by  a  concave  glass. 
In  illustration  a  recent  case  under  my  care  may  be  briefly  quoted:  A 
young  hysterical  girl,  whose  refraction  was  a  hypermetropia  of  1  D., 
during  an  attack  of  spasm  of  the  accommodation  required  — 1  D.  to 
raise  her  vision  to  the  normal  standard,  while  at  the  same  time  her 
amplitude  of  accommodation  was  only  3  D., — that  is,  her  near-point  for 
fine  print  was  33  centimetres,  which  was  brought  back  with  a  suitable 
convex  glass  to  the  normal  point, — namely,  12  centimetres. 

As  Parinaud  points  out,  the  point  of  fusion  may  be  more  or  less 
remote  from  p  or  from  r  without  losing  the  character  of  spasm,  and  the 
distance  of  the  fusion  point  from  p  and  r  must  necessarily  be  in- 
fluenced by  the  refractive  condition  of  the  eye, — that  is,  whether  the 
eye  is  myopic,  hyperopic,  or  presbyopic.  It  will  therefore  be  seen  that, 
according  to  circumstances, — that  is,  according  to  whether  there  has 
been  coincidence  of  p  and  r, — certain  symptoms  must  obtain,  which,  to 
repeat,  are  these :  An  apparent  weakness  of  accommodation  at  the  near- 
point,  a  defect  of  distant  vision  or  a  spasmodic  myopia,  and  as  a  result  of 
the  contracture  of  the  ciliary  muscle  in  a  manner  presently  to  be  ex- 
plained, monocular  diplopia,  sometimes  polyopia,  and  not  infrequently 
micropsia  and  megalopsia. 

The  degree  of  apparent  myopia  from  cramp  of  accommodation  in 
hysterical  patients  varies.  I  have  seen  in  an  hysterical  woman  with 
hypermetropic  refraction  as  follows,  -f-.75s-|-.50c  axis  90,  a  cramp 
represented  by  concave  7  D.,  which  restored  the  visual  acuity  practically 
to  the  normal  standard.  The  most  remarkable  case  of  this  character 
with  which  I  am  acquainted  is  that  reported  by  Loeser.^'^     In  an  hys- 

"  Centralbl.  f.  prakt.  Augenheilk.,  xxvL  1902,  p.  170. 


NEUROSES  AND  PSYCHOSES  643 

terical  boy  without  atropin  a  vision  of  5/10  was  obtained  with  a  — 24  D. 
in  one  eye  and  a  — 22  D.  in  the  other.  After  atropin  the  refraction  of 
the  right  eye  was  -|-  2c  axis  10  and  of  the  left  eye  +  1.50c  axis  25. 

The  other  symptom  to  which  reference  has  been  made,  namely, 
monocular  diplopia  and  polyopia,  is  not  always  manifest  unless  sought 
for  by  the  methods  of  functional  testing, — that  is,  the  patient  does  not 
necessarily  describe  its  presence.  Usually,  when  the  test-object, — for 
example,  a  small  point  of  light, — is  held  at  a  certain  distance  from  the 
eye,  it  is  seen  singly,  but  at  points  nearer  or  further  from  that  at  which 
single  vision  is  obtained  the  diplopia  appears.  According  to  Parinaud, 
the  second  image  usually  separates  from  the  first  on  the  temporal  side, 
but  a  vertical  displacement,  or  one  obliquely  situated  from  the  test- 
object,  is  also  seen.  Occasionally  polyopia,  or,  more  accurately,  tri- 
plopia,  is  evident, — that  is,  a  third  indistinct  image  is  observed  upon 
the  opposite  side.  According  to  Parinaud,  when  it  appears  beyond  the 
point  of  single  and  distinct  vision  it  can  be  made  to  disappear  by  the 
use  of  a  concave  glass,  but  when  it  is  seen  on  the  proximal  side  of  the 
point  of  single  vision,  it  disappears  if  viewed  through  a  convex  glass. 

A  partial  contraction  of  the  muscles  of  accommodation  has  been 
described  by  Borel,  Galezowski,  and  Pansier.  This  irregular  spasm  of 
the  ciliary  muscle  creates  more  or  less  astigmatism,  which  disappears 
with  the  restoration  of  the  patient's  general  health. 

Schwarz  ^*  insists,  in  his  description  of  these  phenomena,  on  the 
difference  of  their  behavior  in  binocular  and  monocular  vision.  Accord- 
ing to  him,  in  binocular  vision  hysterical  accommodation  disturbances 
are  rare.  In  high  grades  of  accommodation-spasm  there  is  usually  an 
associated  convergence-cramp.  The  cyclospasm  appears  only  during 
fixation,  and  the  object  regarded,  at  first  plainly  seen,  becomes  blurred 
and  double  vision  appears.  Should  this  double  vision  continue,  even  if 
one  eye  is  covered,  the  condition  is  one  of  monocular  diplopia,  often 
associated  with  micropsia.  If,  on  the  other  hand,  the  double  vision 
disappears  when  either  of  the  eyes  is  covered,  binocular  diplopia  exists, 
and  there  is,  if  the  diplopia  is  homonymous,  hysterical  convergence-  and 
accommodation-spasm,  provided  paresis  of  the  abducens  is  excluded.  If 
the  double  vision  is  crossed,  then  there  is  weakness  or  paresis  of  con- 
vergence, provided  organic  disease  is  absent. 

Another  symptom  connected  with  hysterical  spasm  of  accommodation 
is  an  inability  properly  to  estimate  the  size  of  a  test-object  which  is  fixed. 


"  Die  Bedeutung  der  Augenst(5rungen  fiir  die  Diagnose  der  Him-  und  Rticken- 
marks-Krankheiten,  Berlin,  1898,  p.  80. 


644  THE    EYE    AND    NERVOUS    SYSTEM 

For  example,  the  test-object  appears  to  grow  larger  when  it  is  made  to 
approach  the  eye,  and  appears  to  grow  smaller  when  it  is  carried  to  a 
distant  point,  corresponding  in  this  respect  to  the  size  of  the  retinal 
images.  To  this  symptom  Parinaud  has  given  the  name  micro-megalop- 
sia,  and  he  considers  it  an  almost  constant  one  in  patients  suffering 
from  hysterical  amblyopia  and  spasm  of  accommodation. 

(2)  Hysterical  Paralysis  of  Accommodation,  or  Cycloplegia. — Al- 
though paralysis  of  the  ciliary  muscle,  which  has  been  observed  to  exist 
both  in  a  partial  and  a  complete  condition,  is  uncommon  as  compared 
with  spasm  of  accommodation,  it  is  not  unusual  to  see  in  young  hysterics, 
and  especially  in  hysterical  children,  mild  degrees  of  accommodative 
weakness,  so  that  the  patient  is  unable  to  read  fine  print  at  the  distance 
normal  to  the  age  and  to  the  condition  of  refraction,  and  is  able  to  do  so 
only  with  the  aid  of  convex  glasses.  As  has  already  been  pointed  out, 
this  does  not  necessarily  mean  that  there  is  a  paresis  of  the  ciliary 
muscle,  because  we  have  seen  that  while  there  may  be  a  recession  of  the 
near-point  there  is  usually  an  advance  of  the  far-point,  and  spasm  is 
therefore  not  excluded,  the  condition  being  one  of  contracture  of  accom- 
modation. 

On  the  other  hand,  in  common  with  many  other  observers,  I  have  a 
number  of  times,  particularly  in  mild  types  of  hysterical  manifestations, 
often  those  following  injury  in  young  people,  observed  undoubted 
paresis  of  accommodation  comparable  in  all  particulars  to  an  imperfect 
power  of  the  ciliary  muscle,  such  as  might  be  produced  if  a  cyclo- 
plegic  had  not  exercised  its  full  power.  Wilbrand  and  Saenger  have 
recorded  instances  of  paresis  of  accommodation  with  transitory  diplopia 
in  nervous  children,  and  have  demonstrated  that  these  temporary  condi- 
tions of  weakness  of  accommodation  may  pass  on  to  paresis  of  the  ciliary 
muscle.  They  are  well  kno\vn  as  part  of  the  symptomatology  of  hys- 
terical asthenopia.     (See  Page  640.) 

According  to  Schwarz,*®  hysterical  accommodation-paralysis  is 
usually  bilateral,  and  generally  associated  with  palsy  of  convergence. 
If  this  is  present  and  there  exists  at  the  same  time  no  divergent  strabis- 
mus at  the  distant  point,  the  hysterical  nature  of  the  disturbance  is 
probable.  The  same  author  insists,  if  convergence  is  normal,  that  the 
binocular  range  of  accommodation  should  be  tested,  after  adjustment  of 
any  difference  in  refraction  of  the  two  eyes.  Should,  under  these  cir- 
cumstances, the  binocular  accommodation  ran^  be  essentially  greater 
than  the  monocular,  the  hysterical  nature  of  the  paralysis  may  be  as- 

"T.OC.  cit..  p.  82. 


NEUROSES  AND  PSYCHOSES  645 

sumed,  because  with  convergence  in  binocular  vision  the  apparently 
paralyzed  accommodation  comes  into  action.  If  the  accommodation  in 
binocular  vision,  in  spite  of  good  convergence,  remains  as  much  in 
abeyance  as  in  monocular  vision,  organic  paresis  is  probably  present. 
This  opinion  is  disputed  by  Blok,^°  who  reports  double  hysterical  spastic 
mydriasis  accompanied  by  bilateral  paralysis  of  accommodation.  So- 
called  typical  cases  of  paralysis  of  accommodation,  with  diplopia,  have 
been  recorded  by  Nonne  and  Beselin ;  they  are  especially  apt  to  occur  if 
the  refraction  is  hyperopic. 

Schwarz's  observation  of  the  disappearance  of  unilateral  hysterical 
accommodation-paralysis  in  binocular  vision  corresponds  to  the  sub- 
sidence of  monocular  amaurosis  in  binocular  fixation.  In  other  words, 
the  unilateral  paralysis  of  accommodation  is  apparent  only,  and  Schwarz 
Ijelieves  that  in  these  cases  it  has  not  been  proven  that  the  palsy  per- 
sisted during  binocular  vision.  Therefore  he  doubts  the  occurrence  of 
such  a  pure  unilateral  hysterical  cycloplegia. 

Although  the  pupils  are  usually  normal  in  cases  of  hysterical  cyclo- 
plegia, this  is  not  necessarily  the  case,  and  mydriasis  has  been  reported, 
— for  example,  by  Donoth,  Nonne  and  Beselin,  and  Bloq,  the  last-named 
reporter,  as  stated  above,  having  recorded  a  case  of  mydriasis  and 
accommodation-paralysis,  at  first  alternating  from  the  right  to  the  left 
eye,  but  later  becoming  stationary  in  both  eyes,  and  associated  with  con- 
vergent strabismus.     The  action  of  eserin  was  only  slightly  manifested. 

Concerning  the  Pathogenesis  of  Hysterical  Accommodation- Dis- 
turbances.— As  has  already  been  sufficiently  stated  and  largely  proved 
by  Parinaud,  the  most  usual  basis  of  the  phenomena  under  considera- 
tion is  a  spasm  of  the  ciliary  muscle,  and  this  spasm,  if  complete, 
wipes  out  the  amplitude  of  accommodation  because  the  eye  is  adapted 
for  a  fixed  point  at  which  there  is  single  vision.  On  either  side  of  this 
fixed  point  diplopia  occurs,  and  the  cause  of  this  phenomenon  is  re- 
ferred by  Parinaud  to  structural  defects  in  the  crystalline  lens.  He 
writes  as  follows :  "  The  disposition  of  the  anterior  portion  of  the  lens 
in  three  segments  favors  its  changes  in  curvature,  but  at  the  same  time, 
owing  to  their  separation  from  one  another  by  layers  of  amorphous 
material,  each  section  possesses  a  focal  point  of  its  o^vn,  capable  of 
producing  images  distinct  from  that  yielded  by  the  lens  as  a  whole. 
The  condition  of  spasm  by  increasing  the  convexity  of  the  anterior 
surface  of  the  lens  serves  to  emphasize  the  diplopia  arising  from  the 
structural  defects  described."     He  believes  that  in  those  cases  in  which 

"Annales  d'Oculistique,  cxix.  1S98,  p.  200. 


646  THE    EYE    AND    KERVOUS    SYSTEM 

anomalies  of  the  central  nervous  system  have  been  brought  forward  to 
explain  multiple  images,  the  condition  of  the  refraction  has  not  been 
perfectly  examined.  On  the  other  hand,  Pierre  Janet,  while  admitting 
Parinaud's  explanations,  is  unwilling  to  deny  the  probable  psychic 
origin  of  some  of  these  phenomena.  The  probable  explanation  of 
micropsia  and  megalopsia  has  been  described,  and  the  interesting 
waking  up  of  accommodative  effort  (which  was  in  abeyance  in  monocu- 
lar vision)  during  binocular  fixation  has  been  recorded. 

V.  ANOMALIES   OF   THE   IRIS   MOVEMENTS,   OR 
HYSTERICAL    PUPIL    PHENOMENA. 

Pupil  disturbances  of  hysterical  origin  have  occasioned  much  dis- 
pute, and  Schwarz's  statement  that  observations  concerning  anomalies  of 
the  iris  movements  of  hysterical  origin  are  often  inexact  is  undoubtedly 
true,  as  is  also  the  remark  of  Parinaud  that  pupillary  anomalies  occur 
rarely  as  permanent  manifestations  of  ocular  hysteria.  The  following 
conditions  have  been  reported  to  be  present  in  hysteria : 

( 1 )  Contraction  of  the  pupil, — that  is,  either  spasmodic  or  paralytic 
myosis. 

(2)  Dilatation  of  the  pupil, — that  is,  either  spasmodic  or  paralytic 
mydriasis,  the  dilated  pupil  in  one  class  of  cases  responding  to  light  im- 
pulses, even  when  light  perception  is  denied,  and  in  another  class  of  cases 
failing  to  contract  under  the  influence  of  light  impulse.  *i 

(3)  Interchange  of  mydriasis  and  myosis. 

(4)  Unilateral  mydriasis  in  one  class  of  cases  unassociated  with 
paresis  of  accommodation,  in  another  class  of  cases  associated  with  pare- 
sis of  accommodation. 

(5)  Dilatation  of  the  pupil  with  preserved  but  sluggish  reaction  to 
light  and  paralysis  of  accommodation. 

Contraction  of  the  pupil,  or  hysterical  spasmodic  myosis,  as  an  iso- 
lated symptom,  if  it  occurs  at  all  in  hysteria,  must  be  exceedingly  rare, 
although  myotic  pupils,  according  to  Westphal,^^  may  appear  indepen- 
dently of  hysterical  seizures,  or  at  the  conclusion  of  them,  or  sometimes 
during  trivial  attacks.  In  so-called  hysterical  pseudomeningitis,  myosis 
has  been  described  (Boissard,  quoted  by  Gilles  de  la  Tourette),  but 
whether  the  myosis  was  spastic  or  paralytic  is  a  matter  of  doubt.  Hys- 
terical rigidity  of  the  pupil  has  been  observ'ed  by  Spiller.  Dilatation  of 
the  pupil  following  myotic  contracture  has  been  reported,  and  Gilles  de 
la  Tourette  considers  this  preliminary  contraction  of  the  pupil  im- 

"  Berlin,  klin.  Wochenschr.,  xxxiv.,  1897,  p.   1024. 


K"EUROSES  AXD  PSYCHOSES  647 

portant.  He  doubts  whether  there  is  a  true  paralysis  of  the  sphincter  of 
the  iris  which  causes  the  subsequent  mydriasis,  and  attributes  the  dila- 
tation to  a  preponderating  effect  of  the  dilator  after  the  contracted  iris 
has  relaxed.  The  presence  of  pupil  contraction  in  association  with  ac- 
commodation and  convergence  cramp,  according  to  Schwarz,  is  un- 
doubtedly often  present,  and  Galezowski  has  noticed  myosis  as  an  ac- 
companiment of  spasmodic  myopia,  as  it  has  already  been  described  in 
a  j^revious  section,  an  observation  which  Parinaud  has  not  been  able 
to  confirm.  In  general  terms,  the  myotic  pupils,  which  should  be 
ascribed  to  a  spastic  condition  of  the  sphincter  of  the  iris,  are  brought 
about  by  psychic  disturbances,  and  as  already  noted,  may  appear  inde- 
pendently of  the  attacks,  or  at  the  conclusion  of  them ;  when  the  psychic 
disturbance  is  removed  the  pupil  may  dilate. 

Spasmodic  mydriasis  in  connection  with  hysteria  has  been  described 
frequently.  The  literature  has  been  reviewed  by  Aurand  and  Frenkel,^^ 
!N^onne  and  Beselin,^^  Binswanger,^^  and  Pichler,^^  ^nd  from  a  study  of 
this  literature  Binswanger  asserts  that  although  hysterical  mydriasis  is 
rare,  it  has  a  true  and  autonomous  existence.  Clinically  it  is  necessary 
to  distinguish  between  a  spasmodic  and  a  paralytic  form,  either  of  which 
may  be  independent,  but  Binswanger  thinks  it  must  be  stated  that  a 
patient  may  have  at  the  same  time  spasmodic  and  paralytic  mydriasis. 
We  must  further  grant  that  in  one  class  of  cases  of  mydriasis,  even  if 
associated  with  amaurosis,  there  is  response  of  the  pupil  to  light  im- 
pulse, while  in  another  class  of  cases  there  is  failure  of  the  iris  to 
contract  under  the  influence  of  light.  Again,  the  dilatation  of  the  pupil 
may  or  may  not  be  associated  with  paralysis  of  accommodation.  In 
many  of  the  cases  of  mydriasis  there  is  amaurosis,  and  the  mydriasis 
disappears  when  sight  is  restored,  but  Aurand  and  Erenkel  think  that  it 
may  occur  quite  independently  of  amblyopia,  or  even  a  greater  deprecia- 
tion of  vision.  Thus,  Parinaud  and  Richer  report  a  case  of  hysteria 
with  paralysis  of  accommodation  and  mydriasis,  Harlan  one  of  mydria- 
sis, accommodation-paralysis,  and  blepharospasm,  while,  according  to 
De  Wecker,  in  hysteria  the  mydriasis  may  be  associated  with  cyclo- 
plegia  or  exist  without  it.  Touching  the  question  of  whether  the 
mydriasis  is  a  paralytic  one  or  not,  Schwarz  maintains  that  although  it 
is  often  so  stated,  such  statement  is  not  necessarily  based  upon  accurate 

°-  Revue  de  Medecine,  xvi.  1896. 

^  Festschrift  zur  Feier  des  achtzigjahrigen  Stiftungsfestes  des  Aerztlichen  Ver- 
eins  zu  Hamburg,  Leipzig,  1896. 
"  Loc.  cit.,  p.  628. 
"Zeitschr.  f.  Augenheilk. 


648  THE    EYE    AND    NERVOUS    SYSTEM 

observation.  Even  when  the  pupil,  widely  dilated,  does  not  react  to 
light,  it  is  not  necessary  that  a  paralytic  mydriasis  should  be  assumed. 
A  pure  paralysis  of  the  iris  movement  not  brought  into  existence  by  a 
medicament  can  be  stated  to  exist  only  if  the  pupil  promptly  responds  to 
the  action  of  pilocarpin  or  eserin.  Even  the  presence  of  hysterical 
cycloplegia  does  not  prove,  according  to  him,  that  the  mydriasis  is 
paralytic,  for,  on  the  one  hand,  one  may  have  paralysis  of  accommodation 
without  pupil  paralysis,  as,  for  example,  in  diphtheria,  and,  on  the 
other  hand,  as  has  already  been  shown,  the  so-called  hysterical  accommo- 
dation-paralysis is  not  a  paralysis  in  the  true  sense  of  the  word.  The 
cases  of  paralytic  mydriasis  reported  by  Roeder,  Donoth,  and  Nonne  and 
Beselin  have  not  been  accepted  by  all  authors.  It  has  been  suggested, 
for  example,  that  atropin  might  have  been  used  without  knowledge,  and 
although  in  Nonne  and  Beselin's  case  eserin  failed  to  contract  the  pupil, 
and  a  central  paralysis  and  a  spastic  mydriasis  were  undoubtedly  ex- 
cluded, and  although  the  patient  was  in  a  certain  sense  watched  in  so 
far  as  the  use  of  atropin  is  concerned,  Schwarz  is  evidently  unwilling  to 
accept  the  paralytic  nature  of  the  mydriasis,  which  these  authors,  how- 
ever, do  not  hesitate  to  maintain,  describing  it  as  an  undoubted  paresis  of 
the  sphincter  of  the  iris  in  a  case  of  typical  grand  hysteria.  They 
record  a  second  case  which  they  describe  as  hysterical  ophthalmoplegia 
interna.  Liebrecht,  on  the  other  hand,  remarks  that  he  has  never 
seen  an  undoubted  case  of  hysterical  internal  ophthalmoplegia. 

As  has  already  been  stated,  mydriasis  in  the  absence  of  paralysis  of 
accommodation  is  due  to  a  spasm  of  the  dilator  muscle.  To  explain  the 
paralytic  mydriasis,  Nonne  and  Beselin,  as  well  as  Donoth,  assume  an 
abeyance  of  the  cortical  centre  governing  the  functions  of  the  sphincter 
of  the  iris  and  the  muscle  of  accommodation,  and  in  this  respect  estab- 
lish an  analogy  between  the  condition  described  in  so  far  as  etiological 
considerations  are  concerned,  and  hysterical  amblyopia,  amaurosis,  dys- 
chroraatopsia,  and  concentric  visual  field  contraction.  Binswanger, 
although  he  does  not  doubt  the  occurrence  of  pupillary  rigidity  or 
pupillary  sluggishness  as  an  hysterical  symptom,  warns  against  its  being 
mistaken  for  reflex  light  rigidity  in  the  narrow  sense  of  the  term,  which 
belongs  to  the  domain  of  the  organic  diseases  of  the  brain  and  spinal 
cord,  pupillary  rigidity  in  hysteria  being  associated  with  myosis  and 
spasm  of  accommodation  and  convergence.  Deutschmann,  Wilbrand, 
and  other  observers  also  point  out  the  necessity  of  making  a  distinction 
between  a  true  reflex  pupillary  rigidity  and  mydriatic  pupils  which  do 
not  react  either  to  light  or  accommodation.  In  response  to  such  objec- 
tion Nonne  and  Beselin  point  out  that  they  do  not  describe  reflex  pupil- 


NEUROSES  AND  PSYCHOSES  649 

lary  rigidity,  but  only  mydriatic  pupils  which  fail  to  respond  to  light 
and  convergence,  and  again  maintain  that  the  condition  is  due  to  func- 
tional injury  of  the  cortical  centres  for  the  innervation  of  the  sphincter 
or  of  the  dilator.^^ 

Pupil  dilatation  may  exist  for  a  short  time,  for  a  comparatively  long 
period,  and  in  rare  instances, — for  example,  in  Harlan's  and  in  Nonne 
and  Beselin's  cases, — for  several  months  or  even  years.  The  pupil 
may  be  made  to  contract  by  suggestion,  hypnotism,  and  by  causing  the 
patient,  as  in  Pichler's  case,  to  read  through  a  plane  glass. 

Thus  far  we  have  spoken  particularly  of  bilateral  dilatation  of  the 
pupil.  There  may  be,  ho^vever,  a  unilateral  mydriasis,  sometimes  asso- 
ciated with  paresis  of  accommodation  and  sometimes  without  such 
association,  and  there  may  be  dilatation  of  the  pupil  with  preserved  but 
sluggish  reaction  to  light  and  so-called  hysterical  paralysis  of  accommo- 
dation. Gilles  de  la  Tourette  describes  a  hypothetical  unilateral  mydria- 
sis in  total  amaurosis  of  one  or  both  eyes  as  the  result  of  the  loss  of  the 
light  reflexes,  which  under  normal  conditions  influence  the  pupillary 
reaction,  but  Binswanger,  commenting  on  this  case,  thinks  that  there 
are  some  cases  in  literature  belonging  to  this  category, — for  example, 
those  reported  by  Mendel.  H.  A.  Hare  and  myself  have  recorded  ^^ 
complete  amaurosis  with  mydriasis  of  the  right  side,  the  dilated  pupil 
reacting  sluggishly  to  light  impulses  and  moderately  to  eserin,  with 
marked  myosis  of  the  left  side  and  moderate  amblyopia,  in  a  hysterical 
woman,  which  had  existed  for  many  years,  the  use  of  drugs  being 
absohitely  excluded  by  keeping  her  under  the  observation  of  trained 
nurses  for  weeks  at  a  time  in  a  hospital  room. 

In  addition  to  immobility  of  the  pupil  there  is  sometimes  an  inter- 
change of  myosis  and  mydriasis,  and  Lagovides  reports  a  case  of  hys- 
terical alternating  and  intermittent  mydriasis,  while  Blok  has  observed  a 
case  of  mydriasis  and  accommodation-paralysis  at  first  alternating  from 
the  right  to  the  left  side,  but  later  stationary  in  both  eyes,  associated 
with  strabismus  convergens. 

Before  dismissing  this  subject  the  condition  of  the  pupils  during 
hysterical  attacks  remains  to  be  described.  We  have  already  noted  that 
pupil  immobility  may  occur  during  an  attack,  at  the  end  of  an  attack,  or 
without  evident  association  with  an  attack,  although  permanent  pupil 
immobility  has  not  been  observed. 

Striimpel,  Oppenheim,  and  Mobius  at  one  time  maintained  that  a 

"For  further  discussion  of  this  subject  and  the  views  of  neurologists  and  oph- 
thalmologists, see  Binswanger,  loc.  cit.,  p.  637. 
"American  Medicine,  April  21,   1901. 


650  THE    EYE    A:N^D    NERVOUS    SYSTEM 

differential  diagnosis  between  epilepsy  and  hysteria  could  be  made  by 
observing  the  i^iipils,  which  in  the  former  w^ere  wide  and  reactionless, 
while  in  the  latter  affection  their  reaction  was  preserved.  Karplus  ^^ 
first,  in  1896,  observed  pupil-immobility  during  hysterical  attacks,  and  a 
year  later  Westphal  established  the  accuracy  of  Karplus's  contention 
that  the  state  of  the  pupils  was  not  sufficient  to  make  a  differential 
diagnosis  between  epilepsy  and  hysteria.  Karplus  still  later  accurately 
investigated  a  series  of  cases  of  spontaneous  and  induced  hysterical 
attacks,  during  which  the  pupils  were  dilated  and  immobile.  He  con- 
siders reflex  pupil  immobility  a  frequent  phenomenon  in  paroxysmal 
hysteria,  and  believes  that  it  depends  upon  a  cortical  origin,  the  pupil- 
dilatation  and  pupil-contraction  being  represented  in  the  cortex,  and 
that  a  tonic  cramp  of  the  muscular  structure  of  the  iris  can  occur,  and 
therefore  light  reaction  not  take  place,  exactly  as  a  tonic  cramp  of  the 
legs  in  hysteria  may  be  responsible  for  the  elimination  of  the  patellar 
reflex. 

Finally,  we  may  have  in  hysteria  a  paradoxic  pupil  reaction  which- 
has  been  reported  by  Westphal  and  by  Lepine.  The  last-named  author's 
patient  showed  dilatation  of  the  pupil  when  light  fell  upon  the  exposed 
eyes,  this  dilatation  remaining  for  a  few  seconds.  The  transitory  and 
inconsistent  changes  in  the  pupil,  to  use  Binswanger's  words,  which  are 
seen  in  so  many  hysterical  patients,  should  be  regarded  chiefly  as  an 
expression  of  a  pathological  perversion  by  virtue  of  which  psychic 
processes  are  more  easily  transmitted  to  the  movement  of  the  pupil  than 
under  normal  conditions.  Thus  it  is  that  we  sometimes  have  a  wide  but 
quickly  reacting  pupil, — that  is,  one  which  dilates  rapidly  under  the 
influence  of  shock,  excitement,  exertion,  etc., — and  also,  as  has  often  been 
observed,  the  increase  of  the  natural  Jiippus  of  the  pupil.  I  have  seen  in 
one  hysterical  patient  of  Dr.  Weir  Mitchell  the  most  marked  example  of 
exaggerated  hippus  that  has  come  under  my  observation,  except  as  I 
have  seen  it  in  one  or  two  cases  of  insanity.  Von  Ketley  ^^  describes 
a  myasthenic  action  of  the  pupils  in  hysteria.      (Compare  Chapter  VI.) 

VI.  ANOMALIES  OF  THE  EYELID  AND  EYE-MUSCLE  MOVE- 
MENTS, OR  HYSTERICAL  SPASMS,  CONTRACTURES,  PSEUDO- 
PALSIES,  AND  PALSIES  OF  THE  PALPEBRAL  AND  BULBAR 
MUSCULATURE. 

1.  Blepharospasm. — This  condition  appears  in  a  clonic,  tonic,  and 
so-called  pseudoparalytic  form. 

^  Lehrbuch  f.  Psychiatric,  xvii.  1898,  pp.  1-53. 
"VVien.  med.  Wochenschr.,  Nr.  33,  1901,  p.  1559. 


I^EUROSES  A^D  PSYCHOSES  651 

(a)  Clonic  blepharospasm,  common  enough  in  hysteria,  is  not  neces- 
sarily an  ocular  stigma  of  this  affection.  It  is  characterized  by  a  vibra- 
tory and  continual  blinking  of  the  lids  during  the  attack.  Usually 
bilateral,  clonic  spasm  of  one  orbicularis  appearing  in  the  form  of 
fibrillary  contraction  has  been  observed.  Frequently  part  of  the  symp- 
tomatology^ of  an  hysterical  seizure,  it  may  arise  independently  of  such 
association.  In  one  form  of  the  affection  described  by  Bernutz  as  the 
attenuated  variety,  there  is  a  constant  vibration  or  trembling  of  the 
closed  or  half-closed  lids,  which  does  not  cease  even  during  sleep. 

(&)  Tonic  Blepharospasm. — This  is  characterized  by  a  convulsive 
closure  of  the  eye,  and,  unlike  the  clonic  variety  of  this  affection,  it  is 
frequently,  if  not  usually,  unilateral,  or  at  least  is  more  marked  upon 
one  side  than  the  other,  and  the  spasm  may  involve  the  neighboring 
muscles  of  the  face  and  neck.  The  skin  of  the  lid  is  forcibly  folded 
and  the  palpebral  borders  are  vigorously  applied  one  against  the  other. 
If  an  effort  is  made  to  raise  the  lid, — for  example,  by  means  of  the 
finger, — a  definite  resistance  is  demonstrable ;  and  if  the  patient  under 
command  tries  to  open  the  eye,  the  only  response  is  a  trembling  move- 
ment without  separation,  or  without  distinct  separation,  of  the  ciliary 
margins  which  have  been  in  contact  with  each  other.  Along  with  this 
effort  there  may  be  a  distortion  of  the  face  and  contraction  of  other 
muscles, — for  example,  those  opening  the  mouth.  Quite  commonly 
blepharospasm  is  associated  with  intense  photophobia,  convulsive  dis- 
placement of  the  eyes  in  different  directions,  dyschromatopsia,  spasm  of 
accommodation,  and  amblyopia.  Indeed,  it  was  originally  believed  by 
Hocken  that  blepharospasm  always  accompanied  amaurosis,  although  we 
now  know  that  such  is  not  the  case. 

Two  clinical  varieties  have  been  described,  the  painful  and  the  non- 
painful.  The  former,  usually  bilateral,  may  be  accompanied  by  the 
visual  troubles  before  named,  especially  lachrymation  and  photophobia, 
while  the  latter,  or  the  non-painful  form,  is  frequently  unilateral. 
Gilles  de  la  Tourette  has  described  in  painless  blepharospasm  an  anaes- 
thetic zone  around  the  eye  extending  for  a  distance  of  two  or  more 
centimetres  above  and  below  the  orbit.  The  conjunctiva  is  insensitive, 
although  the  cornea  may  still  retain  its  sensibility,  except  at  the  internal 
portion.  In  painful  blepharospasm  with  photophobia  the  zone  of  anaes- 
thesia is  replaced  by  one  of  hypersesthesia,  which  may  extend  to  the 
cornea.  Richer  and  Gilles  de  la  Tourette  have  noted  that  compression  of 
the  supraorbital  region  may  cause  momentary  disappearance  of  the 
blepharospasm,  while  pressure  upon  other  areas  in  the  neighborhood  of 
the  eye  may  cause  an  exacerbation  or  a  relapse  after  quietude.     Almost 


652  THE    EYE    AND    NERVOUS    SYSTEM 

complete  cessation  of  the  blepharospasm  of  one  of  my  patients  could  be 
induced  by  hard  pressure  in  the  neighborhood  of  the  supraorbital 
foramen.  Furthermore,  the  blepharospasm — as,  for  example,  in  Ze- 
hender's  patient — may  be  exaggerated  or  called  into  existence  by  press- 
ure on  the  first  cervical  vertebra  or  on  the  sternal  region  or,  as  in  Seelig- 
miiller's  case,  on  the  lower  jaw  in  the  neighborhood  of  the  molar  teeth. 

The  condition  may  develop  without  any  external  cause  in  conjunc- 
tion with  or  after  an  hysterical  crisis,  or  very  frequently  in  the  ordinary 
reflex  manner,  after  a  conjunctivitis,  a  corneal  affection,  or  a  sudden 
flash  of  light.  Sometimes  the  blepharospasm  arises  suddenly  and  dis- 
appears with  suddenness  or,  the  blepharospasm  being  complete,  con- 
vulsive movements  of  the  lids  in  the  form  of  exacerbations  occur  spon- 
taneously, lasting  from  a  few  minutes  to  several  hours.  Contracture  of 
the  orbicularis  does  not  always  disappear  suddenly ;  and  Charcot  and 
Gilles  de  la  Tourette  have  published  cases  in  which  it  has  lasted  for 
several  years.  Again,  as  in  Harlan's  patient,  there  may  be  an  inter- 
mittent violent  blepharospasm  of  one  eye,  with  contraction  of  the  pupil^ 
spasm  of  accommodation,  and  narrowing  of  the  visual  field. 

Binswanger  regards  blepharospasm  as  a  concomitant  of  the  general 
pathological  tendency  in  hysteria  to  the  formation  of  contractures. 
According  to  Gilles  de  la  Tourette,  its  origin  should  be  considered 
central.  Other  authors  believe  that  contraction  of  the  orbicularis,  at 
least  certain  varieties  of  it,  are  due  to  hyperesthesia  of  the  retina,  which 
causes  a  reflex  closing  of  the  lid  to  protect  the  eye  from  the  excessive 
light. 

(c)  Pseudoparalytic  Ptosis. — In  spite  of  this  name,  which  we  owe  to 
Parinaud,  as  we  do  also  in  large  measure  the  differentiation  of  the 
affection  from  a  true  ptosis,  this  condition  is  not  the  result  of  levator 
weakness,  but  depends  upon  a  cramp  or  spasm  of  the  palpebral  portion 
of  the  orbicularis.  Therefore  it  would  seem  that  the  other  term  spastic 
ptosis  is  the  more  appropriate  one. 

According  to  Parinaud,  pseudoparalytic  ptosis  resembles  palsy  of 
the  levator  because  the  upper  lid  droops  without  wrinkling  the  skin  of 
the  lid,  and  if  the  patient  attempts  to  open  the  eye  there  is  a  con- 
traction of  the  occipitofrontalis  similar  to  that  which  one  sees  in  real 
paralytic  ptosis.  The  lid  raised  with  the  finger,  however,  when  released 
falls  more  rapidly  than  in  paralytic  ptosis,  and  the  patient  may,  if  his 
attention  is  directed  elsewhere,  voluntarily  elevate  the  lid,  or  as  dusk 
comes  on,  l)ecause  photophobia  and  amblyopia  are  frequently  accompany- 
ing symptoms,  perform  the  same  act.  According  to  Charcot  and  Lan- 
dolt,  in  true  paralysis  of  the  levator  the  eyebrow  is  elevated,  inasmuch  a& 


NEUROSES  AXD  PSYCHOSES  653 

the  patient  endeavors  to  lift  the  eyelid  by  contracting  the  occipito- 
frontalis,  while  in  pseudoptosis,  which  is  really  due  to  a  partial  spasm 
of  the  orbicularis,  the  eyebrow  follows  the  contraction  of  this  muscle  and 
descends  below  the  normal  level,  and  there  is  obliteration  of  the  wrinkles 
on  the  corresponding  side  of  the  forehead  (Fig.  10).  This  important 
observation  is  of  particular  interest  because  we  may  have,  as  Charcot 
has  shown,  a  simulation  by  hysteria  of  the  syndrome  of  Weber, — that  is 


Fig.  10.— Hysterical  spastic  ptosis.    (After  Wilbrand  and  Saenger.) 

to  say,  a  ptosis  on  the  side  opposite  to  hemiplegia.  It  would  seem,  how- 
ever, that  this  so-called  eyebrow-sign  is  not  a  constant  one,  as  Wilbrand 
and  Saenger  have  shown,  and  that  the  other  indications  referable  to 
blepharospasm  must  be  sought  out  and  examined.  Sometimes  the 
depressed  eyebrow  is  straighter  than  the  one  on  the  opposite  side;  the 
overhanging  folds  of  skin  of  the  upper  lid  are  converted  into  a  series  of 
parallel  or  concentric  folds,  the  free  border  of  the  lid  is  curved,  the 
occipitofrontalis  muscle  comparatively  smooth,  and  the  palpebral  fissure 
not  entirely  closed.  Finally,  there  may  be  some  resistance  to  the  finger  in 
the  effort  to  raise  the  lid,  and  the  pseudoptosis  does  not  disappear  when 
there  is  over-extension  of  the  head.  Binswanger,  who  elaborates  these 
differential  diagnostic  points,  thinks  they  are  important  to  support  the 
diagnosis  of  pseudoptosis  in  doubtful  cases,  and  calls  attention  to  the 
fact  that  a  sharp  distinction  between  spastic  ptosis  and  fully  developed 
blepharospasm  is  often  difficult,  especially  as  there  are  transitory  forms 


654  THE    EYE    AND    NERVOUS    SYSTEM 

— as,  for  example,  in  Zehender's  case,  in  which  blepharospasm  termi- 
nated in  pseudoptosis. 

Although  spastic  ptosis  is  usually  unilateral,  bilateral  varieties  of 
the  affection  have  been  reported,  and  the  condition  may  be  accompanied 
by  hemispasms  elsewhere, — for  example,  glossolabial  hemispasm,  as  in  a 
case  of  Gilles  de  la  Tourette's.  Binswanger  points  out  that  hyperaes- 
thesia  of  the  retina  may  be  a  precursor  of  pseudoptosis,  and,  quoting 
Bardol's  case,  insists  that  sometimes  psychic  causes  alone  are  operative. 
He  agrees  with  Wilbrand  and  Saenger  that  a  palpebral  spasm  brought 
about,  as  in  the  case  recorded,  by  a  flash  of  lightning,  represents  a  patho- 
logical persistence  of  the  voluntary  closing  of  the  lids  to  shut  off  the  sight 
of  the  storm,  and  demands  that  spastic  ptosis  in  hysteropathic  individ- 
uals with  overexcitability  in  the  sensory  sphere  should  be  regarded  as  a 
pathological  exaggeration  of  physiological,  cortical,  and  intracortical 
reflex. 

2.  Hysterical  Parzilysis  of  the  Orbicularis — According  to  Gilles 
de  la  Tourette,  the  title  just  given  is  a  misnomer,  as  palsy  of  the  orbicu- 
laris has  not  been  proved  to  occur  in  hysteria,  and  Wilbrand  and  Saenger 
are  not  able  to  report  a  hysterical  paralysis  of  this  nature.  It  is  true 
that  Gilles  de  la  Tourette  refers  to  transitory  lagophthalmos  during  hys- 
terical attacks,  forming  a  part  of  a  group,  as  he  expresses  it,  of  con- 
tractures and  spasms  difficult  to  analyze.  Even  in  so-called  hysterical 
facial  paralysis,  the  orbicularis  seems  to  be  respected.®^ 

3.  Hysterical  Spasm  of  the  Levator. — According  to  Koenigshofer,^^ 
hysterical  cramp  of  the  elevator  of  the  upper  lid  has,  up  to  the  present 
time,  been  observed  only  once.  No  reference  is  given,  but  doubtless  the 
statement  is  based  upon  the  case  which  has  been  reported  by  Gold- 
scheider,  and  which  is  summarized  by  Wilbrand  and  Saenger.®^  The 
patient,  in  addition  to  cramp  of  the  levator,  was  affected  with  a  cramp- 
like contraction  of  the  inferior  rectus,  which  appeared  when  the  gaze 
was  directed  below  and  to  the  right.  Parinaud  has  observed  the  simu- 
lation of  Graefe's  sign  in  exophthalmic  goitre  by  contracture  of  the 
levator ;  intermittent  retraction  of  the  lids  appeared  even  when  the  eyes 
were  at  rest.  Carl  Kunn  ^^  has  described  cramp  of  the  levator  which 
caused  decided  elevation  of  the  upper  lid  and  widening  of  the  palpebral 
fissure. 


"  For  further  discussion  of  this  subject,  see  Gilles  de  la  Tourette,  Traits  de 
I'Hyst^rie,  I.  partie,  1891,  p.  415. 

"  Encyklopadie  der  Augenheilk.,  Lieferung  10,  Leipzig,  1904,  p.  417. 
"  Die  Neurologic  des  Auges,  Bd.  i.,  Wiesbaden,  1900,  p.  71. 
•*  Deutschmann's  BeitrHge  zur  Augenheilkunde,  iii.  1898. 


LEUKOSES  AXD  PSYCHOSES 


655 


4.  Hysterical   Paralysis   of  the   Levator,   or   Flaccid   Hysterical 

Ptosis. — As  is  well  known,  the  existence  of  hysterical  ptosis  in  the 
form  of  a  flaccid  relaxation  of  the  levator  was  denied  by  Charcot  and  is 
doubted  by  Borel  and  Gilles  de  la  Tourette.  Their  contention  is  that 
hysterical  ptosis,  as  already  noted,  is  due  to  spasm  of  the  orbicularis 
and  not  to  paralysis  of  the  levator.  On  the  other  hand,  equally  capable 
observers,  notably  Schmidt-Rimpler,  Kempner,  Hitzig,  Kieman,  and 
Wilbrand,^^  have  reported  flaccid  ptosis  in  hysterical  subjects  corre- 
sponding completely  to  the  clinical  picture  of  true  paralysis  of  the 
levator  (Fig.  11).  Abadie  has  reported  paralytic  ptosis  of  intermittent 
form  and  of  hysterical  origin. 


Fig.  11.— Flaccid  hysterical  ptosis.    (After  Wilbrand  and  Saenger.) 

Undoubtedly  flaccid  ptosis,  as  compared  with  pseudoptosis,  is  rare. 
Usually  bilateral,  it  has  also  been  observed  by  Schmidt-Rimpler,  Wil- 
brand, and  myself  as  a  unilateral  affection.  It  may  appear  suddenly  or 
develop  gradually,  and  is  usually  seen  in  those  individuals  who  exhibit 
other  well-marked  hysterical  phenomena, — for  example,  contractures  of 
the  bulbar  eye  muscles,  amblyopia,  and  amaurosis,  visual  field  restric- 


"*  For  a  discussion  of  the  literature  of  this  subject,  see  Wilbrand,  Archiv  f.  Augen- 
heilk.,  xxxix.  1899,  p.  172,  and  Wilbrand  and  Saenger,  Die  Neurologie  des  Auges, 
Bd.  i.  pp.  463-477. 


656  THE    EYE    AND    XEKVOUS    SYSTEM 

tion,  and  spasm  of  accommodation.  It  may  be  caused  by  local  irritation 
or  be  one  of  the  symptoms  of  an  attack. 

Schmidt-Rimpler  regards  the  condition  as  a  simple,  often  voluntary, 
relaxation  of  the  levator,  exactly  as  it  occurs  in  sleep,  while  Hitzig 
ascribes  it  to  an  actual  paralysis.  If  the  development  has  been  grad- 
ual an  autosuggestive  origin  may  be  ascertained. 

It  is  conceivable  that  simulation,  even  of  unilateral  flaccid  ptosis,  is 
possible,  although  on  this  point  Wilbrand  and  Saenger  have  expressed 
serious  doubts. 

A  differential  diagnosis  between  flaccid  hysterical  ptosis  and  some  of 
the  forms  of  pseudoptosis  is  often  difficult,  and  those  interested  in  the 
literature  and  comparison  of  the  cases  should  consult  Wilbrand  and 
Saenger. ^^  The  similarity  of  hysterical  ptosis  and  the  ptosis  of  organic 
disease, — for  example,  that  of  ataxia,  multiple  sclerosis,  bulbar  palsy, 
and  even  meningitis, — is  often  striking.  To  distinguish  them  Landolt 
proceeds  as  follows:  The  subject  of  ptosis  is  seated  in  front  of  the 
observer  and  commanded  sharply  to  look  at  one  of  his  eyes.  To  accom- 
plish this  the  patient  inclines  the  head  strongly  backward,  so  that  the 
gaze  may  pass  under  the  drooping  lids.  The  observer  now  puts  his  hand 
on  the  top  of  the  patient's  head,  who  continues  to  regard  the  same  fixa- 
tion point,  and  insensibly  raises  and  even  inclines  it  gradually  forward. 
If  the  pupils  of  the  subject  remain  uncovered  during  this  operation, 
hysteria  may  be  suspected,  because  to  effect  this  the  lids  would  have  to 
raise  themselves  with  the  eyes  while  the  face  is  lowered.  In  other 
words,  as  Landolt  expresses  it,  to  keep  the  eyes  open,  whilst  the  head  is 
inclined  forward  is  equivalent  to  raising  the  eyes  and  lids  when  the 
head  is  straight,  a  thing  absolutely  impossible  in  paralysis  of  the 
levator,  in  which  the  patient  ceases  to  see  the  object  looked  at  as  soon  as 
the  head  leaves  the  compensating  position,  because  the  lids  immediately 
cover  the  pupil. 

Whether  the  so-called  morning  ptosis  of  Gowers,  the  night  ptosis  of 
Weir  Mitchell,  should  be  classed  as  a  variety  of  flaccid  ptosis  is  some- 
what doubtful.  This  phenomenon  will  be  referred  to  again  in  the 
section  on  neurasthenia,  and  may  be  dismissed  now  with  the  statement 
that  certain  nervous  women,  particularly  on  awakening  in  the  morn- 
ing, may  for  some  minutes  find  it  impossible  to  raise  their  eyelids  with- 
out assisting  the  action  of  the  levator  with  their  fingers. 

The  hysterical  anomalies  of  the  external  eye  muscles — that  is,  of  the 
bulbus  musculature — next  claim  attention. 


Loc.  cit.,  pp.  463-477. 


NEUROSES  AND  PSYCHOSES  657 


I.— ANOMALIES   OF   CONVERGENCE. 

(a)  Coijvergence-Insufficiency  and  Convergence-Paralysis. — The  asso- 
ciation in  hysteria  of  insufficiency  of  convergence  and  weakness  of  the 
amplitude  of  accommodation  has  been  referred  to.  Owing  to  the  inti- 
mate relation  existing  between  accommodation  and  convergence,  if  the 
patient  makes  but  feeble  efforts  of  accommodation,  or,  more  accurately, 
is  unable  to  make  but  feeble  efforts  of  accommodation  when  looking 
at  a  near-point,  a  tendency  to  diminution  of  the  convergence-action  will 
appear,  resulting  in  convergence-insufficiency,  or,  as  it  is  sometimes 
called,  a  relative  insufficiency  of  the  fntemi.  Ordinarily,  under  nor- 
mal conditions,  the  amplitude  of  convergence  is  equivalent  to  the  differ- 
ence between  the  maximum  and  the  minimum  of  convergence,  or,  stating 
the  proposition  in  metre  angles,  the  normal  average  of  maximum  con- 
vergence is  9.5  metre  angles  and  the  average  minimum  of  convergence 
is  — 1  metre  angle,  the  amplitude  of  convergence  being,  therefore,  10.5 
metre  angles.  In  hysteria  this  amplitude  of  convergence  is  frequently 
considerably  reduced  at  both  extremities.  In  other  words,  the  far-point 
of  convergence  is  brought  nearer  to  the  patient,  while  the  near-point  is 
farther  removed  from  him,  the  anomaly,  as  Parinaud  has  shown,  pre- 
senting the  same  characteristics  as  those  which  pertain  to  hysterical 
disorders  of  accommodation,  as  described  on  page  29.  In  order  to 
determine  a  condition  of  this  kind,  the  ordinary  tests  with  prisms  are 
required,  diplopia  not  being  spontaneously  present.  Exophoria  for 
distance  may  be  slight  or  absent,  but  is  present  at  the  near-point. 
The  convergence  near-point  usually  is  less  than  three  inches  from  the 
nose  and  may  be  removed  to  many  inches  from  that  point.  Some- 
times the  patient  is  able  to  converge  on  an  object, — for  example,  a 
pencil-point, — when  brought  close  to  the  eyes,  in  excessive  degree,  but 
can  maintain  this  convergence  only  momentarily. 

The  convergence-insufficiency  may  be  so  great  that  it  constitutes  a 
convergence-paralysis,  a  condition  which  has  been  observed  in  hysteria 
by  Parinaud,  Borel,  and  Schweigger.  Although  either  eye  can  be 
moved  inward  to  a  normal  degree  when  the  other  eye  moves  outward,  its 
internal  rectus  does  not  respond  to  the  impulse  of  convergence,  and  the 
convergence  near-point,  in  place  of  being  a  few  inches  from  the  eyes,  is 
removed  to  a  point  one  or  two  metres  from  the  eyes,  and  if  the  test- 
object  is  advanced  within  this  point,  insuperable  crossed  diplopia  at 
once  becomes  manifest.  A  convergence-insufficiency  depending  upon 
structural  or  insertional  anomalies  of  the  interni,  which  has  resulted  in 
the  development  of  a  hysterical  convergence-paralysis,  may  by  operative 
42- 


658  THE    EYE    AND    NERVOUS    SYSTEM 

interference, — for  example,  a  tenotomy  of  the  externiis, — be  followed,  as 
Schwarz  and  Borel  have  shown,  by  sharp  convergence-excess  or  spasm, 
which  in  its  turn  may  be  cured  by  suggestion.  • 

(6)  Convergence-Excess  and  Divergence-Paralysis.. — An  abnormal  tend- 
ing inward  of  the  visual  lines  may  depend  upon  excessive  convergence,  or 
deficient  divergence,  or  upon  these  conditions  combined.®^ 

Just  as  a  convergence-insufficiency  may  be  replaced  by  an  actual 
convergence-paralysis,  so  also  a  divergence-insufficiency  may  be  replaced, 
as  Parinaud,  Uhthoff,  Straub,  von  Hippel,  and  many  other  observers 
have  noticed,  by  an  actual  divergence-paralysis.  This  condition  mani- 
fests itself  by  homonymous  diplofiia  and  convergent  strabismus  when  the 
eyes  are  fixed  upon  a  distant  point.  As  the  test-object  approaches  the 
patient,  and  especially  on  lateral  fixation,  there  is  diminution  of  the 
convergent  strabismus  and  of  the  diplopia,  and  finally  a  point  is  reached 
at  one  or  two  metres  from  the  eye  at  which  single  vision  and  orthophoria 
are  developed,  but  within  this  limit  there  is  exophoria  and  crossed 
diplopia.  Now  this  diplopia  is  peculiar  in  that,  no  matter  whether  it  is 
homonymous  on  the  distal  side  of  the  point  at  which  single  vision  is 
evident,  or  whether  it  is  crossed  on  the  proximal  side  of  this  point,  the 
images  do  not  separate  to  any  perceptible  degree  when  the  test-object  is 
moved  in  the  lateral  direction  from  side  to  side.  According  to  Pari- 
naud, in  some  cases,  although  it  is  possible  to  obtain  fusion  at  the  point 
already  indicated,  in  others  such  fusion  is  impossible,  and  the  crossed 
diplopia  suddenly  becomes  homonymous  diplopia.  There  is,  to  use  his 
phraseology,  a  horror  of  binocular  single  vision.  This  hysterical  di- 
vergence-paralysis is  due  to  spasm  of  convergence  and  not  to  paresis  of 
divergence.  It  depends,  as  Kunn  remarks,  on  cramp  of  the  intemi  with 
at^'pical  diplopia. 

It  therefore  becomes  evident  that  in  hysterical  patients  all  of  the 
factors  connected  M'ith  convergence  may  be  defective,  accommodation, 
fusion,  and  probably  also  "  the  sense  of  convergence"  (Parinaud,  Han- 
sen Grut),  and  we  may  have  an  absolute  or  a  relative  weakness  of  the 
adductors  or  a  disturbance  of  central  origin,  which  creates  a  weakness 
or  a  total  disappearance  of  the  power  of  fusion. 

We  have  already  seen,  on  page  27,  that  painful,  hysterical  visual 
sensations  are  largely  connected  with  the  anomalies  of  accommodation. 
Necessarily,  however,  as  these  anomalies  are  associated  with  those  of 
convergence  just  detailed,  the  combination  produces  the  highest  type  of 
so-called  hysterical  asthenopia,  and  while  it  may  be  true  that  the  defects 

•"  For  the  tests  to  determine  these  conditions,  see  chap.  v. 


NEUROSES  AND  PSYCHOSES  659 

of  accommodation  are  the  predominant  ones,  as  a  rule  both  convergence 
and  accommodation  are  involved,  and  there  arise  photophobia,  retinal 
anaesthesia,  obscuration  of  vision,  headache,  inability  to  maintain  jS.xa- 
tion,  radiating  pain,  not  only  in  the  head  but  extending  to  the  neck, 
shoulders,  and  chest,  all  aggravated  by  an  attempt  to  use  the  eyes,  but 
often  present  without  ocular  efforts  at  close  ranges. 

Koenigshofer  ^"^  thinks  that  in  one  class  of  cases  a  characteristic  con- 
dition is  that  the  patients  cannot  fix  any  one  object,  no  matter  at  what 
distance  from  it  they  are.  As  soon  as  the  attempt  is  made,  all  objects 
swim  before  their  eyes,  and  vertigo  and  nausea  are  predominant  signs. 
These  patients  direct  their  visual  axes  and  accommodation  upon  a 
definite  distance,  so  that  fixation  upon  a  nearer  or  further  point  is  not 
possible,  or  possible  only  to  a  limited  degree.  For  this  form  of  muscular 
asthenopia  he  wishes  to  suggest  the  term  "  convergence  immobility" 
(  Convergenzstarre  ) . 

(c)  Convergence-cramp  or  Contracture,  and  Hysterical  Convergent  Stra- 
bismus.— We  have  seen  in  the  preceding  paragraphs  that  in  hysteria 
there  may  be  convergence-excess,  although  it  should  not  necessarily  be 
regarded  as  an  essential  sign  of  the  disease.  What  may  be  characterized 
as  an  advance  on  convergence-excess  receives  the  name  of  convergence- 
cramp,  or  spasm,  which  certainly  is  a  frequent  hysterical  phenomenon, 
and  when  well  developed  manifests  itself  by  inward — that  is,  con- 
vergent— strabismus  and  is  usually  associated  with  spasm  of  accommo- 
dation. 

The  idea  of  spastic  strabismus  as  part  of  the  symptomatology  of 
hysteria  dates  to  the  observation  of  Landouzy,  who  remarks  that  in  the 
course  of  hysterical  attacks  we  may  notice  a  deviation  of  the  eyes,  to 
which  some  pathologists  have  applied  the  term  hysterical  strabismus. 
The  earlier  literature  of  this  subject  has  been  analyzed  by  Gilles  de  la 
Tourette,^^  to.  whose  treatise  those  interested  should  turn.  In  more 
recent  times  detailed  description  of  spastic  hysterical  strabismus  will  be 
found  in  the  work  of  Borel,^^  and  particularly  good  reports  of  this  con- 
dition have  been  recorded  by  Manz,  Schweigger,  Ulrich,  and  Nonne  and 
Beselin. 

We  have  already  seen  that  contracture  of  convergence,  or  so*called 
spastic  strabismus,  not  infrequently  accompanies  blepharospasm,  and 
have  also  noted  that  a  condition  of  this  character  may  be  seen  in  asso- 


Ophthalmologische  Klinik,  1902,  vi.  p.  113. 
'  Loc.  cit. 
Archives  d'Ophthalmologie,  1886-1887,  and  Annales  d'Oculistique,   1897-1898. 


660  THE    EYE    AXD    l^ERVOUS    SYSTEM 

ciation  with  hysterical  ptosis, — for  example,  a  rotation  of  the  eyeballs 
downward  and  inward,  as  in  Hitzig's  case,  followed  later  by  convergent 
strabismus ;  inward  and  upward,  as  in  a  case  reported  by  Wilbrand  and 
Saenger;  or  into  a  position  of  permanent  convergence,  which  was, 
indeed,  never  entirely  overcome,  as  recorded  by  Oppenheim. 

Convergence-spasm  and  spastic  strabismus,  unaccompanied  by  anom- 
alies of  lid  movement,  in  the  form  of  blepharospasm  or  ptosis,  occur 
and  are  characterized  by  convergent  squint,  limitation  of  the  motility  of 
the  external  recti,  and  by  homonymous  diplopia.  The  condition  resem- 
bles paralysis  of  the  abducens,  for  which  it  may  be,  indeed  has  been, 
mistaken.  But,  under  these  circumstances,  double  images  do  not  be- 
have as  they  would  in  a  paralji;ic  case.  They  have,  as  Ulrich  expresses 
it,  the  same  separation  on  looking  to  the  right  as  when  the  patient  looks 
to  the  left,  a  peculiarity  which  was  exceedingly  marked  in  a  case  re- 
cently under  my  own  care,  in  which,  with  every  attempt  at  fixation, 
there  was  excessive  convergence  and  marked  limitation  of  the  external 
recti  movements,  but  no  alteration  in  the  apparent  distance  between  the 
double  images,  no  matter  to  what  portion  of  the  lateral  field  of  fixation 
they  were  moved.  Schwarz  insists  that  strict  attention  should  be  paid 
to  the  secondary  deviation.  If  this  is  not  greater  than  the  primary  de- 
viation of  the  squinting  eye,  the  condition  is  probably  not  one  of  sixth- 
nerve  palsy,  especially  as  one  is  usually  able  to  establish  in  an  eye  of 
this  character  spastic  myosis,  spasm  pf  accommodation,  micropsia,  and 
sometimes  monocular  diplopia.  Sometimes,  however,  the  diagnosis  is 
extremely  difficult,  as  Schwarz  himself  admits,  as  is  evident  from  an 
examination  of  the  case  reported  by  Nonne  and  Beselin.  Schwarz 
believes  that  the  only  positive  differential  diagnostic  point  between  a 
convergence-cramp  and  an  abducens  paralysis  is  developed  by  an  ob- 
jective investigation  of  the  optical  apparatus.  In  a  case  of  his  own  he 
was  able  to  determine  by  a  skiascopic  examination  that  in  one  direction 
of  the  gaze  at  which  the  double  images  were  evident  an  accommodation 
of  more  than  2  D.  developed.  Nonne  and  Beselin  point  out  that  in 
spasm,  in  contradistinction  to  palsy,  contractions  of  the  muscles  inner- 
vated by  the  facial,  dread  of  light  and  pain  in  and  around  the  affected 
eye  would  be  apt  to  be  present. 

Hysterical  spastic  strabismus,  or,  more  accurately,  as  Parinaud 
properly  insists,  contracture  of  convergence,  may  occur  as  the  prodrome 
of  an  attack  of  grave  hysteria, '  during  hysterical  attacks  and  at  their 
termination,  but  is,  in  general  terms,  a  transitory  phenomenon,  although 
it  may  terminate  in  some  form  of  deviation, — for  example,  a  conjugate 
deviation, — exactly  as  a  conjugate  deviation  may  sometimes  precede  a 


NEUROSES  A^D  PSYCHOSES  661 

convergent  strabismus.  Such  contracture  of  convergence,  or  strabismus, 
may  arise  in  association  with  other  muscular  movements — as,  for  ex- 
ample, in  Shuster's  patient  with  traumatic  hysteria,  in  whom  when  the 
arm  was  raised  strabismus  developed.  It  can  be  brought  about,  as  other 
hysterical  phenomena,  by  suggestion  and  cured  in  like  manner.  Perma- 
nent convergent  strabismus  in  hysteria  must  not  be  regarded  as  a  stigma 
of  the  disease.  An  investigation  will  always  show  other  factors  upon 
which  this  depends,  notably  hyperopia,  anisometropia,  amblyopia  of  one 
eye,  and  absence  of  the  fusion  sense.  In  a  notable  case  under  my  own 
observation  of  long-standing  major  hysteria,  to  which  reference  will 
again  be  made,  a  prolonged  conjugate  deviation  of  the  eyes  to  the  left 
lasting  for  many  months  was  followed  upon  recovering  by  well-marked 
convergent  strabismus,  occurring,  as  is  usually  the  case  in  hysteria,  in 
crises,  but  finally  settling  to  a  well-marked  moderate  inward  strabismus. 
When  investigation  was  thoroughly  made  a  high  degree  of  hyperopia, 
with  all  of  the  symptoms  of  convergence-excess,  was  found,  and  suit- 
able glasses  produced  parallelism  of  the  visual  axes. 

While  it  is  true  that  in  the  convergent  strabismus  of  the  character 
which  has  been  described  as  hysterical  there  may  be  limitation  of  the 
outward  movements  of  the  eyes,  it  should  be  remembered  that  this  is 
not  always  the  case,  and  that  one  sign  in  the  minor  degrees  of  con- 
vergence-contracture  which  helps  to  differentiate  it  from  an  abducens 
palsy  is  that  the  field  of  fixation  is  normal  in  the  one  and  naturally 
limited  in  the  other.  Wlien  the  convergence  contracture  is  excessive, 
then  this  test  cannot  be  applied,  although  it  does  not  in  the  least  destroy 
the  very  evident  etiology  of  the  affection  as  an  anomaly  of  convergence 
innervation. 

Spasmodic  Conjugate  Deviation. — In  discussing  spastic  strabismus  in 
hysteria,  we  have  seen  that  there  may  be  a  spasm  or  cramp  of  an  indi- 
vidual muscle,  or  in  association  with  blepharospasm,  or  various  types  of 
ptosis,  and  have  noted  in  detail  the  well-marked  hysterical  phenomena 
of  convergence-cramp  and  a  strong  inward  strabismus,  often  combined 
with  spasm  of  accommodation.  We  have  now  to  deal  with  varieties  of 
conjugate  deviation,  in  which  a  spasmodic  condition  of  associated  muscle 
groups  is  evident.  During  clonic  or  tonic  hysterical  convulsions  there 
may  be  marked  contraction  of  the  ocular  muscles  and  with  this  a  coin- 
cident contraction  of  the  muscles  of  the  neck,  giving  the  appearance 
of  a  conjugate  deviation  of  the  head  and  eyes,  or,  again,  under  similar 
conditions,  the  eyes  may  be  turned  in  any  direction  by  what  Pansier 
calls  convulsive  rollings,  without- any  apparent  rule  guiding  the  irregu- 
larity of  the  spastic  rotations.     These  are  not  true  forms  of  spastic 


662  THE    EYE    AND    NERVOUS    SYSTEM 

strabismus.  Both  eyes  execute  incessant  movements  but  are  not  per- 
manently deviated.  They  seem,  to  use  the  language  of  Parinaud,  to 
have  lost  the  power  of  fixation.  Often  these  cases  may  be  complicated 
with  photophobia  and  blepharospasm,  and  there  may  be  lagophthalmos 
owing  to  a  contracture  of  the  levator  palpebral,  as  pointed  out  by  Levy 
and  described  by  Gilles  de  la  Tourette  and  Parinaud.  Such  irregular 
contractures  of  the  ocular  muscles  may  be  accompanied  by  diplopia,  or, 
more  accurately,  by  attacks  of  diplopia,  occurring  sometimes  frequently 
during  a  single  day. 

In  typical  spasmodic  conjugate  deviation  the  contractures  affect  the 
associated  parallel  movements,  or,  as  Parinaud  puts  it,  the  associated 
movements  of  direction,  and  while  it  is  true  that  the  deviations  may  be 
in  any  direction,  according  to  the  same  author,  the  inferior  and  oblique 
position  is  preferred.  As  may  have  been  inferred,  the  condition  is  not 
commonly  a  permanent  one,  occurring  as  it  does  during  seizures,  and 
yet  what  may  be  designated  permanent  deviations  of  well-marked  char- 
acter have  been  recorded.  Notable  in  this  respect  is  the  case  of  Frost,'^^ 
in  which  the  eyes  were  directed  downward  and  to  the  right,  upward 
movements  being  almost  impossible,  and  the  condition  lasted  one  year. 
I  studied  with  Dr.  Wharton  Sinkler,  of  this  city,  a  case  of  major  hys- 
teria in  which  there  was  pronounced  unvarying  conjugate  deviation 
upward  and  to  the  left,  with  lagophthalmos,  owing  to  contracture  of  the 
levator  palpebral  of  each  eye,  which  lasted  for  nine  months.  In  less 
persistent  cases  there  may  be  attacks,  lasting  for  days  or  weeks,  coming 
on  with  other  convulsive  seizures;  or  the  type  may  change — that  is,  a 
conjugate  deviation  in  one  attack  may  become  a  spasm  of  convergence  in 
another,  as  I  myself  have  noted  in  the  case  already  referred  to,  and  as 
has  also  been  reported  by  Manz.  The  combination  of  contractures  of  con- 
vergence with  loss  of  lateral  movement  has  already  been  described,  and 
typical  cases  of  this  character  are  not  uncommonly  seen. 

Ordinarily  the  contractures  upon  which  depend  these  conjugate 
deviations  necessarily  indicate  the  implication  of  both  eyes  simulta- 
neously, but  it  should  be  remembered  that  in  unilateral  blepharospasm 
there  may  be,  when  the  patient  attempts  to  fix  the  affected  eye,  a  prompt 
spasmodic  deviation  upward  and  inward.  This  unilateral  condition  of 
spasm  is  illustrated  in  Goldscheider's  case,  whose  patient  exhibited 
nothing  peculiar  in  the  rotations  of  the  ocular  muscles  until  she  was 
required  to  look  to  the  right  and  below,  when  the  left  bulbus  was  spas- 
modically drawn  downward  and  inward,  while  the  left  upper  lid  was 

"British  Medical  Journal,  1884. 


NEUROSES  AND  PSYCHOSES  663 

elevated.  These  spasms  of  the  ocular  muscles,  to  which  Borel  makes 
full  reference,  have  already  been  discussed  under  spastic  strabismus. 
As  in  other  hysterical  ocular  phenomena,  with  these  conditions  may  be 
present  amblyopia,  photophobia,  and  blepharospasm,  and  they  may  be 
brought  into  existence  by  emotion,  trauma,  intoxication,  or  reflexly  by 
dental  irritation,  and  would  seem  to  be  more  often  found  in  young 
hysterics,  who,  curiously  enough,  apart  from  their  convulsive  seizures, 
may  have  little  marked  other  stigmata  of  hysteria. 

Hysterical  Palsies  of  the  Ocular  Muscles :  Hysterical  Ophthalmo- 
plegia.— (a)  Palsies  of  Individual  Muscles. — Binswanger,"^  referring  to 
this  much  disputed  subject,  gives  a  summary  of  what  he  calls  the  ap- 
proximately authentic  cases  of  hysterical  paralysis  of  the  eye  muscles, 
as  follows:  Paralysis  of  the  left  internal  rectus  and  the  superior  recti 
muscles  on  both  sides  (Guttmann'^^)  ;  paralysis  of  the  right  external 
rectus,  later  of  the  left  (Duchenne'^^)  ;  bilateral  paralysis  of  the  ab- 
ducens  (Leber '^*)  ;  paralysis  of  the  abducens  (Parinaud^^)  ;  paralysis 
of  the  right  and  later  of  the  left  abducens  combined  with  contracture  of 
the  internal  rectus  (Borel  ^®);  ptosis,  paralysis  of  accommodation, 
paralysis  of  the  internal  rectus  and  of  the  inferior  rectus  of  the  right 
eye  (Parinaud  and  Richer '^'^);  paralysis  of  the  abducens,  two  cases 
(Wilbrand  "^)  ;  paralysis  of  the  left  inferior  oblique  (Wilbrand  and 
Saenger,  examined  later  by  Nonne  and  Beselin  '^^ ) .  In  the  last  named 
case  paralysis  of  the  oblique  on  the  left  side  was  made  to  disappear  by 
suggestion,  and  so-called  hysterical  paralysis  of  the  right  superior 
oblique  was  later  observed.  Other  cases,  not  quoted  in  this  summary, 
are  these :  Ptosis,  divergent  strabismus,  and  nystagmus,  especially  occur- 
ring in  children  (Steiner*^)  ;  palsy  of  all  eye  muscles  except  the 
internal  recti  ( Stephenson  ®^ )  ;  double  oculomotor  palsy  of  hystero- 
traumatic  origin  (Debove*^)  ;  ptosis  with  complete  fixation  of  the  left 
eyeball  (Cowan  ^^).     Schiifer's  assertion  that  oculomotor  palsy  is  fre- 

"  Loc.  cit.,  p.  389. 

"  Berlin,  klin.  Wochenschr.,  1869. 

"Gaz.  des  Hop.,  1875. 

"  Graefe  u.  Saemische,  Handbuch  der  gesammten  Augenheilk.,  I.  Aufl.,  v.  p.  985. 

"  Soc.  d'Ophthal.  frangaise,  1886. 

^'Arch.  d'Opth.,  1886. 

"  Binswanger,  loc.  cit. 

^'  Jahrb.  der  Hamburger  Staats-Kranken-Anstalten,  1890. 

^'  Binswanger,  loc.  cit. 

^  Jahresbericht  f.  Ophthal.,  xxix.  1898,  p.  567. 

^  British  Medical  Journal,  1902,  i.  p.  205. 

*^  Recueil   d'Ophthalmologie,   xiii. '1891,   p.    124. 

*^Xew  York  Eye  and  Ear  Infirmary  Reports,  January,  1901. 


664  THE    EYE    AND    NERVOUS    SYSTEM 

quent  in  children  has  not  been  confirmed,  and  Binswanger  regards  it  as 
erroneous.  It  is  hardly  necessary  in  an  article  of  this  character — indeed, 
space  does  not  permit — to  analyze  the  case  histories.  Such  analyses 
have  been  well  made  by  Binswanger,  Mobius,  Parinaud,  Kunn,  and 
many  others,  and  it  would  seem  to  be  settled  with  a  fair  amount  of 
definite  accuracy  that,  as  Koenig  has  said,  thus  far  it  does  not  seem 
that  any  true  case  of  hysterical  eye-muscle  palsy  has  been  published. 

Furthermore,  as  Gilles  de  la  Tourette  has  expressed  it,  we  may 
suggest  to  a  patient  palsy,  but  cramp  or  contracture  always  appears. 
Parinaud,  to  whom  we  owe  so  much  for  his  accurate  study  of  hysterical 
cases,  and  who  has  himself  reported  palsy  of  ocular  muscles  in  hysterical 
patients,  remarks :  Paralysis  of  the  third  pair  and  contracture  limited 
to  one  muscle,  or  to  the  territory  supplied  by  one  nerve,  probably  do  not 
exist  in  hysteria,  but  he  further  goes  on  to  say,  as  hysteria  is  a  disease 
continually  presenting  new  phases,  it  is  prudent  to  make  a  reservation 
that  such  a  condition  may  have  been  observed  and  implies  that  it  may  in 
the  future  be  observed.  He  quotes  Samelsohn  as  having  seen  one  case  of 
hysterical  paralysis  of  the  sixth  pair,  but  disputes  Borel's  case  .  of 
abducens  paralysis,  which  he  thinks  on  careful  analysis  indicates  a  con- 
dition to  be  explained  by  the  symptom  already  so  much  dwelt  upon, 
namely,  contracture  of  convergence.  He  feels  sure  that  any  case  of 
well-marked  paralysis  of  the  third  pair  in  a  hysterical  patient — for 
example,  one  that  he  himself  has  reported  which  he  observed  for  a 
number  of  years — should  give  rise  to  the  suspicion  of  another  influence 
and  of  the  coexistence  of  two  separate  diseases. 

How,  then,  are  these  conditions  to  be  explained  ?  Binswanger  holds 
that  the  so-called  hysterical  paralyses  of  the  eye  muscles  always  indi- 
cate a  defect  of  the  associated  lateral  movements,  or  a  motor  insuffi- 
ciency of  certain  muscular  movements  closely  connected  with  psychical 
processes,  and  sometimes  a  combination  of  paretic  and  spastic  motor 
disturbances  pecviliar  to  hysteria.  I  have  never  seen,  in  a  very  large 
experience  in  hysterical  patients,  any  isolated  paralysis  of  an  eye  muscle, 
and  believe  with  Parinaud  and  other  writers  that  the  so-called  hysterical 
palsies  should  be  explained  by  what  is  sometimes  described  as  an  asso- 
ciated ocular  paralysis, — that  is,  although  the  eyes  cannot  make  certain 
movements  in  which  they  are  usually  associated,  the  directing  power  of 
the  muscles  may  be  unimpaired  when  they  exercise  their  function  in  a 
different  direction.  In  other  words,  there  is  a  paralysis  of  movement 
and  not  of  the  muscles  supplied  by  a  given  nerve.    We  have,  then, — 

(h)  Palsy  of  associated  parallel  movements,  or,  in  other  words,  the 
Blickltihmung  of  the   Germans.      Thus,   as,    for  example,    in   a   case 


NEUROSES  AND  PSYCHOSES  665 

of  Claude's,  there  may  be  inability  of  the  eyes  to  move  to  the  left, — 
that  is,  loss  of  the  directing  power  of  the  right  internns  and  of  the  left 
externus,  which  are  associated  antagonists ;  there  is  loss  of  Isevo- 
version,  using  Duane's  phraseology.  In  the  same  way  there  may  be 
loss  of  dextroversion,  and  so  on,  of  the  several  different  associated 
parallel  movements. 

(c)  Hysterical  ophthalmoplegia,  many  cases  of  which  are  on  record,  as 
Parinaud  has  well  pointed  out,  is  characterized  essentially  by  the  fact 
that  the  voluntary  eye-muscle  movements  are  affected,  and  the  patient 
cannot  turn  or  rotate  the  eye  to  any  designated  point,  although  the  same 
movement  can  usually  with  ease  be  involuntarily  accomplished.  The 
condition  has  been  compared  to  the  characteristics  of  mobility  of  the 
lower  extremity  in  hysterical  abasia.  For  example,  at  present  I  am 
studying  with  Dr.  Wharton  Sinkler,  of  this  city,  a  case  of  pronounced 
hysteria  in  which  the  patient  cannot  raise  the  eyes  in  the  least  above 
the  horizontal  level  when  directed  to  do  so,  although  she  is  perfectly 
able  when  her  attention  is  directed  momentarily  away  from  the  thought 
of  upward  rotation  to  make  this  movement.  A  similar  associated 
paralysis  of  the  superior  recti  muscles  is  well  described  by  Nogues  and 
Sirol.^*  If  the  upper  lids  are  raised,  the  upward  rotation  of  the  globe 
above  the  horizontal  plane  is  impossible,  although  all  other  associated 
movements  are  preserved.  The  movement  of  elevation,  which  cannot  be 
excluded  when  the  lids  are  elevated,  can  be  made  when  the  lids  are 
closed.  It  is  not  necessary  to  multiply  instances.  Even  in  cases  of 
conjugate  deviation  it  is  possible — as,  for  example,  it  was  in  Frost's 
case,  in  which  both  eyes  were  directed  downward  and  to  the  right — to 
make  the  one  eye  fix  perfectly  and  follow  the  movements  in  all  direc- 
tions when  the  other  eye  was  closed,  and  the  same  observation  has  been 
made  by  Parinaud  in  one  of  his  patients.  Furthermore,  these  so-called 
palsies  disappear  during  chloroformization. 

Cases  of  hysterical  ophthalmoplegia  have  been  described — for  ex- 
ample, by  Ballet,  Warner,  Bristowe,  and  others — in  hysterical  patients 
the  subjects  of  exophthalmic  goitre,  but  even  here — as,  for  example,  in 
Ballet's  case,  although  there  was  complete  inability  to  move  the  eyes, 
which  were  normally  directed — automatic  movements  were  possible. 
Eaymond  and  Koenig,  referring  to  complete  hysterical  ophthalmoplegia 
characterized  by  complete  loss  of  voluntary  movement,  decline  to  regard 
them  as  true  paralyses  and  insist  on  a  differential  diagnostic  point 
between  this  condition  and  a  genuine  ophthalmoplegia  in  the  absence  of 

**Annales  d'Oculistique,  cxxxv.  1901,  p.  360. 


666  THE    EYE    AND    NERVOUS    SYSTEM 

mydriasis.  Nonne  and  Beselin  quote  cases  of  palsy  of  the  external  eye 
muscles  lasting  for  several  days,  accommodation  and  pupillary  func- 
tions being  normal.  These  may  be  explained  by  assuming  a  transitory 
spasm  of  the  external  eye  muscles,  which,  according  to  Gilles  de  la 
Tourette,  are  common  in  hysteria  and  may  produce,  as  has  already 
been  pointed  out,  temporary  attacks  of  diplopia. 

(d)  Dissociation  of  the  Eye  Movements. — According  to  Kunn,®'  many 
cases  of  so-called  palsy  and  cramp  of  the  eye  muscles  in  hysteria  are 
really  dissociations  of  the  eye  movements.  This  condition  is  character- 
ized, according  to  him,  not  by  a  lasting  and  constant  limitation  of 
movement,  but  by  a  loss  of  movements  or  rotations  that  were  formerly 
associated,  and  takes  place  in  both  eyes,  so  that  exactly  in  the  atypical 
and  changing  condition  of  the  rotation  disturbances  the  characteristic 
symptom  of  the  condition  is  contained.  Borel  has  described  in  hysterical 
patients,  and  by  ingenious  experimentation  proved,  a  loss  of  the  muscu- 
lar sense  of  the  eyes. 

Hysterical  Nystagmus.. — Binswanger  maintains  that  a  genuine  hyster- 
ical nystagmus  lasting  for  some  time  and  occurring  independently  of 
hysterical  paroxysms  does  not  occur.  Pansier  remarks  that  just  as  nys- 
tagmus is  frequent  in  epilepsy  it  is  rare  in  hysteria.  He  quotes  Rouffi- 
net  as  attributing  to  it  the  following  characters :  It  is  mixed, — -that  is 
to  say,  it  exists  as  well  in  a  state  of  fixation  as  in  a  state  of  non-fixation ; 
it  is  unaccompanied  by  refractive  errors;  it  is  permanent  and  its  in- 
tensity can  diminish  with  the  disappearance  or  the  diminution  of  the 
crisis ;  but  it  does  not  disappear  completely  and  differs  from  that  seen 
in  multiple  sclerosis,  inasmuch  as  the  latter  is  exaggerated  by  the  move- 
ments of  the  ocular  globes.  As  is  well  known,  Charcot  insisted  that 
nystagmus  served  as  a  differential  diagnostic  symptom  between  dis- 
seminated sclerosis  and  hysterical  affections,  being  present  in  the  former 
and  absent  in  the  latter.  Sabrazes  thinks  he  has  proved  that  nystagmus 
should  be  regarded  as  a  possible  hysterical  manifestation  because  of  the 
efficacy  of  suggestion  in  one  of  his  cases.  This  inhibited  the  nystagmic 
movements,  and  there  were  in  addition  the  presence  of  other  hysterical 
phenomena.  Oscillation  or  nystagmic  movements  of  the  eye  may  be 
produced  by  hypnotic  suggestion  in  hysterical  patients.  A  rapid  oscilla- 
tion of  the  eyeball,  either  in  a  horizontal  or  oblique  direction,  synchro- 
nous with  contractions  of  the  orbicularis  when  resistance  is  offered  to  its 
contraction  with  the  fingers,  has  been  recorded  by  Stransky  and  is 
described  by  him  as  an  associated  nystagmus.  It  occurs,  according  to 
. « 

"  Deutschmann's  Beitrage  f.  Augenheilk.,  vol.  iii.  1898. 


NEUROSES  AXD  PSYCHOSES  667 

this  author,  in  neurotic  individuals.  Santos  Fernandez  ®^  has  reported 
hysterical  nystagmus.  The  rapid  and  sometimes  bizarre  movements 
of  the  eyeballs  which  occur  during  hysterical  attacks,  and  to  which  refer- 
ence has  already  been  made,  are  well  known  and  cannot  be  regarded  as 
examples  of  true  nystagmus.     (Compare  Chapters  V  and  IX.) 

VII.    PREVIOUSLY    UNCLASSIFIED    HYSTERICAL 
OCULAR  PHENOMENA, 

1.  Disturbances  of  Sensation — Briquet  considered  anaesthesia  of 
the  conjunctiva,  especially  of  the  left  side,  as  a  condition  so  constantly 
present  in  hysteria  that  it  might  be  regarded  as  characteristic.  Schwarz, 
however,  maintains  that  conjunctival  and  corneal  anaesthesia  is  not 
peculiar  to  hysteria,  but  is  seen  in  pure  neurasthenic  cases.  This  is 
contrary  to  my  exj)erience.  The  entire  conjunctival  expansion  may  be 
insensitive,  or  the  anaesthesia  may  be  confined  to  certain  areas,  par- 
ticularly to  an  area  below  the  cornea.  The  tarsal  conjunctiva  is  less 
frequently  anaesthetic  than  the  bulbar  mucous  membrane.  The  cornea, 
more  uncommonly  than  the  conjunctiva,  is  involved  in  anaesthesia. 
According  to  Gilles  de  la  Tourette,  this  is  usually  incomplete,  the 
centre  of  the  cornea  retaining  its  sensibility.  Fere,  as  already  noted, 
believed  that  hysterics  with  hemianaesthesia  unassociated  with  restriction 
of  the  visual  field  and  achromatopsia  did  not  lose  the  sensibility  of  their 
conjunctivas,  but  if  they  had  dyschromatopsia  and  concentric  contrac- 
tion of  the  field  of  vision  the  conjunctiva  would  be  anaesthetic,  while  if 
there  was  achromatopsia  and  almost  complete  loss  of  indirect  vision, 
both  the  conjunctiva  and  cornea  would  be  insensitive.  This  observation 
has  not  been  confirmed  by  other  observers.  The  semeiological  value  of 
corneal  and  conjunctival  anaesthesia  in  hysteria  has  recently  been  dis- 
cussed by  E.obinau.*''^ 

In  moderate  grades  of  conjunctival  anaesthesia  the  palpebral  reflex  is 
retained,  while  in  higher  grades  it  is  lowered  or  lost.  It  may  be  lost,  too, 
if  the  cornea,  being  anaesthetic,  is  touched,  but  if  the  test-object  comes 
into  the  field  of  the  pupil  there  is  a  response.  This,  however,  is  not 
due  to  contact,  but  is  a  retinal  lid-closure  reflex.  The  lachrymal  reflex 
is  retained  even  when  the  conjunctiva  is  wholly  insensitive,  as  has  been 
shown  by  Pitres,  Gilles  de  la  Tourette,  Spiller,  and  others.  This  preser- 
vation of  the  glandular  reflex,  as  Spiller  suggests,  may  serve  to  dis- 
tinguish hysterical  from  organic  anaesthesia.  In  place  of  anaesthesia  of 
the  conjunctiva  and  cornea  there  may  be  Jiypercesthesia  of  these  mem- 

^  Clin.  Opht.,  Paris,  1904,  x.  345. 
*'  Bordeaux  These,  1904. 


668  THE    EYE    AND    NEKVOUS    SYSTEM 

branes  aud  often  associated  with  it  amblyopia,  laehrymation,  photo- 
phobia, and  orbicularis  spasm. 

Anapsthesia  of  the  skin  of  the  lids  and  surrounding  cutaneous  sur- 
face is  also  present  in  hysterics,  but  not  so  frequently  as  the  same  con- 
dition of  the  oculo-mucous  membrane.  As  before  noted,  Gilles  de  la 
Tourette  has  described  a  periorbital  zone  of  anaesthesia  in  non-painful 
hysterical  blepharospasm,  which  in  the  painful  variety  of  the  affection  is 
replaced  by  a  similar  hypersesthetic  area. 

Spots  sensitive  to  pressure  in  the  neighborhood  of  the  eyes  are 
common  in  hysteria,  especially  along  the  inferior  orbital  border.  These 
tender  points  Schwarz  considers  to  be  quite  characteristic  of  hysteria  in 
the  absence  of  neuralgia,  and  if  they  do  not  represent  the  position 
of  a  nerve-trunk  or  one  of  its  points  of  exit. 

Hysterogenetic  zones  in  and  around  the  eye — for  example,  in  the 
infra-  and  supraorbital  region — ^have  been  described,  and  Lichtwitz  has 
recorded  a  case  where  the  conjunctiva,  cornea,  and  lachrymal  canals 
were  occupied  by  hypnogenic,  letharogenic,  and  spasmogenic  zones,  inter- 
ference with  which  produced  conditions  indicated  by  their  descriptive 
names. 

2.  Disturbances  of  Secretion. — Excessive  secretion  of  tears,  some- 
times paroxysmal  in  nature,  is  frequent  in  hysterical  patients,  and  is 
sometimes  called  hysterical  epiphora.  It  is  often  accompanied  by 
spasm  of  the  orbicularis.  Bloody  tears — i.e.,  bleeding  from  the  outer 
angles  of  the  eyes — and  the  so-called  vicarious  menstruation  have  been 
described  by  Hensinger  and  others  in  subjects  of  hysteria  (page  342). 

Chromidrosis,  which  consists  of  a  bluish-black  discoloration  of  the 
lids,  somewhat  oleaginous  in  nature,  and  which  can  be  wiped  away,  is 
occasionally  encountered  in  hysterical  subjects,  but  is  not  necessarily  a 
stigma  of  the  disease.  Hysterical  chromidrosis  lasting  for  months  has 
been  described  by  Putnam.  Severe  cases  of  oily  seborrhoea  have  been 
reported  in  which  the  dusky  secretion  has  been  allowed  to  accumulate 
for  several  years,  and  in  a  remarkable  example  of  this  nature  recorded 
by  Van  Harlingen  ^^  a  thick  black  mask  of  the  face,  covering  the  fore- 
head and  nose,  had  been  allowed  to  form. 

3.  Chromatopsia. — Strzeminski  ^^  has  reported  eryihropsia  as  a 
rare  phenomenon  in  hysteria  and  refers  to  similar  observations  by  Hil- 
bert.  Fano  had  a  patient  who  saw  in  the  field  of  vision  spots  of  color, 
and  Meirowitz  ^^  one  who  when  looking  into  the  air  detected  colored 

"•American  Journal  of  the  Medical  Sciences,  cxxvii.,  No.  2,  1904,  p.  245. 
*"  Recueil  d'Ophthalmologie,  3  Series,  1902,  xxiv.  p.  464. 
■"Journal  of  Nervous  and  Mental  Diseases,  xxvii.,  1900,  p.  286. 


NEUROSES  Al^B  PSYCHOSES  669 

concentric  rings,  processions  of  blue  commas  surrounded  by  green  spots, 
while  objects  and  persons  were  often  surrounded  by  colored  halos.  I 
have  recorded  ^^  a  case  of  hysterical  amblyopia  associated  with  toxic 
amblyopia  in  which  a  marked  symptom  was  xanthopsia.  Ordinary  non- 
chromatic  visual  hallucinations  are  met  with  in  hysterics.  Binet  and 
others  have  shown  that  tactile  sensations  may  be  transformed  in  visual 
sensations  in  hysterical  subjects. 

4.  Organic  Changes. — An  essential  condition  of  hysterical  ambly- 
opia and  amaurosis  is  the  absence  of  organic  lesions  in  the  fundus  of 
the  eyes.  Hyperaisthesia  of  the  retina  may  be  present ;  enlargement  of 
the  retinal  veins  and  serous  exudate  into  the  retina  have  been  recorded 
by  Landolt.  Leber  and  Xuel  have  described  hyperaemia  of  the  retina  and 
papilla,  and  a  gray  discoloration  of  the  nerve-head  which  may  eventuate 
in  atrophy.  But,  as  Nuel  himself  remarks,  in  such  cases  some  other 
agency  should  be  suspected ;  the  hysteria  may  well  have  been  associated 
with  multiple  sclerosis  or  tabes  dorsalis.  The  hysterical  irido-choroiditis 
described  by  Simi  is  open  to  doubt.  Hysterical  amblyopia  may  simulate 
sympathetic  disease,  as  has  been  well  shown  by  Parinaud  and  Kalt.^^ 
The  latter  author  observed  a  young  woman  whose  left  eye  had  been 
destroyed  by  irido-choroiditis.  There  was  depreciation  of  vision  of  the 
right  eye,  restricted  fields  for  blue,  and  red  and  green  achromatopsia. 
All  of  these  symptoms  disappeared  when  the  left  eye  was  enucleated. 
Hapid  opacity  of  the  crystalline  lens  after  an  hysterical  attack  has  been 
reported  by  Masleunikow,^^  He  attributes  the  condition  to  nutritional 
disturbances  caused  by  repeated  cramp  of  the  ocular  muscles.  An  hys- 
terical vasomotor  affection  of  the  conjunctiva  has  been  described  by 
Fromaget  ^"^  in  the  form  of  intermittent  congestion  of  the  bulbar  con- 
junctiva without  inflammatory  phenomena.  Hysterical  oedema  of  the 
lids  is  probably  always  the  result  of  some  inflammatory  condition  of 
the  conjunctiva  or  ciliary  margin,  and  not  an  essential  symptom  of  the 
disease.  Some  hysterics  purposely  bring  irritating  substances  in  contact 
with  their  eyes  to  cause  congestion  and  excite  sympathy,  and  may  even, 
as  did  one  of  my  patients,  deliberately  scratch  their  corneas  with  pins. 
Hysterical  girls  sometimes  pull  out  their  eyelashes,  and  to  the  result  of 
this  performance  Gifford  has  given  the  name  "  hysterical  alopecia  of  the 
eyelids." 


""  Ophthalmic  Record,  viii.    1899,  p.  18. 
^  Annales  d'Oculistique,  vii.    1892,  p.  37. 
"Zeitschr.  f.  Augenheilk.,  v.    1901,  p.  477. 
"*  Reeueil  d'Ophtal.,  3  Series,  24,  1902,  p.  248. 


670  THE    EYE    AND    NERVOUS    SYSTEM 


CONCERNING  THE  ETIOLOGICAL  FACTORS  IN 
HYSTERICAL   OCULAR   STIGMATA. 

In  general  tenns  the  psychoneurosis  to  which  the  name  "  hysteria" 
usually  is  applied  is  responsible  for  the  development  of  the  ocular  signs 
which  have  been  described,  no  matter  which  of  the  causes,  of  the  great 
neurosis  may  have  been  originally  operative.  As  next  to  heredity 
trauma  takes  position  as  a  cause  of  hysteria,  it  is  not  unnatural  that 
many  eye-symptoms  of  this  disease  form  part  of  the  symptomatology  of 
traumatic  hysteria.  It  is  not  germane  to  the  subject  to  discuss  the  dis- 
tinction, if  it  exists,  between  hysteria  and  traumatic  hysteria,  to  some 
phases  of  which,  as  is  well  kno^vn,  under  the  name  traumatic  neurosisy 
Oppenheim  and  Thomson  have  given  a  special  position  in  the  nosological 
index,  except  to  say  that  the  ocular  anomalies  of  both  are  analogous,  if 
not  identical,  and  in  this  respect,  at  least,  if  not  in  all  others,  the  two 
affections  are  also  identical.  In  the  eyes  the  manifestations  of  hysteria 
which  have  been  described  as  hysterotraumatic  are  often  pronounced, — 
more  so,  frequently,  than  those  which  are  associated  with  hysteria  of 
other  origins, — ^but  they  are  not  pathognomonic.  In  other  words,  given 
certain  ocular  stigmata  of  hysteria,  one  could  not  expect  them  to  reveal 
the  original  cause  of  the  hysteria, — that  is  to  say,  whether  it  depended 
upon  heredity,  trauma,  disease,  defective  mentality,  toxaemia,  etc., — 
although  they  might  vary  in  their  severity  and  endurance.  The  eyes 
and,  in  general  terms,  the  optical  apparatus  contain  the  necessary 
structures  through  which  the  well-known  signs  of  hysteria — disturb- 
ances of  sensation,  spasms  and  contractures — may  become  evident  in  a 
manner  similar  to  their  development  in  other  organs  and  regions  of  the 
body. 

To  the  disturbances  of  the  functions  of  the  eyes  we  are  often  able 
to  apply  tests  more  delicate  than  those  which  pertain  to  the  investigation 
of  other  organs,  and  therefore  should  be  able  more  accurately  to  inter- 
pret the  symptoms.  The  admirable  investigations  of  Landolt,  Gale- 
zowski,  Parinaud,  Gilles  de  la  Tourette,  Pansier,  Borel,  Wilbrand, 
Schmidt-Rimpler,  and  Schwarz  are  of  especial  value  in  the  thorough- 
ness with  which  their  studies  have  been  pursued  from  these  and  all 
other  stand-points. 

Unfortunately,  however,  in  many  of  the  detailed  journal  reports  of 
hysterical  ocular  phenomena  the  lack  of  ophthalmological  training  is 
only  too  evident,  and  especially  regrettable  is  the  absence  of  proper 
investigation  of  the  refraction  of  the  eyes.  Thus,  Dejerine  points  out 
that  in  many  cases  of  so-called  unilateral  hysterical  amblyopia  it  is 


NEUROSES  AND  PSYCHOSES  671 

quite  possible  that  the  one  eye  may  have  been  emmetropic,  or  approxi- 
mating that  condition,  while  the  other  may  have  lost  visual  acuity 
owing  to  refractive  anomaly  which  has  wrongly  been  attributed  to  hys- 
teria. Pansier  and  Lapersonne  insist  on  the  difficulty  of  distinguishing 
between  hysterical  spastic  convergent  strabismus  and  convergent  stra- 
bismus associated  with  hyperopia  in  an  hysterical  subject.  The  effect  of 
hyperopic  refraction  on  the  anomalies  of  accommodation  so  commonly 
observed  in  hysterics  must  be  evident,  and  Parinaud  has  not  failed  to 
call  attention  to  it  in  his  beautiful  studies  of  this  subject,  and  to  the 
modifications  which  must  necessarily  be  produced  by  myopia  and  pres- 
byopia. What  the  relations  of  the  various  phases  of  exophoria  and 
esophoria  must  be  to  the  hysterical  anomalies  of  convergence  is  only  too 
plain  to  the  student  of  ocular  miTscle  imbalance.  In  a  word,  there  is 
constant  danger  of  attributing  to  hysteria  an  anomaly  of  ocular  func- 
tion, when  really  this  is  a  condition  belonging  to  the  eyes  of  an  hys- 
terical subject.  More  than  this, — and  it  has  been  strangely  neglected  in 
the  investigation  of  hysterical  phenomena, — refractive  error,  especially 
astigmatism,  muscular  imbalance,  in  short,  "  eye-strain,"  are  responsi- 
ble for  many  cases  of  hysteria  which  manifest  themselves  by  well- 
known  stigmata,  not  only  in  the  eyes  but  in  other  regions  and  organs  of 
the  body,  or  by  the  phenomena  pertaining  to  seizures.  It  may  perhaps 
be  doubted  whether  refractive  error  and  heterophoria  produce  hys- 
terical manifestation  in  individuals  of  sound  constitution  and  normal 
resisting  power,  but  they  certainly  can  call  them  into  existence  in 
those  of  neuropathic  tendency.  Given  a  normal  subject  with  moderate 
hyperopic  astigmatism  and  esophoria  (convergence-excess),  and  the  eye 
troubles  will  interpret  themselves  by  headache,  relieved  by  their  cor- 
rection, or  perhaps  resisted  without  serious  detriment  for  long  periods 
without  correction.  In  a  neuropathic  subject  with  the  same  defects 
their  interpretation  is  likely  to  be  spasm  of  accommodation,  anaesthesia 
of  the  retina,  contracture  of  convergence,  restriction  of  the  visual  field, 
and  areas  of  anaesthesia  often  relieved  by  their  correction,  but  in  the 
absence  of  it  their  influence  is  unresisted,  and  the  long-enduring  phe- 
nomena of  what  may  be  called  a  general  hysteria  result. 
For  the  treatment,  see  page  74. 

OCULAR   MANIFESTATIONS    OF   NEURASTHENIA 
AND  TRAUMATIC  NEUROSES. 

Although  the  disturbance  of  the  visual  sensation  to  which  neuras- 
thenic patients  and  those  who  are  the  subjects  of  traumatic  neuroses  are 
liable  has  been  much  investigated,  it  cannot  be  stated,  as  it  was  of 


€72  THE    EYE    AND    NERVOUS    SYSTEM 

m 

liysteria,  that  such  ocular  signs  as  have  been  described  constitute  a  dis- 
tinct symptomatology.  For  convenience,  the  following  subdivisions  of 
the  subject  may  be  recorded : 

(1)  Defective  and  painful  visual  sensation,  or  neurasthenic — more 
properly  nervous — asthenopia. 

(2)  Anomalies  of  the  iris  movements,  or  neurasthenic  pupil  phe- 
nomena. 

(3)  Anomalies  of  the  eyelid  and  eye-muscle   movements,   or  so- 
called  neurasthenic  insufficiencies  of  the  ocular  muscles. 

(4)  Anomalies  of  the  visual  field. 

(5)  Previously  unclassified  neurasthenic  ocular  phenomena. 
Neurasthenic  or  Nervous  Asthenopia. — Certain  systematic  writers, 

— for  example,  Wilbrand  and  Saenger,^^ — have  made  a  distinction  be- 
tween nervous  asthenopia,  or  the  insufficiency  of  visual  power  seen  in  ner- 
vous persons,  as  it  occurs  in  school  children  and  during  the  years  of  early 
development  and  as  it  appears  in  adults.  School  children,  particularly 
from  the  ninth  to  the  fifteenth  year,  often  are  brought  for  examination 
because  they  complain  of  a  flickering  before  the  eyes, — letters  and 
figures  disappear  as  they  look  at  them  and  the  print  runs  together,  and 
reading  creates  lachrymation  and  severe  brow  and  eye  pain.  When  the 
child  is  asked  to  read  he  gradually  approaches  his  face  to  the  book, 
moves  his  head  from  side  to  side  instead  of  following  the  lines  with  his 
eyes,  and  tries  to  bring  to  his  aid  stronger  and  stronger  light.  In 
association  with  the  difficulty  of  vision  are  contraction  of  the  eyebrows, 
half-closing  of  the  eyelids,  and  often  twitching  of  the  face  and  muscles 
of  the  shoulder,  or,  in  other  words,  the  symptoms  of  the  so-called  habit 
spasm.  This  gradual  approaching  of  the  book  towards  the  face,  so  that 
the  patient  is  able  to  obtain  larger  retinal  images,  is  more  or  less  char- 
acteristic of  this  condition  in  childhood  and  is  not  seen  in  the  adult 
cases.  It  depends  upon  a  cramp  of  accommodation,  and  when  the 
cramp  subsides  and  there  is  tiring  of  the  accommodative  muscle  there  is 
corresponding  blurring  of  the  print  and  running  together  of  the  lines. 
If  the  visual  power  is  tested  in  the  ordinary  manner  with  test-types,  it 
will  usually  be  found  to  possess  a  depreciation  greater  than  would  be 
accounted  for  by  any  error  of  refraction,  and  will  often  be  promptly 
raised  to  normal  if  the  letters  are  viewed  through  tinted  glasses.  In 
other  cases  glasses  fail  to  improve  the  visual  acuity. 

Associated  with   this  asthenopia  and  diminution  of  visual  acuity 

'*  Ueber  Sehstorungen  bei  functionellen  Nervenleiden,  Leipsic,  1892,  p.  58. 


NEUROSES  AND  PSYCHOSES  673 

eoucentric  contraction  of  the  visual  field  may  be  noted,  in  the  manner 
which  will  be  described  in  a  subsequent  paragraph. 

It  is  noteworthy  that  in  children  these  ocular  phenomena  are  marked 
to  the  exclusion  of  other  neurasthenic  phenomena,  while  in  adults  the 
reverse  may  be  the  case.  The  subjective  symptoms  in  adults  are  similar 
to  those  just  detailed.  They  have  been  described  by  Wilbrand,  Standish, 
and  others  somewhat  as  follows :  Headache,  particularly  throbbing  in 
the  brow  and  temples,  occipital  distress,  pain  in  the  back  of  the  neck  and 
spine,  vertigo,  muscae  volitantes,  defective  accommodation,  intolerance  of 
light,  and  improvement  in  vision  in  the  dusk  and  through  tinted  glasses. 
In  addition  to  this,  any  attempt  at  concentrated  vision  is  followed  by  a 
rapid  disappearance  from  view  of  the  object  which  is  to  be  fixed.  There 
are  diminution  of  central  vision,  sudden  attacks  of  obscuration  of  vision, 
processions  of  scotomas,  visual  hallucinations,  lack  of  fixation  of  the 
optical  memory  images,  persistent  and  confusing  after-images,  colored 
vision, — for  example,  erythropsia, — and  a  sudden  red  color  of  the  pages 
of  a  book  while  the  letters  seem  to  be  green.  Associated  with  these 
phenomena  are  the  characteristic  changes  in  the  visual  field  presently 
to  be  described. 

Etiology. — While  it  is  true  that  neurasthenic  patients,  both  children 
and  adults,  may  possess  as  part  of  their  symptomatology  the  symptoms 
of  defective  and  painful  visual  sensation  which  have  been  detailed, 
nervous  asthenopia,  as  Schwarz  ®^  properly  remarks,  is  justified  as  a 
descriptive  term  only  when  the  chief  symptoms  of  asthenopia — namely, 
hyperopia,  astigmatism,  and  ocular  muscle  imbalance — have  been  ex- 
cluded. In  other  words,  as  is  now  well  known  but  as  evidently  was  not 
well  recognized  by  earlier  writers,  these  patients  have  nervous  asthe- 
nopia not  because  they  are  neurasthenics,  but  because  they  have  various 
refractive  errors,  particularly  astigmatism,  and  various  types  of  mus- 
cular imbalance,  which,  indeed,  are  in  many  cases  the  causative  factors 
of  the  neurasthenia  itself,  and  all  of  the  symptoms,  those  referable  to  the 
eye  as  well  as  to  the  general  economy,  are  simply  the  manifestations  of 
eye-strain  occurring  in  individuals  of  neuropathic  constitution.  They 
appear  in  these  bizarre  manifestations,  because  of  such  constitution, 
while  in  another  individual  of  sound  health  they  might  either  be  resisted 
or  find  interpretation  in  a  milder  type  of  so-called  asthenopia. 

Anomalies  of  the  Iris  Movements,  or  Neurasthenic  Pupil  Phe- 
nomena.— Unlike  hysteria,  there  are  no  pupil  phenomena  which  in  any 


"Die  Bedeutung  der  Augenstorungen  fiir  die  Diagnose  der  Hirn-  und  Rucken- 
marks-Krankheiten,  Berlin,   1898,  p.  97. 
43 


674  THE    EYE    AND    NERVOUS    SYSTEM 

sense  are  characteristic  of  the  neurasthenic  state.  It  is  true,  as  Beard 
pointed  out,  that  the  pupils  are  sometimes  unequal,  but  not  more 
frequently,  as  Schmidt-Rimpler  ®^  maintains,  than  has  been  observed 
under  physiological  conditions.  Wilbrand  also  declares  that  the  pupil 
reactions  are  usually  normal,  and  that  the  slight  differences  in  the 
width  of  the  pupil  which  are  often  seen  in  these  individuals  depends 
upon  certain  conditions  of  the  eye  itself,  and  not  upon  the  general  and 
physical  state.  The  inequality  of  the  pupil  observed  in  neurasthenia, 
according  to  Dercum,  is  always  transient  in  character.  It  is  true  that 
the  pupils  of  neurasthenics  are  often  wider  than  those  of  more  stable 
physical  condition,  and  they  sometimes  seem  to  manifest  an  unusually 
prompt  response  to  light  impulse.  On  the  other  hand,  occasionally  they 
also  act  less  promptly  to  light. 

Hippus  is  said  to  be  more  common  in  neurasthenia  in  an  exaggerated 
condition  than  in  other  functional  nervous  states,  but  this  is  doubted  by 
many  competent  observers,  and  my  own  observations  of  many  neuras- 
thenics would  not  lead  me  to  believe  that  hippus  should  be  regarded  as  a 
sign  of  neurasthenia,  nor  have  I  ever  seen  any  pupil  change  which  is 
significant  of  a  pure  neurasthenia.  Should  there  be  reflex  immobility  to 
light  in  a  patient  exhibiting  the  symptoms  of  neurasthenia,  there  would 
be  good  reason  to  suspect  tabes  or  beginning  paresis. 

Anomalies  of  the  Eyelid  and  Eye-Muscle  Movements,  or  So-called 
Neurasthenic  Insufficiencies  of  the  Ocular  Muscles. — In  neurasthenia, 
unlike  hysteria,  anomalies  of  the  musculature  of  the  bulbus  and  of  the 
lids  are  for  the  most  part  wanting.  Certainly  the  various  types  of  con- 
tracture of  the  eye  muscles  are  lacking,  nor  is  blepharospasm,  as  it  has 
been  described  in  connection  with  hysteria,  present.  A  frequent  symp- 
tom in  neurasthenia  is  an  inability  of  the  patient  to  close  the  eyes 
completely  when  standing  with  the  legs  together  (Romberg's  sign), 
associated  with  marked  fibrillary  twitchings  of  the  orbicularis.  This  lid 
phenomenon  has  sometimes  been  called  RosenhacWs  ^^  sign,  which  he 
and  Banas  have  considered  as  a  characteristic  symptom  of  neurasthenia, 
but  which  Schmidt-Rimpler  declines  to  regard  as  peculiar  to  this  affec- 
tion, inasmuch  as  it  is  often  seen  in  healthy  individuals.  According  to 
some  writers, — for  example,  Loewenfeld, — this  symptom  is  also  present 
when  the  legs  are  separated  or  when  the  patient  is  in  the  sitting  position. 
It  has  been  ascribed  to  weakness  of  the  orbicularis  and  to  spasm  of  the 


*'  Die  Erkrankungen  des  Auges  im  Zusammenhang  mit  anderen  Krankheiten, 
Wien,  1898.  p.  276. 

"  Centralblatt  f.  Nervenheilkunde,  ix.,  No.  17. 


KEUROSES  AXD  PSYCHOSES  675 

sympathetic.     As  Knies  puts  it,  the  voluntary  motor  innervation  is 
enfeebled  and  the  sympathetic  reflexes  are  increased. 

According  to  Landolt,^^  there  is  a  form  of  insufficiency  of  con- 
vergence which  accompanies  the  neuroses,  and  especially  neurasthenia,  to 
which  he  has  applied  the  term  neuropathic  insufficiency  of  convergence, 
and  which  he  describes  as  follows:  The  excursion  of  the  eyes  may  be 
normal,  the  action  of  the  abductors — that  is,  of  divergence — also  normal 
or  even  augmented,  but  adduction  is  deficient.  In  such  cases  there  is  a 
reduction  at  both  ends  of  the  range  of  convergence.  ( See  also  article  on 
hysteria,  p.  44.)  Landolt  believes  that  this  insufficiency  of  the  innerva- 
tion of  the  adductors,  or  of  convergence,  is  a  phenomenon  which  should 
be  regarded  as  analogous  to  the  weakness  of  the  orbicularis  which  has 
just  been  described  as  Rosenbach's  symptom. 

A  symptom  often  noted  in  neurasthenic  as  well  as  hysteric  patients 
(see  section  on  hysteria,  p.  656)  is  the  so-called  morning  ptosis  of  Gowers, 
the  night  ptosis  of  Weir  Mitchell,  and  which  has  been  regarded  by 
some  authorities  as  a  form  of  flaccid  ptosis,  which  is,  as  has  previously 
been  noted,  a  frequent  sign  in  hysteria.  Certain  nervous  individuals, 
particularly  women,  usually  on  awakening  in  the  morning,  or  during 
the  night,  find  it  impossible  to  raise  their  eyelids  without  assisting  the 
action  of  the  levator  with  their  fingers.  There  is,  in  other  words,  a 
sensation  as  if  the  lids  were  glued  together,  and  associated  with  it  there  is 
often  a  dry  catarrh  of  the  conjunctiva  or  a  hyperaemic  condition  of  this 
membrane.  I  have  studied  a  number  of  cases  of  night  ptosis  of  this 
variety,  and  all  of  its  subjects  were  nervous  persons  in  the  ordinary 
sense  of  the  term  and  not  hysterics.  Usually  they  have  been  women  past 
middle  life,  who  have  often  been  seriously  alarmed,  thinking  that  they 
had  suddenly  become  the  subjects  of  a  paralysis.  In  most  instances 
proper  general  regimen,  with  correction  of  existing  refractive  errors, 
has  relieved  the  difficulty,  i^ystagmus  has  been  described.  According 
to  P.  C.  Knapp,  strabismus  and  nystagmus  may  be  present  in  certain 
traumatic  neuroses,  but  they  are  really  traumatic  scleroses. 

Anomalies  of  the  Visual  Field. — Of  greater  interest  than  the  other 
ocular  symptoms  associated  with  neurasthenia  are  those  changes  in  the 
field  of  vision  which  interpret  the  ready  exhaustion  to  which  {he  visual 
apparatus  of  the  subjects  of  this  condition  are  liable.     They  have  been 


="  The  Refraction  and  Accommodation  of  the  Eye  and  their  anomalies.  Phila- 
delphia, 1886,  p.  504.  A  System  of  Diseases  of  the  Eye,  edited  by  Norris  and 
Oliver,  vol.  iv.  p.  1.36. 


676  THE    EYE    AND    NERVOUS    SYSTEM 

elaborately  and  especially  studied  by  Forster,^^'^  Wilbrand  and  Saen- 
ggj.101  wiiiieini  Koenig,^*^^  Peters/^^  Frank-Hochwart  and  Topo- 
lanski,^''^  Schmidt-Rimpler,^<>'*  von  Reuss/*''^  H.  Arnlieim '«^  and  L. 
Wolffberg.^^®  To  the  writings  of  these  authors  I  am  much  indebted 
in  the  preparation  of  the  following  pages. 

In  general  terms  these  alterations  in  the  visual  field  may  be  de- 
scribed as  fatigue  constrictions  or  contractions,  and,  according  to  Wil- 
brand and  Saenger,  the  contraction  may  extend  to  the  point  of  dis- 
apjjearance  of  the  visual  field,  or,  more  usually,  to  a  certain  point  of 
concentric  narrowing.  Furthermore,  the  fatigue  phenomena  may  appear 
in  all  of  the  four  halves  of  the  visual  field  or  only  in  certain  ones. 
According  to  the  same  authors,  the  signs  of  fatigue  may  manifest  them- 
selves when  the  extent  of  the  visual  field  and  central  visual  acuity  is  at 
first  normal,  or  when  the  extent  of  the  visual  field  is  at  first  normal,  but 
visual  acuity  is  diminished,  or,  finally,  when  the  extent  of  the  visual 
field  is  concentrically  contracted  from  the  beginning  and  central  visual 
acuity  is  diminished. 

Von  Reuss  insists  that  as  neurasthenia  is  characterized  by  an  easy 
exliaustion  of  the  visual  apparatus,  this  gives  rise  to  visual  fields  of  a 
fleeting  and  changing  nature.  The  diminution  of  the  size  of  the  field 
appears,  according  to  him,  only  during  the  process  of  examination,  or,  if 
it  is  present  from  the  beginning,  it  is  due  to  exhaustion  of  the  eye  by  a 
previous  examination.  Systemic  writers  have  described  certain  types 
of  visual  field  fatigue  as  follows : 

I.  Concentric  Contraction. — There  may  be  a  general  concentric  con- 
traction of  the  visual  field  similar  to  that  seen  in  hysteria,  but  differing 
from  it,  according  to  von  Reuss,  because  in  neurasthenia  it  is  constantly 


"•  Bericht  liber  die  zehnte  Versammlung  der  Ophthalmologischen  Gesellschaft, 
Heidelberg,  1877,  p.  162. 

""  Ueber  Sehstorungen  bei  functionellen  Nervenleiden,  Leipzig,  1892,  and  Wil- 
brand, Die  Erholungsausdehnung  des  Gesichtsfeldes  unter  normalen  und  patholo- 
gischen  Bedingungen,  Wiesbaden,  1896. 

""  Ueber  (Jesichtsfeld-Ermiidung  und  deren  Beziehung  zur  concentrischen  Ge- 
sichtsfeldeinschriinkung  bei  Erkrankungen  des  Centralnervensystems,  Leipzig,  1893. 
(This  work  contains  a  full  bibliography.) 

*"  Deutsche  Zeitschrift  f.  Nervenheilk.,  Bd.  v.,  1894,  p.  302. 

"**  Deutschmann's  BeitrJlge  zur  Augenheilk.,  ii.,  1895,  p.  46. 

"'  Die  Erkrankungen  des  Auges  im  Zusammenhang  mit  anderen  Krankheiten, 
Wien,  1898,  p.  266.     (This  work  contains  an  extensive  bibliography.) 

***  Das  Gesichtsfeld  bei  functionellen  Nervenleiden,  Leipzig  and  Wien,  1902. 

""  Fortschritte  der  Medicin,  vol.  xx.,  1903,  p.  44.  (This  paper  contains  a  critical 
review  of  the  subject  with  an  analysis  of  the  important  papers.) 

""Archives  of  Ophthalmology,  xxxiii.,  1904,  p.  597. 


IS^EUKOSES  ANT>  PSYCHOSES 


677 


changing  during  examination,  while  in  hysteria  it  does  not  alter  its  size 
until  recovery  ensues.  Moreover,  there  is  no  change  in  the  sequence  of 
the  color  circles. 

Temporary  uniform  concentric  contraction  of  the  visual  field,  so  that 
it  resembles  a  circle,  or  closely  approaches  in  appearance  a  reduced 
visual  field,  frequently  follows  an  hysterical,  hystero-epileptic,  or  epi- 
leptic attack,  and  hence  is  not  peculiar  to  neurasthenia. 

Permanent  uniform  concentric  contraction  of  the  visual  field  is  not 
a  neurasthenic  phenomenon,  unless  those  examples  which  Wilbrand  has 
described  in  certain  types  of  traumatic  neuroses  be  excepted. 

II.  Fbrster's  Shifting  or  Displacement  Type  of  the  Visual  Field.  —Peri- 
metric examination  of  certain  nervous  patients  Avill  often  yield  a  visual 
field  of  very  irregular  type,  due  to  a  more  or  less  unsymmetrical  contrac- 
tion of  the  field  from  meridian  to  meridian  brought  about  by  fatigue. 
This  is  the  important  symptom  of  that  condition  called  by  Von  Graefe 


Fig.  12.— Forster's  shifting  or  displacement  type  of  the  visual  field.    (After  Wilbrand./ 

"  anaesthesia  of  the  retina,"  but  now  generally  known  by  the  name  of 
"  nervous  asthenopia,"  proposed  by  Wilbrand  in  place  of  the  more  re- 
stricted term  "  neurasthenic  asthenopia,"  and  the  general  visual  symp- 
toms of  which  have  already  been  described.     (See  Page  672.) 

Forster  ^^®  was  the  first  to  examine  the  fatigue  phenomena  of  the 
visual  field  under  these  circumstances  with  particular  care.  His  method 
is  as  follows:    The  test-object  is  carried  on  the  perimeter  from  the 


>"  Loc.  cit. 


C78  THE    EYE    AND    NERVOUS    SYSTEM 

periphery  of  the  temporal  side  through  the  fixation  point  to  the  nasal 
side  along  all  the  meridians,  and  its  points  of  entrance  and  exit  noted  on 
the  chart.  It  will  be  found  that  the  visual  field  obtained  has  a  greater 
extent  in  the  temporal  half, — that  is,  the  side  where  the  test-object  first 
entered  the  field, — than  on  the  nasal  side,  because  when  the  latter  was 
tested  the  patient  was  already  fatigued.  If  after  an  interval  of  rest  the 
central  test  is  made  by  examining  the  same  meridians  in  the  same  order, 
but  now  starting  from  the  periphery  of  the  nasal  side,  and  the  points  of 
entrance  and  exit  of  the  test-object  noted  on  the  chart,  it  will  be  found 
that  the  visual  field  is  greater  in  extent  on  the  nasal  half  because  it  is  the 
less  fatigued  half  of  the  field.  In  other  words,  two  visual  fields  are 
obtained,  the  outlines  of  which  appear  to  have  shifted  or  been  displaced 
in  their  relation  to  each  other.  For  this  character  of  visual  field  0. 
Koenig  suggested  the  term  "  shifting  or  displacement  type,"  while 
Schiele  described  the  procedure  by  the  name  "  diametrical  method."  ^^'^ 

Schiele  (quoted  by  Wilbrand)  devised  a  method  of  investigating  the 
field  of  vision  to  which  the  name  radial  centripetal  method  has  been 
given.  His  investigations  were  made  with  a  Scherk's  perimeter.  The 
meridians  were  systematically  fatigued,  only  in  one-half  of  the  visual 
field,  from  the  periphery  as  far  as  the  fixation  point,  and  he  found  in 
certain  cases  that  an  equally  large  fatigue  defect  was  found  on  the 
same  meridians  of  the  homonymous  halves  of  the  visual  field  of  the 
other  eye,  which  had  been  covered  during  the  previous  examination,  as 
on  the  half  of  the  eye  which  had  been  examined  first.  To  his  explana- 
tion of  this  phenomenon  we  shall  return  later. 

III.  Wilbrand's  Exhaustion  Type  of  the  Visual  Field. — Because  For- 
ster's  method  is  time-consuming  and  very  fatiguing  to  the  patient,  Wil- 
brand materially  simplified  it,  confining  his  examination  to  the  hori- 
zontal meridian.  Von  Reuss,  however,  declines  to  consider  Wilbrand's 
method  as  a  simplification  of  the  plan  pursued  by  Forster  and  prefers  to 
give  it  a  special  classification,  suggesting  the  title  "  Wilbrand's  exhaus- 
tion type."  According  to  Wilbrand,  fatigue  phenomena,  whenever  pres- 
ent, occur  on  all  of  the  meridians  of  the  corresponding  half  of  the  visual 
field.  Hence,  in  order  to  determine  the  defatigability  of  the  nervous 
system  quickly,  he  proceeds  as  follows :  He  uses  the  horizontal  meridian 
as  being  the  meridian  of  the  visual  field  of  greatest  extent.  Beginning 
at  the  temporal  edge  of  the  perimeter,  he  carries  the  white  test-object 
along  the  horizontal  meridian  with  as  steady  a  movement  as  possible  in 
the  direction  of  the  arrow  (Fig.  13)  toward  the  nasal  half,  and  has  an 

"°  Consult  Wilbrand  and  Koenig,  loc.  cit. 


I^EUROSES  AND  PSYCHOSES 


679 


assistant  mark  on  the  diagram  with  a  O  the  point  at  which  the  white 
test-object  enters  the  field  and  with  a  I  the  point  where  it  disappears. 
Having  reached  the  point  I  on  the  nasal  half,  the  direction  of  the  test- 
object  is  at  once  reversed  and  it  is  carried  with  the  same  uniform 
velocity  along  the  horizontal  meridian  toward  the  temporal  half.  The 
point  on  the  temporal  half  of  the  horizontal  meridian  where  the  test- 
object  again  disappears  after  its  second  fatigue  excursion  is  recorded  on 
the  diagTam  by  the  figure  2.  The  object  is  then  at  once  brought  back 
again  in  the  opposite  direction  and  the  point  at  which  it  disappears  on 
the  nasal  half  indicated  by  the  figure  3,  and  so  on. 

This  method  of  examination  takes  little  time,  and  according  to  Wil- 
brand,  enables  the  examiner  to  recognize  whether  concentric  constriction 
of  the  visual  field  was  already  present  at  the  beginning  of  the  examina- 


FiG.  13.— Wilbrand's  exhaustion  type  of  the  visual  field.    (After  Wilbrand.) 


tion,  and  whether  there  was  with  it  a  slight  degree  of  def  atigability ;  on 
which  half  of  the  visual  field  this  defatigability  is  chiefly  elicited,  and 
whether  it  can  be  extended  as  far  as  the  fixation  point  or  ceases  at  a 
definite  parallel  on  the  perimeter. 

To  these  advantages  (quoted  from  "Wilbrand)  W.  Koenig  adds 
another,  namely,  that  the  method  affords  an  accurate  means  of  measuring 
the  patient's  attentiveness,  or,  in  other  words,  his  ability  to  give  ser- 
viceable answers  when  examined  with  the  perimeter. 

The  portion  of  the  visual  field  which  resists  fatigue  has  been  called 
by  Wilbrand  "  minimal  visual  field."  Analogously  W.  Koenig  desig- 
nates the  visual  field  as  it  was  in  the  beginning  of  the  "  maximal  visual 


680 


THE    EYE    AND    NERVOUS    SYSTEM 


field."  According  to  Wilbrand,  whenever  fatigue  is  present,  it  appears 
on  all  the  meridians  of  the  same  half  of  the  field,  and  the  fatigue  of  the 
visual  field  is  the  same  for  colors  as  for  white.  W.  Koenig,  however, 
thinks  that  while  the  visual  field  becomes  fatigued  for  white,  it  does 
not  lose  its  reactions  to  colors  at  the  same  time.  For  example,  if  a  visual 
field  has  been  fatigued  down  to  the  minimal  visual  field  by  means  of  a 
white  test-object,  if  a  colored  test-object  is  brought  into  the  field  from 
the  temjjoral  side  without  any  interval  of  rest,  the  point  of  entrance  of 
the  said  colored  test-object  approximately  coincides  with  the  point  where 
it  is  first  seen  after  a  considerable  period  of  rest.  Sometimes,  according 
to  Koenig,  only  a  slight  difference  can  be  demonstrated,  and  if  no  inter- 


FiQ.  14.— Oscillating  visual  field.    (After  Wilbrand  ) 

val  of  rest  is  allowed  the  limits  obtained  are  narrower.  The  same 
behavior  to  white  and  the  remaining  colors  is  observed  in  the  visual  field 
if  a  colored  test-object  has  been  used  in  producing  the  fatigue. 

IV.  The  Oscillating  Visual  Field  and  Fatigue  Scotomas. — According  to 
Wilbrand,  in  contrast  with  types  of  constant  progressive  fatigue,  cases 
are  occasionally  observed  in  which,  to  quote  his  own  words,  the  fatigue 
pursues,  so  to  speak,  a  ryhthmical  course,  the  test-object  vanishing  from 
time  to  time  along  the  meridians,  to  emerge  again  after  a  short  interval 
and  again  disappear  and  emerge,  and  so  on.  For  this  condition 
Wilbrand  suggests  the  term  oscillating  visual  field.  He  believes  that 
the  condition  represents  an  alternating  flaring  up  and  extinguishing  of 
the  optic  sensory  tracts.  The  same  oscillation  obtains  when  a  colored 
object  is  used.  Such  visual  fields,  which  are  not  common,  have  been 
observed  by  him  and  others,  particularly  Koenig,  in  various  nervous 


ITEUROSES  AND  PSYCHOSES 


681 


conditions, — that  is,  not  only  in  post-epileptic  states,  but  in  neuras- 
thenic patients. 

These  authors  and  others  have  also  described  temporary  central 
scotomas  representing  fatigue  phenomena  of  the  macular  portion  of 
the  visual  field,  especially  frequent  in  young  persons  of  neurasthenic 
tendency.  The  scotoma  may  be  confined  to  the  fixation  point  or  extend 
beyond  it.  It  is  chiefly  manifest  to  small  colored  objects,  less  commonly 
when  the  test-object  is  white.  It  will  be  remembered  that  in  the  section 
on  hysteria  similar  phenomena  were  mentioned,  and  Von  Reuss  believes 
these  types  of  oscillating  visual  field  and  scotomas  are  really  hysterical 
manifestations. 

V.  Fatigue  Spiral  Field  (Exhaustion  Spiral). — In  ordinary  perimetry, 
the  eye  being  normal,  if  the  points  on  each  meridian  at  which  the  test- 
object  is  first  seen  are  united  with  a  line,  the  well-known  shape  of  the 
visual  field  appears.  If  this  examination  is  repeated,  an  exactly  similar 
map  will  be  produced,  or  one  which  differs  only  slightly  from  the  origi- 


FiG.  15.— Fatigue  spiral  field,    (von  Reuss.) 

nal.  If,  on  the  other  hand,  according  to  von  Reuss,"  ^  fatigue  of  the  vis- 
ual field  develops,  then,  the  first  field  having  already  been  taken,  the 
point  at  which  the  test-object  in  the  second  trial  appears  in  the  visual 
field  on  the  horizontal  meridian,  for  example,  lies  nearer  to  the  centre, 
and  this  is  also  the  case  in  all  meridians.  Wlien  the  examiner  begins  for 
the  third  time,  the  point  of  appearance  of  the  test-object  lies  still  nearer 
to  the  point  of  fixation,  and  so  forth,  until  further  contraction  can  no 
longer  result  and  the  later  points  of  appearance  of  the  test-objects  coin- 
cide with  the  earlier  ones.     If  all  of  these  points  are  connected,  a  spiral 


'"  Loc.  cit. 


()4. 


682  THE    EYE    AND    NERVOUS    SYSTEM 

line  is  formed,  which  ultimately  terminates  with  a  closed  line.  To  this 
form  of  the  visual  field  von  Reuss  has  given  the  name  "  fatigue  spiral." 
It  goes  without  saying  that  the  visual  field  does  not  possess  the  form  of  a 
spiral,  and  that  the  reduction  in  size  does  not  take  place  in  such  a  form. 
The  narrowing  takes  place  because  the  boundaries  are  contracted  con- 
centrically and  the  points  at  which  the  test-object  is  first  noticed  are 
only  the  stages  at  which  the  boundary  lines  cut  the  individual  meridians, 
at  the  moment  when  the  test-object  appears.  These,  connected  by  lines, 
take  the  form  of  a  spiral,  a  snail's  house,  or  a  watch-spring.  Similar 
fields,  according  to  this  author,  have  been  noticed  by  Straub  and  Fuchs. 
He  thinks  the  spirals  are  not  more  frequently  observed  because  they  are 
not  properly  searched  for. 

VI.  The  Recuperative  Extension  Type  of  Visual  Field. — The  contracted 
visual  field  may  enlarge  after  recovery  from  fatigue.  Necessarily  the 
rapidity  of  recuperation  depends  upon  the  previous  degree  of  fatigue. 
Von  Reuss  thinks  that  such  enlargement  is  particularly  easy  to  observe 
in  connection  with  the  spiral  fields.  Such  enlargements,  for  example, 
have  been  not€d  during  psychic  disturbance  and  as  the  result  of  will 
power,  and  thus  explain  the  negative  spirals  of  Fuchs,  who  demon- 
strated graphically  the  successive  dilatation  of  the  visual  field  under 
these  circumstances.  Recovery  of  the  fatigue  of  the  visual  field  may  be 
produced  by  long-continued  rest,  especially  if  the  subject  remains  in  a 
dark  room  and  all  irritations  of  the  optic  nerve  are  removed.  As  is 
well  known,  Wilbrand  has  studied  this  matter  particularly  and  examined 
the  visual  field  in  the  dark,  using  illuminated  colors.  As  von  Reuss 
points  out,  interesting  as  are  his  results,  the  examinations  are  difficult 
and  often  yield  contradictory  results. 

VII.  The  Mixed  Forms  of  Visual  Field. — Reference  has  already  been 
made  to  these  in  the  section  on  hysteria.  Von  Reuss  contends  that  all 
constant  visual  field  contractions  are  of  hysterical  nature,  while  every- 
thing which  is  of  variable  nature  in  the  visual  field  belongs  to  neuras- 
thenia. Contracted  visual  fields,  he  believes,  which  become  still  smaller 
in  the  course  of  examination,  belong  to  the  mixed  forms.  This  mixture 
of  hysteria  and  neurasthenia  is  not  uncommon,  especially  in  trau- 
matic neurosis.  According  to  him,  the  visual  field  under  these  circum- 
stances gives  evidence  of*  the  mixture  of  the  two  nervous  conditions,  the 
constant  concentric  limitation  indicating  hysteria  and  the  exhaustion 
spiral  neurasthenia.  Naturally,  all  precautions  must  be  taken  to  elimi- 
nate organic  disease  of  the  eye. 

Pathogenesis  of  the  Fatigue  Field. — The  exact  nature  of  the 
fatigue  which  brings  about  the  various  forms  of  visual  field  which  have 
thus  far  been  described  is  still  the  subject  of  much  discussion.     The 


:n'euroses  and  psychoses  ess 

whole  matter  has  recently  been  critically  reviewed  in  an  admirable 
manner  by  H.  Arnheim,"^  and  from  his  paper  the  following  interesting 
views  are  quoted :  According  to  Wilbrand,  concentric  and  fatigue  con- 
traction of  the  visual  field  are  interpretations  of  the  same  affection, 
— namely,  insufficiency  of  the  retina, — the  entire  symptom-complex  of 
nervous  asthenopia,  according  to  him,  in  so  far  as  it  relates  to  alterations 
and  insensitiveness  to  light,  is  localized  in  the  external  layer  of  the 
retina,  where  it  is  dependent  upon  changed  conditions  of  metabolism. 
Through  the  mediation  of  the  optic  tracts,  however,  probably  centrifugal, 
these  metabolic  processes  may  come  under  the  influence  of  remote  por- 
tions of  the  brain. 

Peters  regards  the  narrowing  of  fatigue  as  representing  a  disturb- 
ance of  innervation  which  transmits  stimuli  from  every  point  of  the 
retina  to  the  two  optic  tracts.  Any  disturbances  in  the  nervous  paths 
which  subserve  this  function  produce  displacement  types  of  the  visual 
field,  which  accordingly  represent  a  relative  insufiiciency  of  the  optic 
nerve. 

Placzek^^^  contends  that  the  displacement  types  of  the  visual  field 
depend  upon  a  diminution  and  blunting  of  the  centre  of  consciousness — 
that  is,  a  diminution  in  the  respective  power  of  the  centre. 

According  to  Simon,^^'*  the  phenomena  of  fatigue  should  be  referred 
to  a  physical  basis.  The  forms  in  which  fatigue  ceases  after  the  first 
examination  are  ascribed  to  a  diminished  power  of  psychical  perception 
as  compared  with  sensory  irritability,  and  those  which  increase  as  the 
examination  proceeds  are  supposed  to  depend  upon  a  general  fatigue  of 
the  psychic  sphere.  In  a  word,  Simon  believes  that  these  phenomena 
are  due  to  an  exhaustion  of  the  psyche.  This  theory  von  Reuss  also 
adopts  as  being  more  comprehensive  than  the  others. 

Finally,  we  come  to  the  views  of  those  who  do  not  regard  the  con- 
traction of  the  visual  field  depending  upon  fatigue  as  a  pathological 
process,  but  as  an  effect  of  a  disturbance  of  concentration.  In  other 
words,  such  conditions  can  be  brought  about  by  inattention  and  lack  of 
concentration,  and  inattention,  according  to  Schmidt-Rimpler,  best  ex- 
plains the  phenomena  which  have  thus  far  been  described. 

Wolffberg^^^  points  out  that  in  practice  usually  the  suspicion  of 
traumatic  neurosis  or  allied  condition  is  entertained  before  the  visual 
field  examination  is  begun,  and  hence  the  various  types  of  fatigue  field 

"-  Fortschritte  der  Medicin,  vol.  xxi.,  1903,  pp.  44-54. 

'"Berlin,  klin.  Wochenschr.,  1892. 

"*Arch.  f.  Opth.,  xl.,  Bd.  4,  1894. 

"^Archives  of  Ophthalmology,  vol.  xxxiii.   1904,  p.  601. 


684  THE    EYE    AND    NERVOUS    SYSTEM 

are  searched  for  without  examination  of  the  field  in  the  usual  manner. 
But  he  does  not  believe  with  Schmidt-Rimpler  that  contraction  of  the 
field  in  neurasthenic  patients  by  fatiguing  examinations  is  wholly  due  to 
relaxed  attention.  He  thinks,  moreover,  that  von  Reuss  has  given  ex- 
perimental proof  that  the  fatigue  of  the  neurasthenic  is  chiefly  a  psy- 
chical fatigue.  As  the  fatigue  is  psychical,  he  maintains  it  is  also  mani- 
fest in  direct  vision,  and  he  regards  slight  fatigue  of  the  macular  region 
as  a  symptom  of  traumatic  neurosis  not  less  important  than  that  of 
peripheric  vision,  and  that  each  is  connected  with  the  other. 

Schiele's  theory  (his  results  in  visual  field  examinations  under  these 
circumstances  having  already  been  referred  to)  that  each  individual 
portion  of  one  retina  is  in  relation  with  the  homonymous  portion  of  the 
other,  and  that  fatigue  of  the  retina  must  be  regarded  as  fatigue  of  the 
cortex  of  the  occipital  lobe,  has  been  practically  rejected,  although  at 
one  time  Wilbrand  thought  that  it  was  worthy  of  attention.  It  has 
been  disputed  largely  because  Groenouw  and  other  observers  have  been 
unable  to  reach  the  same  results  which  he  did  in  visual  field  examina- 
tions,— that  is,  they  could  not  confirm  his  contention  that  only  homony- 
mous parts  of  the  two  visual  fields  show  contraction,  because  they  found 
always  when  one  sector  of  the  visual  field  was  fatigued  there  were 
changes  in  the  outer  limits  of  the  remaining  portions  of  the  visual 
field  of  the  same  eye. 

Concerning  the  Diagnostic  Value  of  Fatigue  Fields. — As  Wilbrand 
has  already  pointed  out,  when  he  adopted  the  term  "  nervous  asthe- 
nopia" in  place  of  "  neurasthenic  asthenopia,"  fatigue  fields  may 
accompany  any  form  of  nervous  disease.  They  are,  therefore,  not 
peculiar  to  or  diagnostic  of  neurasthenia.  Moreover,  they  are  not  con- 
fined, as  Schmidt-Rimpler  has  said,  and  as  the  investigations  of  Peters 
and  Voges  show,  to  nervous  individuals,  but  may  be  found  in  healthy 
persons.  As  we  kndw,  and  as  Arnheim  points  out,  contractions  of  the 
visual  field  may  be  found  in  diseases  without  definite  anatomical  foun- 
dation,— for  example,  in  epilepsy,  trifacial  neuralgia,  Basedow's  disease, 
heart  disease,  chlorosis,  alcoholism,  etc.  Finally,  it  must  not  be  for- 
gotten that  the  contracted  visual  field  may  be  the  result  of  malingering. 
On  this  point  something  more  will  be  said  in  a  future  paragraph. 

All  things  being  taken  into  consideration,  however,  and  not  denying 
the  foregoing  facts,  it  would  seem  proper  to  agree  with  Arnheim  that 
the  value  in  diagnosis  of  hysteria  and  neurasthenia  of  contracted  visual 
fields  is  undoubted,  although  in  many  complex  cases  it  is  frequently 
difficult  to  decide  what  the  exact  cause  of  the  narrowing  may  be. 
Wliether  one  can  make  such  a  sharp  diflFerentiation  as  von  Reuss  does, 


XEUROSES  AXD  PSYCHOSES  685 

that  fields  which  do  not  change  during  examination  and  do  not  mate- 
rially differ  under  control,  indicate  hysteria,  while  fields  which  at  first 
are  normal  but  change  into  the  variovis  fatigue  types  during  examination 
indicate  neurasthenia,  and  that  an  initial  diminution  of  the  field  which 
is  the  consequence  of  exhaustion  but  which  goes  on  increasing  during 
examination  indicates  hystero-neurasthenia,  I  am  not  prepared  to  say, 
but  of  the  definite  value  of  these  examinations  in  such  cases  and  their 
importance  as  indicators  of  a  search  for  other  symptoms  of  the  condition 
there  can  be  no  question. 

Wolff  berg  ^^^  considers  the  field  of  examination  important.  Accord- 
ing to  him,  fatigue  in  traumatic  neurosis  is  nothing  other  than  dimin- 
ished neuroptic  excitability,  and  the  diagnosis  secured  by  finding  various 
exhaustion  types  of  the  field  can  also  be  reached  by  determining  con- 
traction of  the  color  fields.  Furthermore,  even  more  important  than 
the  condition  of  the  general  field  is  that  every  part  of  the  field,  even 
that  corresponding  to  the  fovea,  is  similarly  affected  by  fatigue.  Reduc- 
tion of  the  excitability  of  the  fovea  can  be  ascertained  by  making  tests  of 
color  perception  in  the  distance.  He  concludes  that  a  field  taken  in 
ordinary  daylight,  which  in  its  limits  for  white  and  colors  corresponds  to 
those  obtained  with  a  normal  eye  in  diminished  illumination,  is  typical 
of  diminished  excitability  of  the  neuroptic  apparatus  and  of,  abnormal 
fatigue  and  hence  of  traumatic  neurosis. 

It  must  always  be  remembered  that  neurasthenics  often  exhibit  no 
change  in  their  visual  fields,  and  the  same  is  true  of  hysterics.  Indeed, 
some  authors, — for  example,  Berger  and  Topolanski  and  Hochwart, — 
almost  never  found  any  changes  in  the  visual  field  which  they  could  con- 
sider as  part  of  the  neurasthenic  process. 

The  tendency  at  the  present  time,  as  Schmidt-Rimpler  says,  is  to 
speak  of  traumatic  hysteria,  traumatic  neurasthenia,  and  traumatic 
hypochondriasis,  instead  of  using  the  general  term,  traumatic  neurosis. 
Hence,  no  attempt  has  been  made  to  separate  the  visual  phenomena 
which  pertain  to  pure  neurasthenia  from  those  which  belong  to  the 
traumatic  variety  of  the  affection,  because,  as  Oppenheim  says,  the  phe- 
nomena of  accident  neuroses,  in  so  far  as  the  visual  field  examinations 
are  concerned,  and  in  general  terms,  the  visual  symptoms,  are,  to  a  great 
extent,  identical  with  those  of  neurasthenia,  hysteria,  and  hypochon- 
driasis. Just  as  the  hysterical  visual  stigmata  are  often  more  pro- 
nounced when  traumatism  has  been  the  etiological  factor,  so  also  the 
ocular  signs  of  fatigue,  whether  they  interpret  themselves  by  the  general 

"'Loc.  cit.,  p.  206. 


686  THE    EYE    AND    NERVOUS    SYSTEM 

symptoms  of  nervous  asthenopia,  or  by  the  symptoms  which  are  par- 
ticularly elicited  by  mapping  the  visual  field,  are  often  more  decided. 

Concerning  the  differential  diagnosis  from  the  ocular  stand-point  of 
hysteria  and  neurasthenia  enough  has  been  said  in  the  preceding  pages 
and  need  not  again  be  summarized  in  the  present  paragraph,  except 
to  reiterate  that  hysteria  presents  certain  definite  ocular  stigmata,  while 
those  which  arise  in  the  course  of  neurasthenia  cannot  be  said  to  be 
peculiar  to  it,  but  represent  forms  of  visual  fatigue,  which  may  be 
produced  by  a  variety  of  nervous  affections,  although  when  taken  into 
consideration  with  other  signs,  they  have  their  definite  place  in  the 
symptom-complex  of  this  affection. 

As  John  K.  Mitchell  and  myself  "''^  have  stated,  in  the  difficult  dis- 
tinction between  certain  types  of  neurasthenic  and  hysteric  patients,  the 
presence  of  the  disturbance  in  the  color-sense  is  of  some  diagnostic  im- 
port. It  is  less  apt  to  be  present  in  the  former  than  in  the  latter,  and  yet, 
as  we  have  seen,  its  absence  is  of  little  meaning.  It  may  not  be  found  in 
many  typical  cases  of  hysteria  and  seems  to  be  present  in  others  which 
are  difficult  to  eliminate  from  the  neurasthenic  category.  Von  Frankl- 
Hochwart  and  Topolanski,  who,  as  before  pointed  out,  believe  that 
neurasthenic  patients  rarely  exhibit  concentric  contraction  of  the  field, 
and  that  their  light-sense  and  even  visual  acuity  are  usually  normal, 
have  noticed  a  certain  tendency  of  lowering  of  color  perception,  particu- 
larly in  connection  with  blue.  If  this  should  be  substantiated,  it  might 
be  considered  as  of  some  diagnostic  importance.  Wolffberg's  view  with 
reference  to  colored  central  color  perception  has  been  referred  to  (pages 
683  and  685). 

Previously  Unclassified  Neurasthenic  Ocular  Phenomena. — Cer- 
tain systemic  writers  have  described  a  neurasthenic  conjunctivitis, 
which,  however,  more  properly  should  be  regarded  as  a  hypersemia  of  the 
conjunctiva  in  persons  of  neurasthenic  condition,  and  which,  owing  to 
their  nervous  instability,  even  though  it  be  moderate  in  degree,  presents 
to  them  a  reality  far  in  excess  of  its  real  importance.  It  is  probable  that 
this  may  account  for  the  well-known  neurasthenic  symptom  of  foreign 
body  sensation  in  the  eye,  sometimes  associated  with  drooping  of  the  lid, 
to  which  reference  has  already  been  made,  a  condition  which  exactly 
simulates  eye-strain  and  which  often  is  totally  unrelieved  by  the  adjust- 
ment of  suitable  glasses,  but  disappears  when  with  the  wearing  of  these 
the  proper  physical  and  mental  hygienic  measures  are  adopted.  The 
same  is  true  of  the  pricking  and  burning  sensation  so  frequently  com- 
plained of  by  these  patients  and  located  along  the  margins  of  the  lid. 

"'  Journal  of  Nervous  and  Mental  Diseases,  January,  1894. 


NEUROSES  AXD  PSYCHOSES  687 

Ophthalmoscopic  phenomena  are  wanting,  or  if  they  are  present  in 
the  form  of  various  hypersemic  conditions  of  the  eye-ground  and  con- 
gestions of  the  margins  of  the  optic  disc,  indicate  the  signs  produced  by 
ametropia. 

A  rare  condition  is  temporary  exophthalmos,  particularly  when  the 
patient  stoops  over,  which  has  been  described  by  Oppenheim. 

TREATMENT  OF  THE  OCULAR  DISORDERS  OF 
HYSTERIA    AND    NEURASTHENIA. 

Naturally,  treatment  from  the  general  stand-point  is  indicated, 
and  as  this  belongs  to  the  domain  of  the  neurologist  and  general 
physician,  it  need  not  be  discussed  here,  but  the  eye-treatment  of  itself 
is  of  the  greatest  importance.  First  and  foremost,  there  should  be  the 
most  thorough  investigation  of  the  refraction  of  the  eyes,  and  search 
made  for  the  smallest  degrees  of  astigmatism.  Suitable  glasses  should 
be  adjusted  after  the  thorough  employment  of  atropine  or  other  suitable 
cycloplegic,  and  they  should  be  worn  constantly.  Anomalies  of  the 
ocular  muscles  require  similar  careful  consideration.  Personally,  I 
believe  that  insufficiency  of  convergence  should  be  treated  by  suitable 
gymnastic  exercises  with  prisms,  and  I  do  not  believe,  a  few  cases  ex- 
cepted, that  the  constant  wearing  of  prisms,  laterally  placed,  is  a  wise 
procedure.  Prisms,  base  in,  associated  with  reading-glasses,  may  be 
required.  On  the  other  hand,  the  symptoms  produced  by  the  various 
types  of  hyperphoria  sometimes  yield  rapidly  if  vertical  prisms  are 
combined  with  the  correcting  lenses,  or  if  a  like  result  is  produced 
by  proper  decentration.  This  is  not  the  place  to  discuss  elaborately  the 
propriety  of  surgical  interference.  I  have  often  expressed  my  belief 
in  regard  to  this  matter.  I  hold  that  surgical  interference  is  to  be 
considered  only  when  all  other  measures  have  been  long  and  faithfully 
tried  and  have  failed  to  give  relief.  While  cases  of  muscular  imbalance, 
best  treated  by  operative  interference,  are  encountered  (aptly  called  by 
Risley,  absolute  insufficiencies,  equivalent  to  the  structural  and  inser- 
tional  anomalies  of  Duane),  in  my  opinion  they  represent  a  limited  por- 
tion of  the  whole  number.  Moreover,  as  our  knowledge  of  the  etiology 
of  the  abnormalities  of  muscular  balance  increases,  our  methods  of  non- 
surgical treatment  improve,  and  this  number  grows  steadily  smaller. 

The  point,  however,  should  be  emphasized,  that  refractive  errors 
and  anomalies  of  muscular  motility  are  frequently  the  cause  of  the  entire 
hysteric  and  neurasthenic  outbreak,  and  therefore  should  be  among  the 
first  problems  to  be  carefully  investigated  in  the  management  of  these 
cases.     This  does  not  mean  that  ocular  treatment  should  be  utilized  to 


688  THE    EYE    AND    NERVOUS    SYSTEM 

the  exclusion  of  general  measures.  To  maintain  this  would  be  absurd, 
but  it  is  equally  absurd  to  attempt  cures  in  many  of  these  cases  without 
consideration  of  the  constantly  associated  disturbances  of  refraction, 
accommodation,  and  muscular  balance.  Moreover,  it  is  a  distinct  mis- 
take to  put  these  patients  through  long  periods  of  so-called  rest-cure,  as 
William  Thomson  long  ago  pointed  out,  without  the  relief  which  neces- 
sarily must  come  from  the  restoration  of  normality  to  the  eyes.  I  do 
not  doubt  that  many  a  rest-cure  would  be  materially  shortened  if  this 
plan  were  adopted.  Because  a  patient  lies  in  bed  or  rests  in  his  room, 
there  is  no  reason  why  he  should  continue  to  submit  to  the  plague  of 
eye-strain,  which,  while  it  may  not  be  as  great  as  it  is  in  the  presence 
of  sustained  close  eye-work,  is  constantly  in  evidence  so  long  as  ametropia 
•eyes  are  utilized  in  the  effort  of  sight. 

This  has  been  fully  recognized  for  years  by  many  neurologists,  and 
accounts  for  many  brilliant  results  which  have  been  obtained  in  the  mod- 
ern treatment  of  functional  nervous  diseases.  Unfortunately  eye-strain 
has  been  brought  into  etiological  relation  with  a  number  of  nervous 
diseases  with  which  it  has  no  such  concern  by  certain  injudicious 
writings,  and  consequently  some  physicians  have  been  led  to  believe 
that  its  influence  has  been  exaggerated.  This  deduction  is  regrettable 
iind  has  induced  them  to  neglect  the  potency  of  eye-strain  in  the  pro- 
duction and  fostering  of  many  neurasthenic  and  hysteric  phenomena. 

Because  photophobia  is  a  frequent  symptom  in  functional  nervous 
diseases,  tinted  glasses  have  been  recommended.  They  should  be  used 
with  caution,  although  in  some  cases  excellent  results  follow  the  adjust- 
ment of  lenses  which  correct  the  refractive  error,  and  which  have  been 
slightly  tinted  or  manufactured  of  Arundel  or  lightly  smoked  glass. 
Division  of  the  supraorbital  nerve  to  relieve  blepharospasm  has  been 
recommended  (von  Graefe,  Panas)  and  practised.  It  would  seem  to  be 
an  unnecessary  procedure  and  probably  acts,  as  Parinaud  says,  by  sug- 
^stion.  Indeed,  many  of  these  patients  readily  accept  suggestion,  and 
may  at  times  be  relieved  by  this  means,  hence  the  success  of  Christian 
Science  and  certain  "  mind  cures."  For  the  same  reason  magnets  have 
been  employed  and  their  use  followed  by  the  disappearance  of  the 
amblyopia  and  other  signs  of  ocular  hysteria. 

THE    OCULAR    MANIFESTATIONS    OF    HYPOCHONDRIASIS. 

There  are  no  ocular  symptoms  characteristic  of  hypochondriasis.  On 
the  other  hand,  some  perfectly  trivial  ocular  complaint  may  be  one 
upon  which  the  morbid  fancy  of  the  patient  centres;    for  example,  a 


NEUROSES  AND  PSYCHOSES  689 

mild  conjunctivitis  or  a  recurring  hjpersemia  of  the  conjunctiva  may 
cause  him  to  go  from  one  consulting-room  to  the  other,  hoping  for  relief 
and  believing  in  all  manner  of  evil  consequences.  The  same  is  true  if  he 
once  becomes  conscious  of  the  shadows  which  the  formed  elements  of 
the  vitreous  cast  upon  the  retina  and  to  which  we  give  the  name  muscae 
volitantes.  They  are  not  caused  by  hypochondriasis  nor  are  they  a 
symptom  in  one  sense,  but  they  are  a  condition  which  may  greatly 
aggravate  the  mental  disturbance.  Long-standing  hypochondriasis  may 
produce  contraction  of  the  visual  field,  and  phenomena  which  have  been 
described  in  general  terms  as  fatigue  fields,  or  exhaustion  fields,  just  as 
they  occur  in  neurasthenic  patients. 

A  matter  of  some  importance  is  the  effect  of  presbyopia  on  these 
patients.  It  seems  to  me  that  there  is  no  doubt,  and  I  find  that  this  is 
the  opinion  of  some  accurate  neurologists,  that  neglected  presbyopia  or  a 
failure  of  accommodation,  for  which  no  adequate  relief  is  sought,  even 
hefore  the  presbyopic  age,  is  frequently  the  cause  of  hypochondriasis 
and  mild  forms  of  melancholia.  The  patients  cannot  be  made  to 
understand  that  it  is  the  physiological  failure  of  accommodative  power, 
and  believe  that  some  form  of  visual  disturbance  is  arising  which  later 
will  eventuate  in  blindness.  The  irritating  inconvenience  of  oncoming 
and  increasing  presbyopia  doubtless  aggravate  these  mental  conditions 
under  many  circumstances.  That  eye-strain  in  general  may  produce 
hypochondriasis  is  well  known. 

OCULAR   MANIFESTATIONS    OF   HYPNOTISM   AND    SLEEP. 

Pansier  ^ '  ^  has  devoted  a  chapter  to  the  ocular  conditions  during 
hypnosis  and  the  action  of  suggestion  on  the  visual  function. 

During  hypnotic  sleep  the  eyelids  are  usually  closed.  They  can, 
however,  be  found  partly  or  sometimes  completely  open,  and  the  eyes 
may  be  rotated  upward  and  the  pupils  dilated  and  sluggish. 

Although  some  writers  have  stated  that  hypnotic  hyperaesthesia  may 
be  concerned  with  all  the  senses  except  the  sense  of  vision,  this  is  evi- 
dently not  an  accurate  statement,  and  more  than  one  author  has  observed 
increased  visual  power  in  the  hypnotic  state,  even  when  the  lids  seem 
closed,  because  the  patient  looks  through  a  scarcely  perceptible  slit  in 
the  palpebral  fissure.  Pansier  maintains  that  there  is  a  hyperaesthetic 
condition  of  the  retina,  which  under  these  circumstances  has  the  effect  of 
rendering  it  more  sensitive  to  the  rays  of  light.    The  same  author  quotes 


"'Les    Manifestations    oculaires    de    I'Hyst^rie.     Oeil    Hyst^rique,    Paris,    1892, 
p.  31. 

44 


690  THE    EYE    AND    NERVOUS    SYSTEM 

Binet  as  having  observed  in  a  subject  passing  from  the  normal  to  the 
hypnotic  state  a  decided  augmentation  of  visual  acuity. 

Koenigshofer  ^  ^®  states  that  in  so  far  as  accommodation  is  con- 
cerned for  vision  at  a  distance,  there  appears  to  be  a  relaxation  of  the 
ciliary  muscle.  In  effect  the  hyperope  seems  to  have  accentuated 
his  ametropia,  the  myope,  on  the  contrary,  to  have  diminished  it.  Now 
it  is  an  interesting  fact  first  noted  by  Heidenheim  that  spasm  of  accom- 
modation is  one  of  the  first  signs  of  beginning  hypnosis,  and  therefore 
distant  vision  would  be  diminished  because  of  the  fictitious  myopia 
which  is  produced.  Hence,  should  there  be  at  a  later  stage  of  the  hyp- 
nosis, relaxation  as  has  just  been  stated,  of  the  muscle  of  accommodation, 
the  patient  would  suddenly  find  after  his  return  to  consciousness,  par- 
ticularly if  he  was  a  hysteric,  that  the  visual  acuity  was  suddenly 
augmented.  Pansier  quotes  Sciamina  and  Parisotti  as  having  found 
in  their  observations  a  constant  augmentation  of  refraction. 

Knies,  on  the  authority  of  Tamborini  and  Sepilli,  states  that  the 
pupil  during  the  cataleptic  state  of  hypnosis  is  dilated  and  does  not 
react  to  light,  and  quotes  Fere  as  describing  dilation  of  the  pupil 
during  cataleptic  sleep  if  the  individual  is  directed  to  look  at  a  distant 
object,  but  contraction  of  it  if  he  looks  at  a  near  object.  This  has  been 
proved  to  be  the  case  in  a  number  of  other  experiments  in  hypnotism. 

According  to  Koenigshofer,  pupillary  reflexes  are  produced  more 
slowly  in  the  hypnotic  than  in  the  normal  state.  Berger  has  caused 
dilation  of  the  pupil  after  strong  sensory  irritation  in  the  most  pro- 
nounced hypnotic  sleep. 

A  number  of  observers  have  studied  the  visual  field  during  the 
various  phases  of  hypnotism  and  have  found  a  dilation  of  the  visual 
field  in  the  somnambulistic  stage.  Pansier  records  a  number  of  in- 
stances of  this  character, — for  example,  a  marked  restriction  of  the 
visual  field  during  waking,  while  during  hypnosis  it  widened  out 
almost  to  normal.  The  visual  field  for  colors  in  one  of  his  cases  was 
also  enlarged,  but  the  colored  circles  (the  patient  was  a  hysteric)  pre- 
served their  characteristic  place  and  inversion  within  the  limits  of  the 
field. 

Moravcsik,'^*^  after  pointing  out  that  a  contracted  visual  field  may  be 
much  widened  in  hysterical  patients  by  certain  irritations, — for  example, 
pouring  ether  on  the  hand,  touching  the  tongue  with  salt,  soimding  a 
loud    tuning-fork    in    the    ear, — demonstrated    in    a    hypnotic    patient 


"*Klin.  Monatsbl.  f.  Augenheilk.,  xxvi.,  1888,  p.  13. 
>»  Neurolog.  Centralbl.,  1899,  p.  230. 


l^EUROSES  AND  PSYCHOSES  691 

that  the  width  of  the  field  may  vary  according  to  suggestion.  I  have 
studied  the  visual  field  during  hypnotism,  and  in  one  case  found  it 
markedly  contracted. 

The  ophthalmoscopic  conditions  during  hypnotism  have  been  investi- 
gated by  several  observers.  In  the  beginning  of  hypnotism  Forster 
found  the  fundus  normal.  I  have  had  only  slight  opportunities  of 
making  observations  under  these  conditions,  but  in  Weir  Mitchell's 
clinic  in  the  Orthopaedic  Hospital  have  not  found  any  alterations  of 
significance,  although,  as  is  well  known,  in  the  later  stages  of  hypno- 
tism, as  particularly  has  been  demonstrated  by  the  researches  of  Luys 
and  Bacchi,  the  retinal  veins  may  be  much  dilated,  and  this  state  of 
passive  hypera^mia  persisted  during  the  stage  of  catalepsy.  The  same 
phenomena  were  observed  in  the  phase  of  fascination.  In  the  cataleptic 
stage  the  iris  was  immobile ;  in  the  somnambulistic  stage  it  reacted  to 
light,  and  at  this  time  the  arteries  and  veins  were  increased  in  volume. 

In  so  far  as  the  action  of  suggestion  on  the  visual  function  is  con- 
cerned, it  may  be  stated  that  all  the  symptoms  of  hysteria  may  be  pro- 
duced by  suggestion,  and  in  general  terms  it  may  be  said  that  both 
binocular  and  monocular  blindness  has  been  produced  by  suggestion,  as  is 
especially  evidenced  by  the  investigations  of  Fontan.  The  dilatation  of 
the  visual  fields  which  occurs  under  the  same  condition  has  already  been 
referred  to.  Koenigshofer  by  his  experiments  confirmed  the  statement 
that  hypnosis  can  produce  amblyopia  and  amaurosis  and  all  hallucina- 
tions of  vision,  but  the  opinion  maintained  by  several  authors  that  color- 
blindness can  thus  be  produced  is,  according  to  him,  an  error.  He 
maintains  that  it  is  not  the  impression  produced  by  the  color  which  is 
neutralized,  but  it  is  the  designation  of  the  color, — that  is,  the  idea  of 
red  or  green,  for  example,  which  is  abolished. 

As  already  pointed  out,  the  condition  of  the  pupil  and  accommoda- 
tion varies  according  as  the  suggestion  is  made  to  the  subject  of  viewing 
objects  which  are  near  or  far  away.  This  augmentation  of  accommo- 
dation has  been  proved  by  Pansier  by  keratoscopic  examinations  to  occur 
during  suggestion  to  the  subject  that  he  shall  look  at  some  object  close  at 
hand,  while  if  the  suggestion  is  made  that  the  object  is  now  removed 
to  a  long  distance,  there  is  relaxation  of  accommodation  and  a  replace- 
ment of  the  visual  axes  from  convergence  to  parallelism. 

The  action  of  suggestion  on  the  ocular  muscles  and  the  artificial 
reproduction  of  contractures  of  the  muscles  has  been  studied  by  Fontan 
and  particularly  by  Borel,  and  in  this  manner  ptosis,  strabismus,  both 
convergent  and  divergent,  have  been  brought  into  existence.  Divergent 
strabismus  is  much  more  difficult  to  produce  than  the  convergent  variety 


692  THE    EYE    AND    NERVOUS    SYSTEM 

of  the  affection,  and  it  is  said  that  even  an  immobilization  of  the  eye  in 
tlie  condition  of  conjugate  deviation  has  been  created  by  similar  methods. 

The  condition  of  the  eyes  during  ordinary  sleep  may  now  receive 
brief  notice.  Sleep  begins  with  closure  of  the  lids,  or,  in  other  words, 
with  ptosis.  This  sleep-ptosis  Mauthner  regarded  as  representing  a 
temporary  nuclear  paralysis  of  the  levator.  Inasmuch,  however,  as 
there  is  relaxation  of  the  tonus  of  the  sympathetic  fibres  which  pass  to 
the  blood-vessels  during  sleep,  Knies  and  other  observers  consider  that 
the  drooping  of  the  lids  is  also  an  evidence  of  a  paresis  or,  at  least,  an 
insufficient  action  of  the  sympathetic  nerve.  This  is  further  confirmed 
by  the  fact  that  during  sleep  the  pupils  are  contracted,  while  when  the 
lids  are  simply  closed  during  waking  they  are  dilated.  In  other  words, 
together  the  two  symptoms  of  drooping  of  the  lids  and  contraction  of  the 
pupils  would  produce  the  phenomenon,  as  Knies  puts  it,  of  sympathetic 
ptosis  with  myosis. 

The  visual  axes  are  divergent — that  is  to  say,  during  sleep  there  is  a 
relaxation  of  convergence-innervation — and  the  eyes,  to  use  the  language 
of  Hansen-Grut,  take  up  their  anatomical  position  of  rest. 

Ophthalmoscopic  examination  of  sleeping  patients  would  be  ex- 
tremely difficult  to  make  except  under  unusual  conditions.  So  far  as  I 
am  aware,  nothing  of  interest  has  been  developed  in  this  line  of  examina- 
tion, although  it  is  possible  that  there  might  be  some  change  noted  in  the 
calibre  of  the  retinal  vessels. 

TESTS    FOR    DETECTING    PRETENDED    AMBLYOPIA 
(MALINGERING). 

In  order  to  investigate  the  various  amblyopias  which  occur  in  hys- 
teric patients  it  is  necessary  to  apply  the  tests  which  are  ordinarily  used 
for  detecting  malingering.  To  a  certain  extent  they  have  been  referred 
to  in  the  preceding  pages.  The  subject  has  been  especially  investigated 
by  Professor  S.  Baudry,^^^  to  whose  writings  those  interested  in  the 
great  variety  of  tests  which  have  been  devised  and  which  often  are 
extremely  useful,  especially  in  medico-legal  cases  and  in  work  connected 
with  the  examination  of  recruits  for  the  army  and  navy,  should  turn. 
Evidently  for  practical  purposes  it  is  sufficient  to  briefly  describe  a  few 
that  are  in  common  practice. 

The  information  which  may  be  gained  by  testing  the  pupils  in  their 
reactions  is  elsewhere  described  (see  Chapter  VI),  and  we  are  therefore 
concerned  for  the  moment  with  simulated  blindness  of  one  eye,  simu- 


"'  Simulated  Blindness,  System  of  Diseases  of  the  Eye,  edited  by  Norris  and 

Oliver,  vol.  iv.  p.  861. 


:N"EUE0SES  A:N^D  psychoses  693 

lated  blindness  of  both  eyes,  and  simulated  contractions  of  the  visual 
field. 

Simulated  Blindness  of  One  Eye — To  detect  this  condition  the  fol- 
lowing methods  may  be  used  : 

(1)  The  Diplopia  Test. — This  is  performed  in  the  usual  manner  of 
the  ordinary  examinations  of  the  external  ocular  muscles  at  5  metres, 
and  a  T  degree  prism  is  placed  before  the  admittedly  sound  eye.  If 
now  superimposed  double  images  are  acknowledged,  there  is  binocular 
vision  and  the  fraud  is  detected.  The  examiner  may  vary  the  test  by 
placing  the  prism  before  the  supposed  blind  eye,  either  base  up  or 
base  down. 

(2)  Harlan's  Test. — This  is  an  extremely  useful  and  simple  device 
and  is  performed  as  follows :  Place  in  position  an  ordinary  trial  frame 
and  put  before  the  admittedly  sound  eye  a  high  convex  glass  (-|-  16D) 
and  before  the  eye  which  is  claimed  to  be  blind  a  plane  glass  or  a  weak 
concave  spheric  ( — .25D),  which  will  not  interfere  with  vision.  If 
the  proper  letters  placed  at  a  distance  of  5  metres  are  read,  the  act  of 
reading  must  have  been  done  by  the  eye  which  was  claimed  to  be  sight- 
less, inasmuch  as  vision  at  that  distance  with  the  other  eye  is  excluded 
by  the  presence  of  the  high  convex  lens.  The  test  may  be  further 
elaborated  by  covering  the  pretended  blind  eye  and  requesting  the 
patient  to  read  the  letters.    If  he  is  unable  to  do  so  the  fraud  is  exposed. 

(3)  Baudry's  Test  with  Monocular  Diplopia. — Inasmuch  as  Graefe's 
method  of  producing  monocular  succeeded  by  binocular  diplopia  by 
means  of  a  prism  is  rather  difficult  of  application,  Baudry  has  invented 
an  apparatus  which  consists  of  a  prism  supplemented  at  its  base  by  the 
juxtaposition  of  a  piece  of  plane  glass  equal  in  thickness  to  the  width  of 
the  base ;  the  prism  itself  is  divided  near  its  centre  by  a  section  parallel 
to  the  base ;  it  thus  consists  of  three  pieces,  the  whole  shaped  like  a 
piece  of  the  edge  of  bevelled  glass.  The  apparatus  is  mounted  in  a 
brass  cell,  covered  on  both  sides.  The  cover  is  pierced  on  each  side  by  a 
central  opening  of  6  and  3  millimetres  apertures  respectively.  By  a 
simple  mechanism  the  three  pieces  of  glass  slide  in  the  mounting,  so  that 
one  or  other  of  the  two  dividing  lines  with  the  parts  of  the  glass  adjacent 
may  thus  be  placed  before  the  pupil  of  the  seeing  eye, — in  other  words, 
either  the  base  of  the  prism  and  the  piece  of  plane  glass  adjacent  or  the 
two  pieces  of  the  prism  itself.  Inasmuch  as  the  two  dividing  lines  and 
adjacent  parts  look  exactly  alike,  monocular  and  binocular  diplopia 
can  be  produced  with  the  greatest  ease  and  without  the  subject's  know- 
ing, even  if  acquainted  with  the  apparatus,  what  variety  of  diplopia 
(whether  binocular  or  monocular)  is  present  at  any  given  instant. 


694  THE    EYE    A:N^D    NERVOUS    SYSTEM 

• 
The  method  of  using  the  apparatus  is  simple.  The  eye  that  is 
alleged  to  be  blind  is  lightly  covered  by  the  examiner's  hand,  and  the 
subject  asked  to  look  at  the  test  flame  2  or  3  metres  distant  and  covered 
with  the  dark-red  glass.  The  apparatus  is  then  placed  before  the  seeing 
eye,  so  as  to  bring  the  dividing  line  between  the  base  of  the  prism  and 
the  piece  of  plane  glass  into  coincidence  with  the  horizontal  diameter  of 
the  pupil.  The  patient  sees  two  lights.  The  instrument  is  now  re- 
moved and  adjusted,  without  his  knowledge,  so  as  to  bring  the  dividing 
line  in  the  prism  itself  before  the  central  aperture  in  the  metal  cell, 
and  placed  again  before  the  seeing  eye,  the  other  eye  being  left  uncov- 
ered, apparently  and  unintentionally.  If  the  patient  now  sees  double 
he  is  convicted,  for  the  diplopia  is  binocular.  If  the  simulator  stub- 
bornly denies  the  existence  of  diplopia  at  first,  both  phases  of  the  pro- 
cedure may  be  repeated  in  reversed  succession.  ^^^ 

(4)  Tests  with  Colored  Glasses  and  Letters. — These  are  numerous. 
The  one  generally  employed,  or  some  modification  of  it,  is  known  as 
Snellen^  method.  In  general  terms  the  patient  is  required  to  look  at 
alternate  red  and  green  letters.  The  admittedly  sound  eye  is  now 
covered  with  a  red  glass,  and  if  the  green  letters  are  read  evidence  of 
fraud  is  present.  Instead  of  a  red  glass  a  green  glass  may  be  used, 
through  which  the  red  letters  would  be  invisible. 

Very  ingenious  letters  based  upon  the  fact  that  red  upon  a  white 
background  viewed  through  a  red  glass  disappears,  and  viewed  through 
a  green  glass  appears  black,  have  been  designed  by  Dr.  von  Hasel- 
berg.^^'"^    I  have  found  these  very  useful. 

(5)  Tests  with  Stereoscopes. — Any  of  the  ordinary  stereoscopes  may 
be  utilized  for  this  purpose  and  in  the  usual  manner  tests  for  binocular 
vision  applied.  For  this  purpose  Worth's  amblyoscope,  and  especially 
its  modification  by  Black,  should  prove  useful,  on  account  of  the  ease 
with  which  it  is  adjusted  to  suit  the  position  of  the  eyes  and  the  condi- 
tion of  the  ocular  muscles.  Any  refractive  error  may  be  previously  elim- 
inated by  adjusting  suitable  glasses,  which  are  determined  by  the 
shadow-test.  With  this  instrument  full  investigation  of  simultaneous 
vision  of  dissimilar  objects,  true  fusion  and  the  sense  of  perspective  can 
be  investigated,  and  its  application  to  pretended  blindness  of  one  eye 
would  be  very  easy. 

(6)  Tests  by  Pseudoscopic  Apparatus. — These  depend  upon  the  prop- 


•^  Description  taken  from  T.  P.  Schneideman's  review  of  Baudry's  paper,  Oph- 
thalmic Review,  xvii.,  1898. 

•=°Archiv  f.  Augenheilk.,  xlviii.  1901. 


IS^EUROSES  AND  PSYCHOSES  695 

erties  of  a  plane  mirror, — that  is,  the  malingerer  is  induced  to  read  let- 
ters which  he  helieves  he  is  seeing  with  the  admittedly  sound  eye,  when 
really  he  is  looking  at  them  with  the  eye  which  he  maintains  is  blind. 
An  instrument  of  this  character  known  as  the  box  of  Fles  is  much  used 
abroad,  and  has  consequently  been  employed  in  investigating  the  eyes  of 
hysterical  patients.  It  has  been  modified  in  many  ways  by  a  number  of 
investigators.  In  our  own  country  an  ingenious  test  based  upon  this 
principle  has  been  designed  by  Fridenberg,  and  is  known  as  Fridenberg^s 
inirror  test. 

Tests  for  Simulated  Blindness  of  Both  Eyes. — Naturally,  it  is 
much  more  difiicult  to  detect  a  malingerer  who  claims  to  be  blind  in 
both  eyes  than  one  who  maintains  blindness  of  one  eye  alone.  The 
tests  which  have  been  described  are  inapplicable,  and  formerly  it  used 
to  be  stated  that  the  only  satisfactory  means  of  detecting  the  fraud 
was  by  placing  a  careful  watch  over  the  patient  and  taking  him  un- 
awares. The  contraction  of  the  pupil  on  exposure  to  light  under  such 
circumstances  would  not  prove  that  there  is  sight  in  the  eye,  because, 
as  Swanzy  points  out,  a  lesion  of  the  centre  of  vision,  or  in  the  course 
of  the  fibres  connecting  this  centre  with  the  corpora  quadrigemina,  would 
produce  absolute  blindness,  and  would  still  permit  an  imperfect  reaction 
of  the  pupil  to  light. 

Priestley  Smith  and  Edward  Jackson  have  suggested  the  following 
simple  test  for  feigned  binocular  blindness :  Place  a  lighted  candle  in 
front  of  the  subject.  Now  hold  a  6-degree  prism  base  out  before  one 
eye.  If  both  eyes  see,  the  one  behind  the  prism  will  move  inward  and 
on  removing  the  prism  will  move  outward,  the  other  eye  remaining 
fixed. 

Tests  for  Simulation  of  Changes  in  the  Visual  Field. — According 
to  Koenig,  no  case  has  yet  been  published  in  which  it  has  been  proved 
that  any  one  has  succeeded  in  continuously  deceiving  an  experienced 
physician  by  simulating  concentric  restriction  of  the  visual  field.  Koe- 
nig's  experiments  on  this  subject  are  very  interesting.^^^  He  succeeded 
in  simulating  a  concentric  restriction  of  the  visual  field  without  fatigue 
phenomena  to  the  extent  of  about  45  to  50  degrees  on  the  temporal  side, 
but  when  he  attempted  to  simulate  a  larger  visual  field  the  irregularities 
became  great.  He  was  never  able  to  simulate  a  visual  field  with  only 
moderate  constriction.  Similar  opinions  are  held  by  Wilbrand  and 
Saenger.  Simulation  of  simple  concentric  constriction  of  the  visual 
field  of  high  degree  may  be  possible  even  without  preliminary  practice 
with  the  perimeter,  but  continuous  deception  would  be  difficult, 

"*  Ueber  Gesiclitsfeld-Ermiidung,  1893,  p.  131. 


696  THE    EYE    AND    NERVOUS    SYSTEM 

Detection  of  simulated  visual  field  phenomena  could  be  secured  by 
varying  the  examination  with  the  campimeter  and  the  perimeter,  and 
especially  by  altering  the  point  of  fixation,  as  the  malingerer  would  not 
likely  be  aware  of  the  changes  in  the  size  of  the  visual  field  which  must 
take  place  under  these  circumstances.  In  like  manner,  the  tests  with 
different  sized  test-objects  would  be  sure  to  bring  about  detection. 
Baudry  describes  Schmidt-Rimpler's  method  as  follows:  WTiile  the 
test-object  is  situated  on  the  limit  of  the  field  of  vision  of  one  eye,  but 
is  still  seen  by  the  patient,  a  prism  that  is  too  strong  to  be  overcome  is 
placed  before  this  eye,  both  eyes  being  open.  It  is  necessary  to  ascer- 
tain before  the  adjustment  of  the  prism  whether  the  test-object  is  seen 
by  both  eyes,  or  by  one  only.  In  the  former  case,  if  there  has  been 
simulation,  the  patient  would  have  stated  that  he  saw  two  objects,  the 
true  and  the  false  image.  If,  on  the  contrary,  he  had  been  honest,  he 
would  have  seen  but  one  object.  If  originally  the  object  is  seen  by  one 
eye  only,  then  if  the  patient  is  truthful,  the  prism  placed  before  this 
eye  makes  the  object  disappear;  the  patient  sees  nothing.  In  case  of 
simulation  the  object  remains  visible. 

Other  methods  of  taking  the  field  of  vision  will  naturally  suggest 
themselves.  For  example,  Koenig's  method  in  which,  instead  of  examin- 
ing the  individual  meridians  in  the  usual  way,  the  examiner  first  picks 
out  one  and  then  another  at  random,  thus  rendering  it  difficult  for  the 
subject  to  remember  the  same  point,  as  the  point  where  the  object  enters 
the  visual  field.  As  already  noted,  von  Reuss  believes  that  his  fatigue 
spirals,  if  present,  are  a  real  evidence  of  the  absence  of  malingering, 
because  they  probably  would  be  entirely  unknown  to  the  individual  who 
was  being  examined.  The  capacity  of  the  eye  to  recuperate  has  been 
recommended  as  a  test  for  malingering.  The  principle  of  the  method, 
according  to  Arnheim,  is  that  an  eye  after  nervous  fatigue  requires  a 
greater  time  for  its  recovery  than  one  that  has  become  fatigued  normally. 
For  example,  if  a  person  whose  visual  field  by  diffused  daylight  ex- 
hibits a  high  degree  of  concentric  narrowing  very  soon  presents  a  normal 
field  in  a  dark  room, — that  is,  recovers  rapidly, — the  original  statement 
should  be  rejected  as  false.  Simulation  of  the  visual  field  is  the  sub- 
ject of  a  long  research  by  Bichelonne.^^" 

Simulation  of  partial  achromatopsia  and  of  dyschromatopsia  can 
best  be  detected,  according  to  Baudry,  by  means  of  Stillings'  isochro- 
matic  tables,  or  even  better,  by  those  of  Pflueger. 

'"Annales  d'Oculistique,  cxxix.,  1903,  p.  252. 


CHAPTER    XVII. 

MIGRATE,    TETANUS,  TETANY,  EPILEPSY,  BLINDNESS  DUE 
TO  UE^MIA  AND  OTHER  BLOOD  STATES.* 

By  JAMES  HENDRIE  LLOYD,  A.M.,  M.D. 

MIGRAINE, 

Definition. — Migraine  (Megrim,  Ilemicrania,  or  Sick-Headache) 
is  a  neurosis,  characterized  by  pain  in  the  fifth  nerve,  by  affections  of 
vision,  and  by  gastric  irritation.  These  three  groups  of  symptoms  may 
be  said  to  constitute  the  tripod  upon  which  the  disease  rests.  In  its 
simple  or  typical  form  they  are  always  present,  and  they  appear  in  a 
certain  order  of  evolution.  Moreover,  migraine  is  a  periodical  affection,, 
although  its  periods  do  not,  as  a  rule,  appear  with  regularity.  Its  onset 
is  usually  sudden ;  so  sudden,  in  fact,  that,  like  epilepsy,  migraine  is 
sometimes  called  one  of  the  explosive  neuroses. 

As  the  object  of  this  article  is  to  describe  especially  the  ocular,  or 
ophthalmic,  symptoms  of  migraine,  these  symptoms  will  be  given  special 
prominence;  but  at  the  same  time  the  other  classical  symptoms  of  the 
disease  will  be  stated  briefly  in  order  that  the  account  may  not  lack 
symmetry. 

Varieties  of  Migraine. — The  disease  has  a  variety  of  forms,  some 
of  w'hich  have  been  recognized  only  in  recent  years.  It  is  significant 
that  the  two  chief  varieties  are  named  after  the  ocular  symptoms.  First 
of  these  is  the  ordinary  or  classical  migraine,  which  has  already  been 
defined,  and  which  is  now  often  called  Ophthalmic  migraine.  In  this 
form  the  affection  of  vision  assumes  special  prominence;  with  some 
well-marked  variations  in  a  few  cases  from  the  ordinary.  Second,  Oph- 
thalmoplegic migraine,  in  which  a  new  and  distinct  element  is  present, 
— namely,  a  paralysis  of  the  third  nerve.  Third,  Psychical  migraine,  in 
which  mental  symptoms  predominate  in  place  of  the  usual  ocular  and 
neuralgic  ones.  In  addition  to  these  main  varieties  there  are  aberrent 
or  abortive  types,  which,  however,  can  best  be  described  under  one  or 
other  of  the  main  groups. 

Causes. — Migraine  has  been  known  and  studied  for  many  years. 
In  fact,  it  is  one  of  the  most  familiar  of  nervous  diseases,  and  there  is 


*  In  the  preparation  of  this  chapter  tlie  author  has  to  acknowledge  the  valuable 
assistance  of  Dr.  C.  D.  Camp,  especially  in  the  literary  research. 

697 


698  THE    EYE    AND    NERVOUS    SYSTEM 

a  very  abundant  literature  upon  it.^  In  spite  of  this  fact,  the  essential 
causation  and  patholog}'  of  the  disease  are  quite  unknown,  and  much 
that  is  merely  speculative  is  written  about  these  aspects  of  it. 

Sex  is  probably  not  a  determinative  factor  of  great  importance, 
although  some  authors,  among  whom  is  Eulenburg,  contend  that  mi- 
graine is  more  common  in  women  than  in  men.^  But  Sinkler  has  well 
remarked  that  this  may  be  due  to  the  fact  that  men  do  not  seek  treat- 
ment for  the  disease  as  persistently  as  do  women.  A  far  more  important 
factor  is  age.  In  the  great  majority  of  cases  the  disease  makes  its 
appearance  before  or  about  puberty.^  This,  in  truth,  is  only  the  expres- 
sion of  another  law, — namely,  that  migraine  is  usually  an  hereditary 
affection.  The  earlier  the  disease  takes  origin  the  worse  and  more 
inveterate  it  is.  Cases  in  which  the  onset  is  delayed  until  after  puberty 
are  not,  as  a  rule,  so  severe,  and  some  authors  have  contended  that 
migraine  never  begins  in  full  adult  life.  But  the  statement  by  Tissot 
that  the  disease  never  begins  after  the  age  of  25  years  may  be  open  to 
question.  Xevertheless,  it  is  a  rule  that  the  longer  it  is  delayed  the  less 
severe  it  is  likely  to  be. 

Heredity  is  the  most  important  factor  in  the  causation  of  migraine. 
This  law  is  manifest  in  a  variety  of  ways.  For  instance,  the  heredity 
may  be  direct  or  by  transformation.  In  the  first  mode,  the  transmission 
is  from  one  or  more  migrainous  ancestors,  and  may  be  shown  in  either 
a  lineal  or  collateral  relation.  Thus  the  patient  may  acquire  the  disease 
from  a  parent  or  grandparent,  in  which  case  the  descent  is  lineal ;  or 
it  may  appear  collaterally,  as  in  an  aunt  and  nephew,  or  as  in  the  case 
reported  by  Chabbert,^  in  which  the  disease  showed  itself  in  two  sisters. 
The  descent  is  said  to  be  by  transformation  when  the  patient  comes  of  a 
neurotic  stock,  in  which  other  nervous  diseases  than  migraine  are  prom- 
inent in  the  ancestry,  as  in  Sieffer's  case,^  in  which  the  disease  appeared 
in  two  children  of  an  insane  mother  and  an  alcoholic  father.  This 
method  of  inheritance  by  transformation  is  not  uncommon  in  other 
nervous  diseases,  as  in  epilepsy,  hysteria,  and  insanity.  When  the 
family  stock  is  neurotic,  a  nervous  disorder  may,  as  it  were,  be  ingrafted 

'  Liveing,  On  Megrim,  Sick  Headache,  and  Some  Allied  Disorders,  London,  1873. 
Eulenburg,  Ziemssen's  Cyclopaedia,  vol.  xiv.  p.  5.  Sinkler,  Migraine,  in  Pepper's 
System  of  Medicine,  vol.  v.  p.  406. 

'  Eulenburg  claims  that  5  to  1  is  the  proportion  of  women  to  men ;  a  statement 
which  could  only  be  tested  by  elaborate  statistics. 

'The  present  writer,  who  is  a  victim  of  migraine,  can  recall  attacks  in  the  very 
earliest  period  of  childhood  to  which  his  memory  extends. 

'Le  Progres  Med.,  1895,  p.  241. 

» Berl.  klin.  Woch.,  1900,  No.  30,  p.  657. 


MIGKAI:N^E  699 

upon  it  instead  of  being  directly  inherited.^  Mobius  claims  that  90 
per  cent,  of  the  cases  of  migraine  show  heredity,  and  the  disease  has 
been  known  to  descend  through  four  generations,  and  to  show  itself  in 
eight  members  of  the  same  household."^  Gowers  ^  calls  attention  to,  and 
gives  instances  of,  the  association  of  migraine  with  other  nervous  dis- 
eases in  the  same  family,  especially  with  epilepsy  and  pure  neuralgia. 
Its  relationship  with  gout  has  probably  been  exaggerated,  especially  by 
physicians  who  practice  among  an  old  and  gouty  population,  and  see 
gout  in  everything,  and  possibly  have  it  themselves.  Trousseau  said 
that  "  migraine  and  gout  are  sisters ;"  but  the  kinship  is  not  easily 
recognized  in  America. 

There  is  more  reason  to  suppose  that  migraine  may  be  related  to 
gastro-intestinal  disorders.  This  is  in  part  a  popular  notion,  due  to 
the  gastric  irritation  which  is  one  of  the  prominent  symptoms  in  many 
cases,  and  which  is  not  so  much  a  cavise  as  an  effect  of  the  disease.  But 
there  is  some  scientific  ground  for  the  belief :  attacks  may  sometimes  be 
aborted  by  prompt  action  on  the  intestinal  tract ;  and  the  idea  is  in 
accord  with  the  opinion  that  migraine  is  due  to  an  autoinfection.  In 
some  cases  as  soon  as  vomiting  occurs  in  the  ordinary  evolution  of  that 
symptom,  relief  begins,  and  may  even  be  prompt. 

The  action  of  overwork,  overstrain,  or  overstudy  may  be  contribu- 
tory to  attacks  by  reducing  strength,  and  impairing  the  resistive  power 
of  the  system,  but  further  than  this  it  would  not  be  safe  or  conservative 
to  make  statements.  The  same  may  be  said  of  shock,  moral  and  physi- 
cal, as  well  as  of  worry,  grief,  etc. 

Symptoms. — Migraine  usually  begins  abruptly.  It  is,  as  we  have 
said,  one  of  the  explosive  neuroses.  Some  authors  describe  prodromes, 
such  as  a  sense  of  depression  or  irritability  for  some  hours  or  even  a 
day  beforehand.  In  rare  cases  a  sense  of  elation  or  well-being  precedes 
the  attack.  But  in  the  commoner  type  of  simple  migraine  the  debut  is 
unannounced.  It  comes  in  the  midst  of  work  or  play,  at  any  time  of 
the  day  or  night;  in  fact,  it  is  sometimes  nocturnal,  occurring  during 
sleep,  the  patient  awaking  in  the  midst  of  a  full-blown  seizure. 

The  initial  symptom,  or  aura,  is  usually  sensory,  and  in  the  vast 
majority  of  cases  it  is  visual.     Exceptions,  however,  occur.     A  sensory 


'  Liveing  (op.  cit.,  p.  30)  discusses  Hereditary  Transformations.  The  earliest 
discussion  of  this  subject  was  by  Moreau  de  Tours  (De  I'Etiologie  de  I'Epilepsie, 
1852). 

'  Oppenheim,  Diseases  of  the  Nervous  System,  translated  by  Mayer,  1900, 
p.  748. 

"  Diseases  of  the  Nervous  System,  vol.  ii.  p.  837. 


700  THE    EYE    ANB    NERVOUS    SYSTEM 

aura  may  occur  in  other  parts  of  the  body,  as  in  a  limb.  The  writer 
once  had  a  patient  who  always  felt  a  numb  or  tingling  sensation  in  his 
arm.  This  mounted  slowly  upward,  and  as  it  reached  the  shoulder, 
neck,  and  head,  it  gradually  disappeared,  and  the  pain  set  in.  The 
analogy  with  epilepsy  is  here  striking,  but  an  important  difference  is 
seen  in  the  fact  that  the  aura  of  migraine  is  of  much  longer  duration 
than  that  of  epilepsy.  It  is  never  instantaneous.  It  may,  and  usually 
does,  endure  for  some  minutes,  even  for  a  half  hour. 

The  patient,  of  course,  from  past  experience,  knows  the  full  import 
of  this  aura.  He  usually  drops  everything  and  seeks  a  retreat,  and  not 
seldom  he  is  much  disquieted  in  mind.  This  mental  state  during  the 
aura  of  migraine  is  important  to  recognize,  for  it  is  at  this  time  in 
susceptible  patients  that  hysteria  may  show  itself  and  complicate  the 
clinical  picture.     We  shall  refer  to  this  subject  later.® 

The  visual  aura  of  magraine  is  characteristic,  and  has  special  sig- 
nificance for  us  in  these  pages.  It  is  usually  a  form  either  of  ambly- 
opia or  amaurosis.  The  visual  field  is  obscured  in  a  variety  of  ways. 
This  is  its  simplest  type.  The  sight  is  confused,  or  partially  obliterated. 
The  outlines  of  objects  seen  are  blurred  and  indistinct ;  in  other  words, 
there  is  an  amblyopia.  The  patient  is  not  able  to  read,  or  reads  with 
difficulty ;  the  same  is  true  of  objects  and  faces,  which  he  can  scarcely 
recognize.  When  a  true  amaurosis  occurs,  whole  sections  of  the  visual 
field  may  be  obscured,  and  the  patient  may  even  be  hemianopic.  Thua 
there  may  be  seotomata,  in  which  large  areas,  as  it  were,  are  blotted  out. 
There  may  also  be  scintillating  lines,  flashes  of  light  and  color,  blazing- 
or  flaming  splotches,  and  zig-zag  or  whirling  figures,  the  forms  of 
animals,  terrifying  apparitions,  and  other  curious  illusions.  In 
mild  cases  of  ordinary  migraine  these  visual  aura?  may  be  but  little 
marked,  but  in  the  type  of  the  disease  known  as  ophthalmic  migraine 
they  form  an  important  element,  and  will  be  described  more  in  detail 
under  their  appropriate  head. 

It  is  the  characteristic  of  the  aura  of  migraine  to  disappear  before 
the  onset  of  the  pain.  This  is  the  almost  uniform  rule.  The  visual 
field  entirely  clears,  so  that  the  patient,  if  he  did  not  know  otherwise 
from  bitter  experience,  might  suppose  for  a  few  minutes  that  he  was 
entirely  relieved  of  his  attack.  But  the  respite  is  brief;  in  fact,  in 
some  cases,  the  transition  from  the  stage  of  visual  defect  to  the  stage  of 
pain  is  almost  imperceptible. 


"  Gilles  de  la  Tourette  (Traitfi  de  I'Hysterie,  Paris,  1891)  does  not  mention 
migraine  among  the  exciting  causes  of  hysteria,  an  omission  which  strikes  the  present 
writer  as  rather  remarkable. 


MIGRAIXE  701 

The  pain  of  migraine  is  a  neuralgia,  usually  intense,  in  the  distri- 
bution of  the  fifth  nerve.  It  is  commonlv  described  as  a  hemierania 
(and  in  fact  the  word  migraine  is  a  French  derivative  of  the  Latin 
hemierania),  but  it  is  not  always  strictly  localized  on  one  side  of  the 
head.  It  may  be  an  intense  frontal  headache,  and  may  even  appear  to 
radiate  over  a  large  part  of  the  cranium.  In  other  cases  it  is  more  dis- 
tinctly unilateral.  Some  patients  even  describe  it  as  limited  to  one 
branch  of  the  trigeminal,  and  the  branch  most  involved  is  the  first  or 
ophthalmic  division. 

This  f)ain  is  cumulative,  as  Liveing  has  pointed  out.  It  begins  as 
a  slight  affection,  but  gradually  increases,  and  in  the  course  of  an  hour 
or  two  reaches  its  maximum.  It  is  aggravated  by  motion,  and  by  noise 
and  light.  The  patient  usually  seeks  absolute  rest  and  seclusion.  The 
character  of  the  pain  is  described  as  boring,  or  throbbing:  Du  Bois 
Reymond,  who  was  a  victim  of  migraine,  said  that  in  his  own  case  the 
pain  throbbed  in  unison  with  the  temporal  artery.  The  intensity  varies, 
but  it  is  usually  very  great;  in  some  cases,  however,  the  pain  is  mild 
or  even  aborts  early.  Cases  have  even  been  described  in  which  it  is 
absent,  or  replaced  by  a  vertigo;  these  aberrant  types  will  be  referred 
to  again.  The  duration  of  the  pain  also  varies  in  different  cases, 
although  it  is  likely  to  be  equal  in  the  same  case.  As  a  rule,  the  pain 
does  not  endure  over  night;  it  is  cured  by  a  night's  sleep.  Cases  in 
which  it  lasts  for  twenty-four  hours  are  rare,  although  instances  are 
reported  in  which  it  persisted  for  even  two  or  three  days.^^  The  termi- 
nation of  the  pain  is  sometimes  abrupt  or  critical ;  this  is  true  in  cases 
in  which  it  is  relieved  promptly  by  vomiting  or  by  a  copious  movement 
of  the  bowels.     It  may  be,  and  often  is,  terminated  by  sleep. 

Gastric  disorder  is  very  common  in  migraine ;  in  fact,  it  is  one  of 
the  three  characteristic  symptoms.  It  is,  as  a  rule,  a  late  or  terminal 
manifestation.  Early  in  the  attack,  however,  there  is  complete  aversion 
to  food,  the  patient  not  only  feeling  a  physical  disgust  for  it,  but  also 
apprehending  its  effect  upon  the  nausea  and  vomiting  which  are  almost 
sure  to  appear  in  time.  That  the  nausea  and  vomiting  are  usually  late 
or  terminal  symptoms,  has  long  been  recognized.^ ^  In  some  cases  they 
have  the  character  of  a  crisis,  and  the  pain  is  often  promptly  relieved 
by  emesis.  This  fact  gives  a  hint  for  treatment.  I  have  known  a  severe 
bout  of  migraine  promptly  aborted  by  the  administration  of  an  emetic.*^ 

"  Piorry,   Memoirs,   etc. 

"Romberg,  "A  Manual  of  Nervous  Diseases"  (Syd.  Soc.  Trans.,  vol.  i.  p.  176). 

"An  old  writer,  Robert  Whytt  (On  Nervous  Disorders,  1765,  p.  305),  fully 
recognized  the  value  of  emetics  in  these  periodical  headaches.  "  When  they  are 
regular  as  to  their  periods,  vomits  should  be  given  an  hour  and  a  half  beforehand." 


702  THE    EYE    AND    NERVOUS    SYSTEM 

The  same  result  can  sometimes  be  obtained  by  a  saline  purge.  In  fact, 
the  vomiting  of  migraine  may  be  a  natural  mode  of  termination;  a 
form  of  vis  medicatrix  naturae.  It  is  nature's  way  of  securing  relief. 
In  some  severe  cases,  however,  the  vomiting  is  not  always  efficient  at 
once,  and  it  may  recur  during  several  hours  before  complete  cure  is 
effected.  In  some  cases  the  stage  of  vomiting  does  not  appear;  there 
may  be  very  slight  nausea  or  even  none  at  all.  This  seems  to  be  so 
especially  of  the  attacks  later  in  life,  when  the  disease  is  losing  its 
hold  on  the  patient.  But  there  are  distinct  aberrent  types,  even  early 
in  life,  in  which  the  stage  of  gastric  irritation  is  conspicuous  by  its 
absence. 

The  vomiting  of  migraine  is  often  described  as  a  cerebral  vomiting, 
especially  by  the  older  writers,  who  affected  to  see  in  it  an  irritation  of 
the  pneumogastric  nerve.  It  is  true  that  it  occurs  without  reference  to 
the  presence  of  food  in  the  stomach,  just  as  the  vomiting  of  pregnancy, 
of  sea-sickness,  or  of  meningitis ;  and  in  severe  cases  may  afford  no 
relief.  It  is  then  attended  with  retching  and  great  prostration,  and 
instead  of  relieving,  only  adds  to  the  patient's  suffering.^ ^ 

It  is  important  to  bear  in  mind  that  the  varieties  of  migraine  are 
not  separable  by  hard  and  fast  lines.  They  merge,  as  it  were,  into 
one  another.  Thus  the  distinction  between  "  ordinary"  migraine  and 
ophthalmic  migraine  as  made  by  some  writers  is  an  artificial  one.  In 
fact  "  ordinary'"  migraine  is  really  only  a  form  of  the  disease  in  which 
the  visual  aurae  are  merely  not  so  conspicuous  as  in  the  ophthalmic 
variety.  So  of  ophthalmoplegic  migraine ;  it  is  similar  in  many  ways 
to  the  ordinary  type  except  that  it  has  in  addition  a  motor  disorder, 
— namely,  a  paralysis  of  one  or  other  of  the  ocular  nerves.  In  fact, 
it  might  perhaps  be  justifiable  to  say  that  there  are  two  main  types  of 
migraine,  a  sensory  and  a  motor  type.  In  the  one  (ophthalmic)  a 
sensory  nerve,  the  optic,  is  especially  involved.  In  the  other  (ophthal- 
moplegic) a  motor  nerve,  the  ocjilomotor,  or  rarely  the  abducens,  is 
implicated.  We  must  recall  also  that  there  is  still  another,  but  rare, 
type,  in  which  the  chief  symptoms  are  psychical.  These  distinctions 
call  to  mind  the  analogy  of  migraine  to  epilepsy — an  analogy  which 
some  authors  are  particularly  fond  of  pointing  out. 

OPHTHALMIC   MIGRAINE. 
Originally  the  term  ophthalmic  migraine  was  used  to  bolster  up  a 
theory  of  the  causation  of  the  disease.    This  theory  was  that  of  Piorry, 
who  held  that  migraine  may  be  caused  by  overstrain  of  the  iris  and  over- 

"  Liveinp,  op.  cit.,  p.   136  et  infra. 


OPHTHALMIC  MIGRAINE  703 

stimulation  of  the  retina.  Here  we  have  a  very  early  statement  of  the 
theory  of  eye-strain,  which  has  since  been  worked  up  to  such  good  pur- 
pose by  some  specialists.^'*  The  idea,  in  brief,  was  that  an  irritation  of 
the  iris  and  retina  was  the  cause  of  the  disease ;  hence  the  theory  itself, 
rather  than  the  disease,  should  receive  the  name  "  ophthalmic."  Piorry 
apparently  had  no  knowledge  of  the  motor,  or  ophthalmoplegic,  form  of 
migraine,  and  he  was  merely  interested  in  establishing  a  plausible 
explanation  of  the  disease.  For  him  all  migraine,  however,  was  not 
ophthalmic.  He  recognized  that  it  could  be  caused  by  other  conditions 
than  eye-strain. 

We  speak  of  ophthalmic  migraine  to-day  to  distinguish  that  sensory 
form  in  which  the  visual  aura?  are  conspicuous,  as  distinct  from  the 
motor  or  ophthalmoplegic  form  in  which  there  is  paralysis  of  ocular 
muscles.  The  credit  of  first  using  the  term  "  ophthalmic"  for  this 
purely  sensory  form  of  migraine  is  often  given  to  Charcot  (as  are  so 
many  other  things  in  neurology),  but  as  constituting  the  commonest 
form  of  migraine,  this  variety  had  really  been  recognized  for  many 
years  antedating  the  great  French  neurologist.^^  It  is  true,  however, 
that  since  Charcot,  and  probably  in  part  due  to  his  influence,  great 
pains  have  been  taken  by  many  writers  to  describe  minutely  the  visual 
disorders  of  migraine,  until  now  we  can  almost  say  that  these  disorders 
have  been  overdescribed. 

The  visual  symptoms  in  ophthalmic  migraine  may  be  divided  as 
follows :  1.  Amblyopia ;  2.  scintillations ;  3.  scotoma ;  4.  hallucina- 
tions.^® 

By  amblyopia,  as  already  said,  is  meant  a  simple  blurring  of  the 
visual  field.  It  is  perhaps  the  commonest  of  these  symptoms,  and  is 
often  seen  in  simple  cases  of  sick  headache.  It  is  not  accompanied  with 
distinct  scotomata,  or  blotting  out  of  definite  areas  of  the  field,  but  it 
is  probably  closely  allied  with  them.     In  fact,  it  appears  to  be  only  a 


"Piorry's  work  was  his  M6moire,  etc.  His  theory  is  discussed  in  full  by  Live- 
ing,  op.  cit.,  p.  254  et  infra.  Piorry  anticipated  many  of  the  specious  arguments  of 
later  extremists,  and,  like  them,  he  quoted  cases  to  prove  his  position.  By  much 
the  same  process  of  logic  it  would  be  possible  to  prove  that  eye-strain  is  responsible 
for  locomotor  ataxia,  or  for  cancer  of  the  womb,  or,  in  fact,  for  any  disease  which 
happens  to  appear  in  a  patient  who  has  a  little  astigmatism. 

''  Berbez,  Les  Migraines,  in  Gaz.  Hebdom.,  2  S.  xxxvi.  19.  The  disease  was 
recognized  by  Sazenne  (1748),  Plenk  (1783),  Stole  (1795),  Demours  (1818),  Wol- 
laston  (1824)  ;  Madame  de  Pompadour  was  said  to  have  been  a  victim.  See  also 
Muller-Syer,  Ueber  Ophthalm.  Migraine,  in  Berl.  Klin.  Woch.,  1887,  xxiv.  p.  787. 

'"Galezowski  (Etude  de  la  Migraine  Opthalmique,  in  Arch.  G§n.  de  M6d. 
Juin,  1878)  gives  a  slightly  different  classification. 


704  THE    EYE    AND    NERVOUS    SYSTEM 

minor  degree  of  amaurosis.  In  this  amblyopia  the  patient  sees  objects, 
but  sees  them  indistinctly;  the  outlines  are  confused,  although  with 
care  and  attention  objects  can  be  made  out,  as  for  instance,  print  or 
small  articles.  This  would  hardly  be  so  if  there  were  distinct  scotomata, 
or  localized  blind  areas.  Still,  it  is  difficult  sharply  to  differentiate  a 
mere  amblyopia  from  areas  of  amaurosis  or  real  blindness.  Patients 
themselves  are  poor  observers  of  these  phenomena;  they  know  that 
vision  is  obscured  for  a  time,  and  they  know  the  import  of  the  symptom, 
but  beyond  that  they  do  not  attempt  to  observe  or  describe.  An  expert 
examination  in  these  cases  might  possibly  reveal  some  small  spots  of 


Fig.  1.— Scotoma  scintlllans.    (After  Charcot.) 

total  blindness  scattered  over  the  field.  But  as  the  symptom  is  a  tran- 
sient one,  lasting  only  a  few  minutes,  there  is  little  chance  to  have  these 
cases  examined  with  care.  In  some  cases  this  amblyopia  passes  into  a 
stage  of  scintillation  just  before  it  ends. 

The  scintillations  of  migraine  are  observed  in  many  cases.  They 
consist  of  waving  or  vibrating  lines  of  light  extending  across  or  at  the 
edges  of  the  visual  field.  Not  unfrequently  they  are  confined  to  the 
•edges  of  darkened  areas,  constituting  then  the  phenomena  known  as 
scintillating  scotomata.  These  spectral  illusions  were  fully  and  graphi- 
cally described  by  Liveing.*^  They  consist  of  a  luminous  border  sur- 
rounding the  blind  area.  This  area,  at  first  small,  gradually  expands, 
and  as  it  increases  in  size,  the  scintillating  rim  makes  its  appearance 
and  grows  in  intensity.    Usually  this  rim  is  particolored  (although  not 

'"  Op.  cit.,  p.  75  et  infra.  Liveing  gives  very  graphic  colored  diagrams  of  these 
scintillating  scotomata.    See  the  plate  at  the  end  of  his  work. 


OPHTHALMIC  MIGRAINE  705 

always  so)  and  it  is  apt  to  assume  a  zig-zag  shape,  which  was  described 
by  Fothergill  ^*  as  consisting  of  luminous  angles  like  those  of  a  forti- 
fication. These  images  have  been  described  in  a  variety  of  ways  by 
different  observers  and  they  evidently  vary  somewhat  in  different 
cases.  They  are  seen  whether  the  eyes  are  open  or  shut,  but 
sometimes  better  in  twilight  or  darkness.  Various  colors  may  be  inter- 
mingled with  them,  but  in  mild  cases  they  are  colorless,  and  not 
arranged  in  such  fixed  patterns  as  the  imagination  of  some  writers 
suggests.  They  may,  in  fact,  be  mere  fleeting  and  dimly  perceived 
vibrating  lines,  appearing  just  before  the  obscuring  of  vision  passes 
away.  In  some  cases  the  scintillation  is  centrifugal;  that  is  to  say,  it 
appears  at  the  edge  of  the  shaded  area,  and  tends  to  spread  towards 
the  circumference  of  the  visual  field,  gradually  dying  out.  In  other, 
and  rarer  cases,  the  scintillation  is  centripetal,  appearing  at  the  edge 
and  gradually  extending  towards  the  centre,  thus  covering  the  visual 
field.  There  has  been  some  discussion  whether  the  scintillations  are 
binocular  or  monocular,  but  the  weight  of  opinion  is  in  favor  of  their 
appearing  in  both  eyes,  a  fact  which  can  be  proved  by  the  patient  closing 
one  eye  at  a  time,  when  in  some  cases  the  scintillations  disappear  from 
the  closed  eye ;  also  as  to  whether  they  appear  on  only  one-half  of  each 
field  (hemiopic),  and  if  so,  on  which  half.  We  shall  return  to  this 
subject  in  discussing  the  scotoma. 

Besides  the  wavy  and  zig-zag  scintillations,  there  are  otlier  forms. 
These  have  been  variously  described  as  showers  of  sparks,  colored  hail 
or  rain,  flashes  of  light,  flaming  bodies,  and  spots  of  fire.  These  may 
either  fill  the  whole  field,  or,  what  perhaps  is  more  common,  they  appear 
around  the  edges  of  blind  areas  or  scotomata.  In  fact,  where  there  are 
genuine  scotomata,  these  pyrotechnics  could  not  well  be  seen  except 
about  the  borders  of  the  blind  areas.  It  is  possible  to  suppose  that  these 
various  illuminations  are  due  to  an  irritation  of  the  optic  fibres; 
whereas  the  scotoma,  or  blind  spot,  is  due  to  a  paralysis  of  optic  fibres. 
The  latter  are  conceivably  the  more  central  fibres,  the  former  are  the 
fibres  arranged  around  the  others  whose  functions  are  for  the  time 
abolished. 

These  scintillations  may  be  the  only  visual  signs,  except  some  slight 
amblyopia ;  but  in  many  cases  they  are  accompaniments  of  scotomata  or 
blind  spots,  to  be  presently  described.  In  some  cases  they  are  not  seen 
in  the  early  years  of  migraine,  but  make  their  appearance  later  in  life. 
Thus  in  the  case  of  a  woman  aged  32  years,  who  had  had  migraine  since 


'Remarks  on  Sick  Headache,  Works,  p.  597. 
45 


706  THE    EYE    A:N"D    NERVOUS    SYSTEM 

childhood,  the  muscae  volitantes,  colored  spectra,  flashes  of  light  and 
scintillating  scotomata,  with  photophobia,  were  only  present  after  the 
thirtieth  year.^® 

By  a  scotoma  is  meant  a  blind  spot  in  the  field  of  vision,  and  this 
spot  is  caused  by  and  corresponds  to  an  area  in  the  retina  in  which  the 
function  of  sight  is  abolished.  In  migraine  such  scotomata  are  not 
uncommon  as  part  of  the  visual  disorder  present  in  the  early  stage; 
in  other  words,  as  part  of  the  aura.  It  is  from  this  symptom  that 
migraine  is  often  called  "  blind-headache."  These  scotomata  may  be 
variously  placed  and  of  variable  extent.  Thus  they  may  be  central,  or 
near  the  centre,  or,  as  is  not  unusual,  spread  over  a  part  of  one  side  of 
the  field.  They  are,  as  a  rule,  present  in  both  eyes.  The  most  important 
is  the  scotoma  scintillans,  already  referred  to,  in  which  the  edge  of  the 
blind  area  is  the  seat  of  zig-zag  or  "  fortification"  lines  of  wavy  and 
particolored  light. 

The  effect  of  these  scotomata  is  to  cause  curious  impairment  of 
vision.  Objects  are  seen  only  in  part.  Thus  Mobius  ^^  had  a  patient 
who  said  that  men  walked  about  without  their  heads ;  and  Abemethy, 
who  was  a  victim  of  migraine,  said  humorously  that  he  could  see  no 
more  of  his  own  name  than  the  "  knee"  (.ne)  and  the  "  thigh"  (thy).^^ 
The  scotoma  lasts  but  a  short  time,  ten  to  twenty  minutes,  rarely  as 
long  as  an  hour;  although  Galezowski  recorded  an  unusual  case  in 
which  it  lasted  for  five  months,  without  objective  findings.  In  fact, 
ophthalmoscopic  examination  during  the  time  of  the  attack  reveals 
nothing. 

This  is  the  uniform  experience.  These  attacks  are  sometimes  at- 
tended with  intense  photophobia.  In  some  cases,  as  Mobius  points  out, 
the  lids  droop,  but  this  may  be  due  to  an  instinctive  desire  to  shut  out 
the  light.  He  also  says  that  pupillary  changes  sometimes  accompany 
attacks  of  ophthalmic  migraine,  but  in  this  he  is  not  confirmed  by  many 
observers.     They  are  seen,  however,  in  the  ophthalmoplegic  form. 

These  scotomata  may  be  %o  extensive  in  some  cases  as  to  include  one- 

"  Da  Costa,  "  A  Case  of  Ophthalmic  Migraine,"  in  the  Jour.  Nerv.  and  Ment. 
Dis.,  1890,  XV.  p.  213. 

"Mijjrainp,  in  Xothnagel's  Spec.  Path.  u.  Therap.,  Bd.  xii.  See  also  Dianoux, 
Scotoma  Scintillant  ou  Amaurosc  Partielle  Temporaire,  These,  1875. 

"  This  must  have  been  a  stock  joke  of  Abernethy's,  and  made  to  do  frequent 
duty,  or  else  its  author  has  been  much  misquoted.  Abernethy  perpetrated  this  pun 
in  a  lecture,  published  in  the  Lancet,  October  21,  1826,  and  said  the  disorder  of 
vision  in  his  own  case  was  caused  by  an  injury  (a  blow  on  the  nose),  and  he  made 
no  reference  whatever  to  migraine.  The  lecture  has  recently  been  republished  in  the 
Lancet  (October  22,  1904,  p.  1170). 


OPHTHALMIC  MIGEAIi^E  707 

half  of  each  visual  field,  in  which  case  there  is  an  hemianopsia.  This 
has  been  reported  by  not  a  few  observers.^-  \\Tien  these  scotomata  are 
thus  hemianopic  they  may  appear  either  on  the  side  upon  which  the 
pain  is  preseilt  or  on  the  opposite  side.  When  the  pain  is  strictly  local- 
ized on  one  side,  it  is  usually  on  the  side  of  the  hemianopsia.  This  is 
true  also  of  other  sensory  aura?,  such  as  a  numb  feeling  in  a  limb;  it 
is  usually  on  the  same  side  as  the  pain.  Exceptions,  however,  occur, 
as  was  pointed  out  by  Liveing,  Calmeil  and  others.  This  subject 
needs  more  careful  study.  The  hemianopsia  may  be  lateral  or  ver- 
tical.23 

Visual  hallucinations  have  been  observed  in  migraine.  But  they 
must  be  rare,  for  they  are  reported  by  only  a  few  observers.  S.  Weir 
Mitchell  ^^  has  put  such  cases  on  record.  Thus  a  school-teacher,  aged 
30  years,  saw  a  large,  black  and  hairy  dog  which  disappeared  as  the 
pain  began.  Another  patient  saw  an  image  of  her  dead  sister.  Still 
another  saw  an  image  of  a  near  relative  covered  with  blood,  especially 
if  the  attack  happened  at  her  menstrual  period.  In  a  fourth  case  there 
was  a  scotoma  scintillans,  but  after  some  attacks  the  patient  had  visions 
of  a  trellis  of  silver  covered  with  vines  and  flowers  of  brilliant  hue,  best 
seen  with  the  eyes  open.  Some  of  these  were  associated  with  geometrical 
figures,  pink  or  red.  It  would  be  interesting  to  know,  in  such  cases, 
whether  there  were  hysterical  symptoms  present,  or  whether  such  com- 
plex visions  might  be  the  result  of  suggestion.  Certainly  they  are  not 
of  the  ordinary  type  of  visual  phenomena  seen  in  migraine,  especially 
when  they  occur  after  the  attack.  The  analogy  of  such  cases  with 
epilepsy  is  also  suggested,  but  of  course  not  proved ;  for  visual  hallu- 
cinations have  been  noted  among  the  rare  aurse  of  the  sacred  disease. 
A  curious  form  of  visual  hallucination,  or  more  properly  illusion,  has 
been  noted  in  migraine  by  Gowers.^^  This  might  be  described  as  a 
projection  outward  of  the  scintillation  or  coruscation ;  thus  to  one 
patient  it  appeared  as  though  a  plate  which  sat  before  him  on  a  table 


^Mobius,  op.  cit.  Galezowski,  op.  cit.  Da  Costa,  op.  cit.  Dufour,  Sur  la 
vision  nulle  dans  I'hemiopie,  in  Rev.  Med.  de  la  Suisse  roun.,  1889,  p.  445.  Schmidt's 
Jahrb.,  ccxxvi.  p.  74.  Liveing,  op.  cit.  Airy,  On  a  Distant  Form  of  Transient 
Hemiopia,  in  Philosoph.  Trans,  for  1870,  p.  247.  Harris,  Hemiopia,  etc.,  in 
Brain,  1897,  vol.  xx.  p.  308. 

^  Airy  made  use  of  the  word  "  teichopsia"  (from  reixoc,  a  wall,  and  oipig  vision) 
for  this  symptom,  especially  where  there  is  the  appearance  of  a  "  fortification"  line. 

=*  Neuralgic  Headache,  with  Apparitions  of  Unusual  Character,  in  Am.  Jour, 
of  the  Med.  Sci.,  vol.  xciv.  p.  415. 

^  Diseases  of  the  Nervous  System,  vol.  ii.  p.  846. 


708  THE    EYE    AND    NERVOUS    SYSTEM 

was  surrounded  with  the  colored  spectrum.  But  neither  Gowers  nor 
Liveing  mentions  true  hallucinations.^*' 

A  few  observers  have  reported  cases  of  that  rare  symptom  known  as 
"  hemianopic  hallucinations."  Harris  ^"^  has  collected  a  few  references 
to  these  cases,  from  which  it  is  evident  that  they  are  due  to  organic 
lesions  chiefly.  Tuke  ^^  mentions  them  in  connection  with  migraine 
and  epilepsy,  but  Harris  gives  no  instance  that  was  not  due  to  organic 
disease.  The  latter  author  indulges  in  some  speculation  about  the 
origin  and  seat  of  such  visual  symptoms,  and  adopts  Hughlings- Jack- 
son's phraseology'.  For  instance,  he  holds  that  a  visual  aura  in  migraine 
is  caused  by  an  "  epileptic  discharge" — a  needless  confusion  of  the 
two  diseases.  In  hemianopic  hallucinations  the  patient  sees  the  false 
images  with  one-half  of  the  visual  fields  only.^^ 

In  rare  cases  migraine  is  associated  with  symptoms  of  other  affec- 
tions. Among  these  is  aphasia.  Charcot  ^"  made  the  distinction  be- 
tween simple  ophthalmic  migraine  in  which  the  symptoms  are  scotoma 
scintillans,  transient  hemiopia,  headache,  and  vomiting;  and  migraine 
ophihalmique  accompagnee,  in  which  in  addition  there  are  a  more  or 
less  permanent  hemianopsia,  difficulty  in  speech,  and  numbness  of  one 
side  of  the  face.  He  claimed  that  both  the  prognosis  and  treatment 
are  different  in  this  latter  and  graver  type.  But  this  distinction  was 
not  original  with  Charcot,  for  it  had  been  made  long  before  him  by 
Leberf^^  and  others.^^  It  is  doubtful,  however,  whether  all  of  the  re- 
ported cases  were  true  instances  of  migraine.  Some  of  them  appear  to 
have  been  due  to  cerebral  disturbance,  probably  vascular  or  toxic,  in 
which  there  was  not  only  aphasia  but  also  transient  hemiplegia  and 
hemiana?sthesia,  with  mental  confusion;  and  the  suspicion  is  aroused 
that  these  attacks  in  some  cases  may  have  been  obscure  symptoms  of 
Bright's  disease,  or  even  of  hysteria.     Fere  ^  also  draws  attention  to 


"Zacher  (Berlin,  klin.  Woch.,  xxix.  p.  694)  mentions  a  case  in  which  hallucina- 
tions of  sight  were  followed  by  epileptic  attacks,  but  the  case  was  evidently  one 
of  epilepsy  with  a  visual  aura,  not  migraine. 

"Hemianopia,  in  Brain,  20,   1897,  pp.  318,  300,  361. 

■*  Diet.  Psych.  Med.,  vol.  i.  p.  568. 

"  Liveing,  op.  cit.,  who  described  the  hemiopia  of  migraine  very  fully,  apparently 
makes  no  mention  of  hemianopic  hallucinations,  except  the  fortification  spectra 
already  alluded  to  in  the  text. 

""LeQons  de  Mardi.  1887,  p.  27. 

'"Traits  Prat,  des  Mai.  Canc§reuses,  Paris,  1851. 

"  Liveing  discusses  the  subject  at  length  in  his  classical  work. 

""  Sur  la  Migraine  Ophthalmique,  in  Rev.  de  Med.,  1883,  iii.  p.  194. 


OPHTHALMIC  MIGKAIXE  709 

the  fact  that  these  cases  may  mean  a  grave  prognosis.  He  reports  a 
case  in  a  patient,  53  years  old,  in  whom  the  speech  defects  became  more 
frequent.  There  were  finally  permanent  aphasia,  right  hemiplegia  of 
the  face  and  arm,  local  convulsions  becoming  general,  and  death.  There 
seems  to  be  no  sufficient  reason  to  attribute  all  these  grave  cerebral 
symptoms  to  migraine,  simply  because  they  occurred  in  a  migrainous 
patient.  Meige  ^*  also  reports  a  case,  in  which  there  appears  to  have 
been  disease  of  the  cerebral  arteries. 

Some  authors  have  also  spoken  of  migraine  as  an  early  symptom  of 
general  paresis.  It  is  doubtful  what  the  exact  relationship  is,  even  if 
there  be  any  relationship  other  than  a  coincidence.^^  Blocq  thinks  that 
the  relation  is  anatomical.  He  reports  three  cases,  all  with  neurotic 
heredity.  There  is  no  reason,  of  course,  why  a  patient  with  migraine 
should  not  also  develop  general  paresis,  but  a  distinct  pathological  rela- 
tionship seems  not  to  have  been  made  out.  Mickle  says  that  precedent 
migraine  often  disappears  at  the  development  of  general  paresis ;  and 
Chase  ^^  observes  that  severe  headache  is  sometimes  seen  as  an  early 
symptom  of  paresis,  but  he  does  not  call  such  headache  migraine.  There 
is  possibly  some  confusion  of  identity  in  some  of  the  reported  cases. 

The  same  may  be  said  of  locomotor  ataxia,  of  which  migraine  is 
sometimes  described  as  a  prodromal  affection.^"  This  is  certainly  not 
observed  in  the  vast  majority  of  cases  of  tabes,  and  the  association  is 
probably  accidental. 

That  some  of  the  bizarre  symptoms  noted  above  are  due  to  hysteria, 
there  can  not  be  much  doubt.  Among  these  are  speech-defects,  hemi- 
ana?sthesia,  hemiplegia,  convulsions,  and  other  hysterical  stigmata.  This 
truth  has  been  fully  recognized  by  some  authors,  but  strangely  over- 
looked by  others.  In  fact,  we  have  in  migraine  an  exciting  cause  of 
hysteria.  This  simple  fact  is  readily  understood  in  view  of  the  alarm- 
ing nature  of  the  migrainous  symptoms,  such  as  the  vivid  scintillations 
and  blind  spots,  followed  by  a  period  of  distressing  headache  and  vom- 
iting. More  or  less  mental  or  emotional  disturbance  is  not  uncommon 
in  the  first  stage  of  migraine.  The  patient  is  usually,  at  least,  depressed, 
and  sometimes  alarmed.  He  knows  full  well  the  import  of  the  visual 
aura,  and  understands  that  he  is  fated  to  a  painful  bout,  which  will 


"Rev.  Neurologique,  Xo.  18,  1904. 

^  Parinaud,  ^ligraine  Ophthalmique  au  debut  de  la  Paralysie  (Jenerale,  in 
Arch,  de  Neurolog.,  1883,  v.  p.  57.  Blocq,  Migraine  Ophthalmique  et  Paralysie 
Gen^rale,  in  Arch,  de  Neurolog.,  xviii.  p.  321. 

'"General  Paresis,   1902,  p.   146. 

"  Raullet,  Etude  de  la  Migraine  Ophthalmique,  These  de  Paris,  1883. 


710  THE    EYE    AND    NERVOUS    SYSTEM 

knock  him  out  for  a  day  or  two,  and  entail  much  physical  suffering.  It 
is  small  cause  for  wonder,  therefore,  that  in  some  neurotic  patients  the 
symptoms  of  hysteria  should  complicate  the  case.  The  writer  has  kno^vn 
such  cases,  and  has  been  led  to  suppose  that  they  are  more  common  in 
children  and  young  persons.  Babinski  ^*  reports  such  a  case  in  a  girl 
six  years  old.  She  had  such  hysterical  symptoms  as  hemianesthesia, 
dyschromatopsia,  contraction  of  the  visual  fields,  and  globus;  these 
were  associated  with,  or  excited  by,  the  migraine,  especially  the  scotoma 
scintillans  and  the  violent  hemicrania.  To  show  how  susceptible  such 
patients  may  be  to  suggestion,  it  was  only  needful  to  make  pressure  on 
the  sixth  dorsal  vertebra  (in  this  case,  a  hysterogenous  point)  to  cause 
the  appearance  of  a  scotoma.  In  some  cases  in  which  so-called  epileptic 
equivalents  have  been  reported,  the  convulsions  were  probably  hysteri- 
cal.^'' In  cases  in  which  the  scotoma  is  reported  as  unduly  prolonged 
(as  in  Galezowski's  case,  referred  to  on  a  preceding  page)  it  is  likely 
that  the  symptom  is  due  to  the  great  neurosis. 

Reference  has  already  been  made  to  the  forced  resemblance  between 
migraine  and  epilepsy.  Both  diseases  are  explosive,  both  are  preceded 
by  an  aura,  and  both  occur  in  neurotic  patients,  but  beyond  these  few 
points  the  resemblance  ceases,  or  at  least  is  not  marked.^®  An  important 
question  arises,  however,  as  to  the  possibility  of  the  transition  from 
migraine  to  epilepsy.  This  claim  is  made  by  some  authors,  but  the 
present  writer  has  never  seen  or  heard  of  an  authentic  case,  and  does  not 
believe  in  the  doctrine.  The  cases  reported  are  probably  instances  of 
hysterical  convulsions,  or  they  are  cases  in  which  migraine  and  epilepsy 
coexist  in  the  same  patient — which  is  by  no  means  a  proof  that  the  one 
disease  causes  or  merges  into  the  other.^^ 

Pathology. — It  is  proper  here  to  make  a  brief  reference  to  the  claim 
that  migraine  is  caused  by  eye-strain.    As  already  said  (page  703),  this 

**  De  la  Migraine  Ophthalmique  Hysterique,  in  Arch,  de  Neurolog.,  November, 
1890,  p.  305.  In  this  paper  Babinski  reports  several  cases  of  hysteria  caused  by 
migraine.  Among  the  hysterical  stigmata  were  mutism,  hemianiesthesia,  anaesthesia 
of  the  palate,  monocular  diplopia,  and  contraction  of  the  visual  fields.  He  even 
claimed  that  attacks  had  been  cured  with  hypnotism.  Thomas  (Migraine  Oph- 
thalmique Associf>e  de  Nature  Hysterique  chez  I'enfant,  in  Rev.  Med.  de  la  Suisse, 
xii.  p.  800)  reports  the  case  of  a  boy  whose  migrainous  attacks  were  accompanied 
with  paresis  of  the  right  side  and  difficulty  in  speech,  lasting  an  hour,  with  other 
stigmata  of  hysteria.  See  also  Fink,  Des  Rapports  de  la  Migraine  Ophthalmique 
avec  I'Hysterie,  Paris,  1891. 

^*  Mengazzini,  Rev.  Sper.  di  Freniatria,  abstracted  in  Cent.  f.  Nervenheil.,  xvii. 
p.  1G2. 

*"  Mobius,  Ueber  ^ligrsine,  in  Cent,  f,  Neurolog.,  viii.  p.  244. 

"  Spiller,  The  Relation  of  Migraine  to  Epilepsy,  in  Am.  Journ.  Med.  Sci.,  1900. 


OPHTHALMIC  MIGEAI:N^E  711 

theory  was  advanced  by  Piorry  many  years  ago,  but  it  fell  into  obscurity 
until  revived  recently  by  a  few  extremists.  Martin  has  reported  no 
fewer  than  352  cases  in  which  he  thought  he  was  able  to  detect  a  low 
degree  of  astigmatism  as  the  cause  of  migraine/^  but  he  probably  mis- 
took a  post  hoc  for  a  propter  hoc,  a  conceivable  error  in  a  hasty  observer 
in  view  of  the  wide  prevalence  of  astigmatism.  Gould/^  who  has  writ- 
ten voluminously  on  the  subject,  makes  the  sweeping  claim  that  the  near 
use  of  astigmatic  eyes  is  always  required  to  produce  migraine,  but  his 
conclusions  are  widely  rejected.  Paul  Bert^*  held  that  scintillating 
scotoma,  generally  attributed  to  fatigue  of  the  retina,  is  in  reality  due 
to  fatigue  of  the  nervous  centres ;  but  there  seems  to  be  no  real  scientific 
warrant  for  such  dogmatic  statements. 

Attempts  to  describe  the  pathology  of  migraine  have  been  equally 
speculative  and  futile.  Dufour  ^^  held  that  the  disease,  or  rather  the 
scotoma,  was  due  to  "  vasomotor  anaemia"  of  the  occipital  region  of  the 
brain.  Ball  ^^  supposed  it  was  due  to  a  "  local  asphyxia."  Latham  *^ 
believed  that  the  visual  disturbance  was  due  to  "  excitation  of  the 
sympathetic,"  and  so  on.  Recently  the  tendency  has  been  to  suppose 
migraine  to  be  due  to  an  infection;  but  this  theory  is  merely  an  evi- 
dence of  the  present  trend  towards  a  humoral  pathology,  and  it  cannot 
be  said  to  rest  upon  solid  facts. 

Grerman  writers,  as  Eulenberg,^^  have  been  fond  of  insisting  on  the 
distinction  between  the  "  angiospastic"  and  "  angioparalytic"  types  of 
migraine.'     The  distinction  is  based  on  the  supposed  condition  of  the 

"  Ann.  d'Oc,  xcix.  pp.  24  and  205. 

"  Journ.  Am.  Med.  Asso.,  Jan.  1904. 

"  Comp.  Rend,  des  Stances  de  la  Soc.  de  Biol.,  1882,  p.  571.  The  very  extrava- 
gance of  the  claims  of  some  of  these  writers  tends  to  cause  distrust  of  their  opinions. 

"  Sur  la  Vision  Mille  dans  I'Hemiopie,  in  Rev.  Med.  de  la  Suisse,  1889,  p.  445. 

**  Quoted  by  RauUet,  Etude  de  la  Migraine  Ophthalmique,  Th6se  de  Paris, 
1883. 

"  On  Nervous  or  Sick  Headache,  1873. 

*'0p.  cit.  Quaglino  (Annali  del  Ottamologia,  1871,  p.  7,  cited  by  Raullet)  held 
that  ophthalmic  migraine  was  due  to  "  spasm  of  the  arteries  of  the  retina."  It  is 
extraordinary  how  completely  the  advocates  of  these  vasomotor  theories  ignored 
the  question  of  a  possible  cause  for  the  alleged  action  of  the  vasomotors.  There 
could  hardly  be  a  vasomotor  spasm  without  something  to  cause  it.  These  theories 
grew  out  of  the  solidist  school  of  pathologists,  who  explained  every  disease  by  a 
change  in  the  tissues,  and  complacently  ignored  the  necessity  for  finding  causes  for 
such  alleged  changes. 

Among  other  works  on  ophthalmic  migraine  are  the  following:  Robiolis,  Con- 
tribution a  I'Etude  de  la  Migraine,  etc..  These  de  Montpellier,  1884.  Baralt,  Con- 
tribution a  I'Etude  du  Scotoma  Scintillant,  etc..  These  de  Paris,  1880.  Dianoux, 
Du  Scotome  Scintillant,  Th6se  de  Paris,  1875.  See  also  Brewster,  Philosophical 
Magazine,  1865,  p.  503. 


T12  THE    EYE    AND    NERVOUS    SYSTEM 

vasomotor  supply.  In  one  form  the  face  is  pale,  the  eye  hollow,  the 
pupil  dilated,  and  the  arteries  tense.  Eulenberg  even  claimed  to  have 
found  the  temperature  of  the  external  ear  reduced  four-tenths  of  a 
degree!  In  the  other  form  the  reverse  of  all  these  conditions  is  seen, 
and  the  temperature  of  the  ear  rises  two-tenths  of  a  degree.  These  fine 
physiological  studies  have  never  thrown  the  slightest  light  on  the  nature 
of  migraine,  for  the  question  still  remains,  What  causes  the  vasomotor 
change  ? 

Some  pathologists  reach  impotent  conclusions  about  migraine,  as, 
for  instance,  Oppenheim,  who,  at  the  autopsy  of  an  old  migrainous 
patient,  found  central  softening  from  embolism  as  the  cause  of  death, 
and  who  thought  vasomotor  disturbance  had  caused  the  old  migraine 
and  likewise  the  recent  embolus.     Surely  this  is  a  far-fetched  conclusion. 

OPHTHALMOPLEGIC   MIGRAINE. 

This  form  of  migraine  is  characterized,  as  its  name  implies,  by  a 
paralysis  of  some  of  the  ocular  muscles.  This  paralysis  is  usually  a 
sequel  or  result  of  the  migrainous  attack,  and  is,  of  course,  like  that 
attack,  periodical.  The  muscles  involved  are,  as  a  rule,  those  supplied 
by  the  third  nerve,  but  a  few  cases  have  been  observed  in  which  the 
paralysis  has  occurred  in  the  fourth  or  the  sixth  nerve. 

The  term  "  ophthalmoplegic  migraine"  was  suggested  by  Charcot  *® 
in  1800,  but  Charcot  was  not  the  first  to  record  an  instance  of  this 
interesting  disease.  Mobius  ^^  is  usually  credited  with  this  distinction 
(in  1884),  but  even  before  that  date  Adams,^*  in  1877,  had  reported 
a  case  of  paralysis  of  the  left  third  nerve  with  remarkable  intermission, 
relapse,  and  recov'er}',  which  may  have  been  an  instance  of  ophthalmo- 
plegic migraine.  Saunby,"^^  in  1882,  had  reported  a  clear  case  of 
migraine  with  paralysis  of  the  third  nerve,  and  the  distinction  of  prior- 
ity seems  indubitably  to  belong  to  him.  After  1884  reports  of  cases 
became  quite  numerous;  and  merely  to  repeat  the  titles  of  the  papers 
would  now  be  a  task  quite  irksome  and  prolonged.?* 

"  Sur  un  eas  de  Migraine  Ophthalmoplegique,  in  Cliniques  des  Maladies  du 
Syst.  Nerv.,  1892,  p.  70. 

"  Neurol.  Beitrag.,  iv.  Deut.  Zeit.  f.  Nervenheilk.,  Bd.  xvii.  299. 

"Ophthalmic  Hosp.  Rep.,  Dec,  1877. 

«  Lancet,  1882,  Sept.  2,  p.  345. 

"The  following  are  the  principal  early  reports  besides  those  already  named: 
Snell,  Lancet,  1885,  vol.  ii.  p.  939.  Clark,  ibid.  Beevor,  ibid.  Thompson,  Char. 
Ann.,  1885.  p.  507.  Manz.  Berl.  klin.  Woch.,  1885,  Nr.  40.  Parinand,  Soc.  d'Oph. 
Francaise,  Janv.  29,  1895;  also  Schmidt's  Jahrb.,  Bd.  ccvii.  p.  245.  Suckling,  Brain, 
1887,  July,  p.  241.  Senator,  Zeit.  f.  klin.  Med.,  Bd.  xiii.  p.  252.  See  Charcot's 
paper,  op.  cit.,  for  a  brief  discussion  of  the  history,  with  references  to  early  cases. 


OPHTHALMOPLEGIC    MIGRAINE  713 

In  ophthalmoplegic  migraine  we  have  that  motor  type  of  the  dis- 
ease, to  which  reference  has  already  been  made,  in  distinction  from  the 
sensory  or  ophthalmic  type,  which  has  just  been  described.  This  dis- 
tinction seems  warranted  by  the  fact,  as  pointed  out  by  Charcot  and 
others,  that  the  ophthalmic  aura,  such  as  the  scintillating  scotoma,  is 
often  absent  in  this  motor  form,  whereas  the  paralysis  of  some  of  the 
ocular  muscles  is  pronounced  and  characteristic. 

Ophthalmoplegic  migraine  generally  pursues  about  the  following 
course:  The  attack  is  usually  divided  into  two  periods.  First,  the 
period  of  pain.     Second,  the  period  of  paralysis. 


Fig.  2.— Paralysis  of  the  right  third  nerve,  to  illustrate  ophthalmoplegic  migraine,  although  in  this 
case  the  disease  was  brain  syphilis. 

The  period  of  pain  begins  brusquely,  and  the  pain  itself  is  localized 
upon  one  side.  This  localization  is  more  marked  than  in  ordinary 
migraine.  The  pain  is  often  centred  about  the  eye, — i.e.,  in  the  oph- 
thalmic division  of  the  fifth  nerve.  The  visual  aura,  such  as  scotoma 
scintillans,  hemianopsia,  etc.,  is  usually  absent.^^  Although  the  pain 
begins  suddenly,  it  does  not  reach  its  maximum  at  once,  but  gradu- 
ally increases  in  severity ;    and  although  it  is  usually  localized,  it  may 


"  Charcot,  op.   cit.     Suckling,  op.   cit.     Kollarits,   Deut.   Zeit.   f.   Nervenheilk., 
1904,  Bd.  xxvi.  H.  1,  2. 


714  THE    EYE    AND    NERVOUS    SYSTEM 

in  some  cases  extend  even  to  the  occiput.  But  it  almost  invariably 
remains  upon  one  side.  This  pain  may  become  most  severe.  Its  dura- 
tion varies  in  different  cases;  in  some  it  is  short,  a  matter  of  a  few 
hours;  in  others  long,  extending  over  some  days.  As  in  ordinary 
migraine  the  bout  of  pain  is  usually  terminated  by  vomiting.  This 
vomiting  may  be  in  the  nature  of  a  crisis;  it  appears  to  bring  relief, 
and  this  relief  is  prompt,  even  sudden.  In  other  words,  the  termination 
of  the  painful  period  is  as  abrupt  as  its  beginning. 

As  the  pain  and  vomiting  disappear  the  second  period,  or  period  of 
paralysis,  asserts  itself.  This  jDaralysis  has  certain  characteristics,  which 
are  quite  common  in  the  majority  of  cases,  although  exceptions  to  the 
rule  occur,  as  will  be  noted.  The  palsy  is  an  ophthalmoplegia,  and  in 
the  great  majority  of  cases  it  is  confined  to  the  third  nerve.  The  paraly- 
sis of  the  third  nerve  is  usually  total  and  complete.  That  is  to  say,  it 
is  total  in  the  sense  that  all  the  branches  of  the  nerve  are  involved,  and 
it  is  complete  in  the  sense  that  the  muscles  are  not  merely  weakened  but 
absolutely  paralyzed.  Hence  we  have  presented  the  classical  picture 
of  a  third-nerve  palsy,  such  as  occurs  from  an  organic  lesion.  The 
upper  eyelid  droops,  the  eyeball  is  rolled  outwards,  and  the  pupil  is 
dilated.  Moreover,  the  pupil  is  quite  immobile;  it  does  not  react  to 
light,  nor  upon  convergence  or  accommodation.  Thus  there  is  an 
ophthalmoplegia  both  externa  and  interna. 

The  duration  of  these  two  stages  varies,  as  we  have  said,  in  different 
cases.  The  period  of  pain  is  usually  shorter  than  the  period  of  paraly- 
sis. This  paralysis,  indeed,  may  last  for  a  comparatively  long  time. 
In  Schilling's  case  ^^  it  endured  for  from  four  to  six  weeks ;  in  Pari- 
naud's  case  for  from  two  to  three  months.  But  in  many  cases  the 
paralysis  disappears  in  a  few  days.^° 

Charcot  claimed  that  the  paralysis  is  always  confined  to  one  eye; 
it  never  shifts  from  one  eye  to  the  other,  and  in  a  given  case  it  always 
appears  in  the  same  eye  in  succeeding  attacks.  It  may  be  in  the  left 
eye  or  in  the  right  eye,  but  always  in  the  same  eye  in  the  same  patient. 
But  this  rule  has  been  found  by  other  observers  not  to  be  constant.  Thus 
Demicheri  ^^  observed  a  case  in  a  woman  aged  64  years  in  which  the 
palsy  appeared  first  on  the  left  and  then  on  the  right  side;  and 
Karplus  ^^  had  a  case  in  a  woman  aged  39  years  in  which  there  was 

"  Miinch.  med.  Woch.,  1903,  No.  18,  p.  776.  See  also  Wilbrand  and  Saenger, 
Die  Neurologic  des  Auges,  1,  2  Ab.,  p.  575. 

**  Manz,  op.  eit.;     Borthen,  Klin.  Monatsblat.  f.  Augenheilk.,  Sept.   1893. 
"La  Clinique  Ophthalm.,  1899,  18. 
"  Jahr.  f.  Psych.,  xii.  p.  158. 


OPHTHALMOPLEGIC    MIGKAINE  715 

a  left-sided  oculo-motor  paralysis  following  migraine,  which  reappeared 
on  the  right  side.     These  cases,  however,  appear  to  be  quite  exceptional. 

Some  cases  of  this  recurrent  palsy  of  the  oculo-motor  nerve  observe 
a  distinct  periodicity,  but  this  is  not  true  of  all.  Kollarits's  patient  had 
attacks  three  or  four  times  a  year.  In  Suckling's  case  they  occurred 
every  two  weeks;  in  one  of  Karplus's  patients  every  year.  In  women 
the  attacks  have  occasionally  been  seen  to  coincide  with  the  menses. 
On  the  other  hand  the  occurrence  has  sometimes  been  most  irregular 
and  at  very  long  intervals.  In  one  case  ^^  the  onset  was  in  the  patient's 
thirteenth  year,  and  the  attack  was  very  brief  (only  one  day)  ;  then 
after  an  interval  of  ten  years  the  disease  recurred,  and  later  became 
periodical  at  short  intervals — two  or  three  attacks  monthly.  Charcot 
called  special  attention  to  the  fact  that  the  duration  of  the  access  of 
ophthalmoplegic  migraine,  even  the  shortest,  is  usually  longer  than  the 
access  in  ordinary  migraine ;  and  he  thought  it  was  the  rule  that  in 
cases  in  which  the  attacks  were  short  the  intervals  between  the  attacks 
were  also  short.  When  the  attack  does  not  exceed  four  or  six  days,  the 
seizures  may  occur  as  often  as  once  a  month,  as  in  Senator's  case. 

The  onset  of  migraine  in  these  cases  is  often  at  a  very  early  age ; 
in  fact,  the  majority  of  them  seem  to  date  their  origin  from  childhood 
or  even  from  infancy.  Thus  in  one  case  ®^  the  first  attack  happened 
when  the  patient  was  only  a  year  old ;  the  second  attack  occurred  at 
seven,  and  thereafter  the  attacks  recurred  every  nine  or  ten  months.  In 
Suckling's  case  the  attacks  had  occurred  also  since  infancy;  and  de 
Schweinitz  ^^  has  reported  a  case  which  began  in  an  infant  one  year 
and  a  half  old,  and  which,  after  repeated  attacks,  left  a  permanent 
paralysis  of  the  third  nerve.  Not  a  few  of  the  cases  began  before  or  at 
the  age  of  puberty;  thus  cases  are  recorded  which  had  their  onset  at 
5^,  7,  8,  11,  13,  and  14  years. 

It  is  a  noteworthy  fact  that  there  is  often  a  change  of  type  in  these 
cases.  Thus  in  the  early  years  the  disease  presents  the  type  of  an 
ordinary  or  ophthalmic  migraine,  and  this  changes  later  to  the  ophthal- 
moplegic type.  This  seems  to  prove  that  the  latter  form  is  a  genuine 
migraine,  and  not  a  different  disease,  as  some  observers  have  claimed. 
This  fact,  however,  does  not  avoid  the  inference  that  some  few  excep- 
tional cases  of  recurrent  palsy  of  the  third  nerve  are  not  cases  of  true 

"  Darkschewitsch,  Arch.  f.  klin.  Med.,  Bd.  xlix.  S.  457. 

'°  Ormerod  and  Holmes  Spicer,  A  Case  of  Recurrent  Paralysis  of  the  Third 
Nerve,  with  Migraine,  in  Lancet,  Dec.  21,  1895,  p.  1580. 

*^  Recurrent  Oculomotor  Palsy,  with  a  Case,  in  Boston  Med.  and  Surg.  Journ., 
1895,  p.  341. 


Tie  THE    EYE    AND    NERVOUS    SYSTEM 

migraine,  a  fact  which  will  be  discussed  under  the  head  of  diagnosis. 
In  cases,  however,  in  which  there  is  this  change  of  type  the  correlated 
facts  are  too  obvious  to  admit  of  a  doubt  that  the  two  types  are  really 
the  expression  of  one  and  the  same  morbid  entity.  Chabbert  ®^  reports 
the  case  of  two  sisters  who  had  ophthalmic  migraine  with  scotoma  scin- 
tillans,  hemianopsia,  etc.  In  one  of  these  cases  at  52  years  the  attacks 
became  complicated  with  right-sided  oculomotor  palsy.  Joachim  ®^  had 
a  patient  who  had  ordinary  migraine  from  the  eleventh  year;  at  twenty 
years  of  age  she  suddenly  developed  oculomotor  palsy  with  diplopia,  etc., 
after  a  rather  slight  attack.  Somewhat  similar  cases  have  been  reported 
by  Stezeininski,^^  Bouchaud,*"^  Stock,""  Senator,"^  and  Lapersonne.*® 
In  Bouchaud's  case  the  oculomotor  palsy  developed  in  a  woman  aged 
sixty  years  who  earlier  in  life  had  had  migraine.  Charcot  ®®  records  a 
case  in  which  ordinary  migraine  had  existed  in  early  life  and  had  ceased 
in  the  patient's  twenty-third  year;  but  in  her  thirtieth  year  she  began 
with  attacks  of  recurring  oculomotor  palsy.  It  is  impossible,  as  Char- 
cot says,  not  to  see  some  relationship  between  these  two  types,  even 
widely  separated  as  they  were  in  time. 

It  has  been  observed  in  some  cases  that  after  repeated  attacks  of 
ophthalmoplegic  migraine  the  paralysis  tends  to  become  more  and  more 
persistent  and  even  in  time  to  become  permanent.  This  is  true  espe- 
cially of  those  cases  in  which  the  period  of  paralysis  is  prolonged  and 
occurs  at  prolonged  intervals.  In  such  cases  the  intervals  between  the 
attacks  are  not  always  entirely  free  from  the  appearance  of  some  paresis ; 
and  even  the  pain  may  persist  in  slight  degree.  Senator,  noting  these 
facts,  said  that  the  disease  was  not  so  much  periodical  as  it  was  "  con- 
tinuous with  periodical  exacerbations."  The  suggestion  of  an  organic 
lesion,  such  as  an  inflammation  of  the  nerve-trunk,  is  likely  to  occur  to 
the  observer  of  these  cases;  and  in  fact  some  such  organic  lesion  may 
be  the  cause  of  the  permanent  palsy.  In  Schilling's  case  there  was  some 
interparoxysmal  paresis ;  as  also  in  Ballet's  case.^"  In  a  case  recorded 
by  Karplus"^  a  permanent  ptosis  was  left;    an  interesting  instance  of 


"Le  Progrfis  Med.,  1895,  No.  15,  p.  241. 

"Deut.  Arch.  f.  klin.  Med.,  Bd.  xliv.  p.  185. 

"  Rec.  d'Ophthalmol.,  September,  1897. 

*»Pres8e  M4d.,  Avril  28,  1897. 

"  Cited  by  Paderstein,  loc.  cit. 

•'  Loc.  cit. 

"Progrf's  MM.,  1903,  No.  10,  p.  161. 

"Op.  cit. 

"De  la  Migraine  Ophthalmoplegique,  Thfese  de  Paris,  1896. 

"  Jahr.  f.  Psych.,  vol.  xii.  p.  158. 


OPHTHALMOPLEGIC    MIGRAINE  T17 

only  one  branch  of  the  third  nerve  being  permanently  injured.  In  de 
Schweinitz's  case  in  a  child  a  divergent  squint  remained  in  the  intervals, 
but  the  ptosis  appeared  and  disappeared  with  each  succeeding  attack 
until  the  twenty-eighth  year,  when  the  ptosis  too  became  permanent.  In 
Ormerod  and  Spicer's  case  some  of  the  paralyzed  muscles  had  never 
recovered.  Whatever  may  be  the  explanation  of  this  permanent  palsy 
in  a  disease  which  is  usually  called  "  functional,"  it  is  necessary  for  the 
present  to  hold  in  abeyance  any  theor}'^  which  does  not  include  the 
possibility  of  an  organic  lesion.  It  is  likely  indeed  that  this  permanent 
injury  to  the  motor  nerves  is  the  result  of  some  irritant,  such  as  a  toxin, 
acting  as  the  essential  cause  of  migraine. 

The  paralysis  in  ophthalmoplegic  migraine  is  not  always  limited  to 
the  third  nerve,  and  in  a  few  rare  cases  the  third  nerve  has  escaped 
•entirely,  while  the  fourth  or  the  sixth  nerve  has  been  implicated.  Leiz- 
enberger  ''^  reported  the  case  of  a  man  aged  29  years  who  had 
migrainous  attacks  every  eight  days  followed  by  isolated  paralysis  of 
the  trochlear  nerve.  Ordinarily  when  the  fourth  nerve  is  involved,  it 
is  in  association  with  the  third  nerve,  as  in  the  case  recorded  by  Cou- 
touzis,'^^  in  which  there  was  conjoint  palsy  of  the  oculomotor  and 
trochlear  nerves  with  violent  headache.  The  attacks  were  distinctly 
migrainous,  with  pain  in  the  parietal  region,  vomiting  and  salivation 
for  forty-eight  hours,  followed  by  the  loss  of  power  in  the  two  nerves. 
There  was  no  scotoma  or  hemianopsia.  The  paralysis  lasted  for  twelve 
days,  but  longer  in  the  superior  rectus  muscle. 

Paralysis  of  the  sixth  nerve  has  been  observed  in  a  small  number 
of  cases.  Charcot  has  made  such  a  case  the  subject  of  a  detailed  report.''^^ 
The  patient,  aged  41  years,  had  ordinary  migraine  beginning  at  the 
age  of  16,  which  ceased  at  32  years,  to  be  followed  at  38  years  by  a 
violent  attack  of  left-sided  neuralgia  without  nausea  or  vomiting,  and 
lasting  for  four  hours.  Diplopia  resulted,  and  was  due  to  an  isolated 
paralysis  of  the  abducens  nerve,  lasting  for  five  days.  After  an  interval 
of  two  years  a  similar  attack,  but  on  the  right  side,  occurred,  and  was 
followed  by  paralysis  of  the  oculo-motor  nerve ;  six  months  later  oc- 
curred still  another  attack,  but  on  the  left  side,  followed  by  paralysis 
again  of  the  sixth  nerve.  Charcot  was  obliged  to  conclude  from  this 
case  that  the  attacks  are  not  always  unilateral ;  the  fact  was  shown,  how- 
ever, that  the  third  and  sixth  nerves,  although  paralyzed  in  opposite 
eyes,  were  paralyzed  each  on  the  same  side  in  each  attack.    This  palsy  in 

"  Abstracted  in  Neur.  Cent.,  xvii.,  2,  p.  73. 

"  Journ.  de  Med.  et  de  Chir.  Prat.,  Avril  25,  1897. 

''*  Rev.  Neurolog.,  5,  8. 


718  THE    EYE    A:N^D    NERVOUS    SYSTEM 

Charcot's  case  was  long  continued,  but  not  in  all  branches  equally,  for 
the  levator  paliiebrae  and  superior  rectus  were  most,  or  longest,  involved. 
Paderstein  "^^  reported  a  case  in  which  there  was  a  paralysis  of  the  left 
abducens  nerve  and  a  weakness  of  the  left  superior  rectus  muscle,  with 
equal  pupils  and  no  paresis  of  the  levator  palpebral;  showing  again 
that  the  various  branches  of  the  third  nerve  are  not  always  equally  at 
fault.  De  Schweinitz  ''^  also  put  on  record  a  case  of  recurrent  palsy  of 
the  abducens  nerve,  which  began  when  the  patient  was  only  one  year 
old,  and  recurred  one  year  later,  and  again  in  five  years.  The  attack 
was  ushered  in  with  fever,  nausea,  vomiting  and  headache,  and  recovery 
after  each  attack  was  complete. 

A  very  rare  case  was  that  reported  by  Rossolimo,^"  in  which  a  recur- 
rent paralysis  of  the  seventh  nerve  was  always  preceded  by  a  migrainous 
attack.  The  paralysis  was  in  the  superior  as  well  as  the  inferior  dis- 
tribution of  the  nerve;  so  it  was  evidently  peripheral.  There  was  no 
involvement  of  ocular  muscles.  Rossolimo  referred  to  a  similar  case 
reported  by  Ilatscliek.^^ 

It  is  a  remarkable  fact  that  but  few  authors  make  mention  of  ansBS- 
thesia  of  the  fifth  nerve  in  these  cases  of  ophthalmoplegic  migraine. 
The  inference  is  that  such  anaesthesia  seldom  occurs.  When  we  con- 
sider the  fact  that  the  nerve-storm  vents  a  large  part  of  its  fury  on  the 
fifth  nerve,  causing  the  intense  headache,  and  that  the  cause,  whatever 
it  is,  is  sufficient  to  paralyze  motor  nerves,  it  seems  rather  remarkable 
that  the  sensory  nerve  is  not  also  paralyzed.  Knapp  ^°  has  reported  a 
case  in  a  man  aged  41  years  in  which  there  was  a  distinct  anaesthesia  in 
the  first  and  second  divisions  of  the  fifth  nerve.  The  attack  was  of  long 
duration,  and  the  sensory  condition  was  w^hat  is  known  as  anaesthesia 
dolorosa ;  that  is,  there  was  a  partial  loss  of  sensation,  the  face  feeling 
as  though  it  were  covered  with  leather,  and  objects  were  felt  as  through 
this  substance ;  associated  with  pain  on  pressure  or  even  on  slight 
touch.  Ilaynes  ^^  also  has  reported  a  case  in  which  the  eye  felt  sore, 
and  there  was  anaesthesia  of  the  supra-orbital  branch  of  the  fifth  nerve, 
accompanied  with  or  followed  by  ptosis.®^ 

"Deut.  Zeit.  f.  Nervenheilk.,  xv.,  5  u.  6,  p.  418. 

■"Philada.  Polyclinic,  vi.  39. 

"Neur.  Centbl.,  1901,  S.  744. 

"  1894,  Klin,  von  Nothnagel.     See  also  Knapp's  paper,  cited  below. 

™  Boston  Med.  and  Surg.  Jour.,  September  27,  1894,  p.  308.  Knapp  gives  a 
bibliography  including  many  of  the  reported  cases  of  recurrent  oculomotor  palsy 
up  to  that  date. 

""Recurrent  Ptosis,  in  N.  Y.  Med.  Journ.,  1897,  No.  7. 

"  See  the  case,  reported  by  the  present  writer,  of  unilateral  complete  and  total 


OPHTHALMOPLEGIC    MIGKAINE  719 

Some  authors  speak  of  recurrent  oculo-motor  palsy  without  pain,  and 
cases  have  been  reported,  but  they  are  probably  not  migrainous.*^  In 
fact  there  is  not  a  little  chance  of  confusing  cases  of  organic  paralysis 
of  the  third  nerve  with  cases  of  migraine.  This  subject  will  be  referred 
to  more  at  length  presently. 

Troemner  ^^  has  reported  a  rare  case,  namely,  ophthalmoplegia  in- 
terna following  severe  attacks  of  migraine.  There  was  no  palsy  of  the 
extraocular  muscles.  The  right  pupil  was  widely  dilated  and  immobile 
to  light.^^ 

Diagnosis. — Paralysis  of  the  oculo-motor  nerve  is  not  an  uncommon 
result  of  organic  diseases  of  the  brain,  among  the  most  common  of  which 
are  brain-tumors  and  brain-syphilis.  Both  these  lesions  are  productive 
of  pain,  and  in  both  of  them  the  palsy  of  the  third  nerve  may  be 
paroxysmal,  although  this  is  less  likely  to  be  so  with  tumors  than  with 
syphilis.  The  latter  affection  is  indeed  very  apt  to  cause  oculomotor 
palsy.  It  was  a  favorite  saying  with  Ricord  that  syphilis  puts  its  sign 
manual  on  the  third  nerve.  Thus  an  error  in  diagnosis  might  easily 
be  made  in  cases  which  at  first  view  may  appear  to  be  migrainous. 

In  order  to  establish  the  differential  diagnosis  the  history  and  course 
of  the  case  must  be  considered.  In  cases  of  brain-tumor  the  symptoms 
are  not  usually  limited  to  the  third  nerve ;  there  are  other  evidences  of 
intracranial  disease,  such  as  choked  disk,  possibly  convulsions,  and  other 
and  more  widespread  paralyses.*^  Moreover  the  symptoms  do  not  inter- 
mit so  markedly  as  in  migraine ;    the  course  is  steadily  progressive.*^ 

ophthalmoplegia,  with  anaesthesia  of  the  fifth  nerve  and  optic  atrophy,  in  a  woman 
with  syphilis.     (Univ.  Med.  Mag.,  October,  1899.) 

It  is  well  also  to  be  on  guard  against  a  mere  hysterical  anaesthesia  in  the  terri- 
tory of  the  fifth  nerve.  The  present  writer  has  reported  (Phila.  Med.  Journ.,  March 
30,  1901)  a  ease  of  Bell's  palsy  in  which  the  whole  side  of  the  face  and  head  was  the 
seat  of  an  hysterical  anaesthesia.  It  is  to  be  noted  in  such  cases  that  the  anaesthesia 
is  not  confined  strictly  within  the  limits  of  the  fifth  nerve,  for  it  is  a  peculiarity  of 
hysterical  anaesthesia  everywhere  not  to  be  located  within  the  exact  distribution  of 
any  particular  nerve. 

^  Schmidt-Rimpler,  Handbuches  von  Xothnagel,  Bd.  xxi. 

'^Cent.  f.  Xervenheilk.,  October,   1899. 

"  The  following  papers  on  the  general  subject  of  ophthalmoplegic  migraine  have 
not  been  obtainable:  Marina,  Ueber  Multiple  Augenmuskellahmung,  Wien,  1896; 
Chiarini,  La  Emicrania  Optalmoplegica,  Rif.  Med.,  1895,  xi.  169;  Graef,  Mauth 
Vortrag.  xiii.  398. 

'^Riehter  (Arch.  f.  Psych.,  1887,  Bd.  ix.  p.  259)  records  a  case  of  recurrent 
palsy  of  the  third  nerve  caused  by  a  fibrochondroma  of  the  dura  involving  the  nerve. 
There  were  epileptic  attacks,  and  the  case  difi"ered  otherwise  from  the  syndrome  of 
ophthalmoplegic  migraine.     See  also  Karplus   (Wien.  klin.  Woch.,  1895,  No.  50). 

^^  For  a  case  of  brain-tumor  causing  paralysis  of  the  third  nerve  see  a  report  by 
the  present  writer   (A  Case  of  Tumor  of  the  Mid-Brain  and  Left  Optic  Thalamus, 


720  THE    EYE    AND    NERVOUS    SYSTEM 

In  the  case  of  brain-syphilis  the  difficulty  of  making  a  diagnosis  may  be 
greater,  and  in  fact  some  of  the  reported  cases  of  recurrent  oculo-motor 
palsy  have  probably  been  of  syphilitic  origin.  The  history  of  many 
preceding  attacks,  always  the  same,  would  be  in  favor  of  migraine,  as 
would  also  the  transitory  nature  of  the  attack  in  most  cases.  Optic 
neuritis  may  or  may  not  be  present  in  brain-syphilis;  it  is  never  seen 
in  ojjhthalmoplegic  migraine.  In  case  the  history  is  obscure  great  care 
should  be  exercised  in  making  this  diagnosis.^' 

The  nuclear  ophthalmoplegia,  described  by  Hutchinson,  is  an  insidi- 
ous and  progressive  disease  in  which  as  a  rule  one  nucleus,  and  there- 
fore one  nerve  branch,  after  another  is  involved,  and  in  which  there  is 
not  a  tendency  to  periodical  recurrence  and  recovery.  Moreover  the 
affection  is  usually  bilateral,  and  not  accompanied  with  pain,  although 
in  the  case  reported  by  the  present  writer  pain  was  a  symptom. 

A  history  of  syphilis  can  be  elicited  in  some  of  these  cases  of  ophthal- 
moplegic migraine,^^  but  that  is  not  necessarily  a  proof  that  this  affec- 
tion is  syphilitic.  In  the  great  majority  of  cases  there  is  no  such  history 
recorded. 

A  basilar  pachymeningitis  has  been  known  to  cause  a  one-sided 
oculomotor  palsy  with  symptoms  resembling  migraine.*®  The  same  has 
been  observed  in  a  case  of  tubercular  meningitis  by  Weiss,®®  in  whose 
case  there  was  tuberculosis  of  both  lungs,  and  a  recurrent  palsy,  the  last 
time  permanent,  of  the  left  third  nerve.  Wadsworth  ®^  records  a  case 
associated  with  otorrhea,  but  the  association  was  probably  a  mere  coin- 


in  the  Med.  News,  January  30,  1892,  p.  129).  In  this  case  there  was  a  crossed 
paralysis, — i.e.,  the  third  nerve  on  the  side  of  the  lesion  and  the  arm  and  leg  on  the 
opposite  side  were  involved.  This  crossed  paralysis,  due  to  a  lesion  in  the  mesen- 
cephalon, has  been  called  by  the  French  the  syndrome  of  Weber.  It  is  signifi- 
cant of  an  organic  lesion,  although  it  may  be  closely  simulated  by  hysteria. 

"  The  present  writer  has  recorded  three  cases  of  ophthalmoplegia  of  various 
kinds,  all  of  organic  origin.  The  first  was  a  case  of  total  and  complete  unilateral 
ophthalmoplegia  (interna  and  externa)  caused  by  a  syphilitic  neoplasm  at  the  base 
of  the  brain  just  behind  the  orbit.  (Annals  of  Ophthalmology,  January,  1898.)  The 
second  was  a  case  of  nuclear  ophthalmoplegia  in  a  woman,  closely  following  the  type 
described  by  Hutchinson.  In  this  ease  the  palsy  was  not  unilateral,  neither  was  it 
total ;  that  is  to  say,  not  all  the  orbital  muscles  were  involved.  ( Philada.  Med. 
.Journ.,  June  3,  1899.)  The  third  case  was  one  of  unilateral  ophthalmoplegia,  total 
and  complete,  associated  with  anaesthesia  in  the  fifth  nerve  and  atrophy  of  the  optic 
nerve — probably  caused  by  syphilis.     (University  Med.  Mag.,  October,  1899.) 

"•  Mengazzini,  cited  by  Mobius,  records  a  syphilitic  case.  See  also  Leaffer,  Berl. 
klin.  Woeh.,  1900,  No.  30,  p.  657. 

^Gubler  (Dis.  Inaug.,  Dresden,  1897).  See  also  Ziehen  (Neurol.  Centblt.,  1899, 
xviii.  4,  p.  173).     Also  de  Schweinitz's  case   (Philada.  Polyclinic,  vi.  39). 

""  Wien.  med  Woch.,  1885,  17. 

"'  Trans.  Am.  Ophthal.  Soc.,  1887.  iv.  460. 


OPHTHALMOPLEGIC    MIGRAIIS^E  Y21 

cidence.  The  periodicity  of  some  of  these  eases  has  naturally  suggested 
malaria  as  a  cause,^^  but  the  relationship  is  not  clear;  although  the 
present  writer  has  seen  a  case  of  transient  paralysis  of  some  of  the 
ocular  muscles  in  a  well-marked  case  of  malarial  poisoning  in  his  service 
at  the  Methodist  Hospital.  Remak  ^^  has  recorded  a  case  of  ocular 
palsy  in  a  case  of  juvenile  muscular  atrophy,  but  it  has  no  bearing  on 
the  subject  of  migraine.  Thompson  ®^  had  a  case  associated  with  con- 
vulsions and  dementia  in  a  child  aged  5  years,  and  one  of  Joachim's 
cases  was  in  a  backward  child  who  also  had  epileptiform  convulsions.®^ 

In  a  few  cases  of  recurrent  oculo-motor  palsy  there  has  been  noted 
a  nasal  catarrh.  This  was  seen  in  Knapp's  case ;  also  in  a  case  recorded 
by  Ahlstrom.®*'  The  latter  observer  thought  the  catarrh  was  the  cause 
of  the  palsy,  which  he  believed  was  "  reflex."  But  the  query  arises 
whether  the  catarrh  may  not  be  an  effect  of  the  involvement  of  the  fifth 
nerve  supply  to  the  mucous  membrane  of  the  nose.  The  symptom  has 
not  been  recorded  in  many  cases.  Mobius  ®^  and  Bernhardt  ®*  suggested 
an  **  autoinfection"  as  the  cause,  or  it  may  be  "  rheumatic."  These 
ideas  are  merely  speculative. 

In  a  case  recorded  by  Karplus  in  which  the  attacks  occurred  as  a 
rule  every  two  or  three  months,  no  seizures  occurred  during  pregnancy. 
This  exemption  is  analogous  to  what  is  seen  in  epilepsy  in  not  a  few- 
cases. 

Some  observers  have  seen  a  relationship  between  tabes  dorsalis  and 
migraine;  a  relationship  which  is  suggested  especially  by  recurrent 
palsy  of  the  third  nerve  in  locomotor  ataxia.  Thus  Oppenheim  ®®  in 
85  cases  of  tabes  found  12  cases  with  attacks  of  migraine,  only  two  of 
which,  however,  were  of  the  ophthalmoplegic  type.  He  claimed  that 
migraine  is  a  frequent  symptom  of  beginning  tabes.  But  it  seems  of 
doubtful  propriety  to  call  the  recurring  palsies  of  the  third  nerve  in 
tabes  instances  of  migraine.  They  are  due  to  the  pathological  process 
underlying  the  tabes,  and  the  history  and  clinical  course  are  not  the 
same  as  in  migraine.  It  is  desirable,  moreover,  to  avoid  mistakes, 
such  as  overlooking  the  onset  of  locomotor  ataxia,  and  calling  a  tabetic 
third-nerve  palsy  a  symptom  merely  of  migraine.     The  same  may  be 

»=  Klialschkin,  Xeurolog.  Centblt.,  1897,  xvi.,  5. 
"'Neurolog.  Centblt.,  1884. 
"Char.  Ann.,  188-5,  p.  67. 
*^  Jahrb.  b.  Kinderheilk.,  Bd.  xxviii.  p.  101. 
•"Ophthal.  Rev.,  xv.  191. 
»•  Berl.  klin.  Woch.,  1884,  38. 
'"Ibid.,  1889,  33. 
»»Ibid.,    1884,  38. 
40 


722  THE    EYE    AND    NERVOUS    SYSTEM 

said  of  general  paresis,  of  which  disease  also  Oppenheim  *°*'  sees  an 
early  symptom  sometimes  in  ophthalmic  migraine.  As  in  the  ophthal- 
mic type  of  migraine,  so  in  the  ophthalmoplegic  type,  hemiparesis  and 
aphasia  have  been  noted. ^^^  It  is  well  to  be  on  the  lookout  in  such  cases 
for  hysteria. 

As  already  said  (page  709)  hysteria  may  complicate  migraine,  but 
the  instances  in  which  hysterical  stigmata  have  been  noted  in  ophthal- 
moplegic migraine  are  not  numerous.  Ilasner  ^^^  had  a  patient  who 
every  month  had  a  paralysis  of  the  oculo-motor  nerve,  lasting  three  days, 
and  coinciding  with  the  appearance  and  disappearance  of  the  menses, 
but  this  fact,  although  it  suggests,  does  not  prove  hysteria.  Jacobi  ^^^ 
has  reported  a  case  of  hysterical  paralysis  of  the  right  third  nerve, 
which  was  complete  except  in  the  superior  oblique  and  the  pupil ;  the 
latter,  while  it  was  dilated,  would  react  slowly  to  light.  There  was  no 
migraine,  and  cure  was  obtained  by  suggestion.  In  studying  hysterical 
affections  of  the  muscles  of  the  eye  it  is  important  not  to  confuse  paraly- 
sis with  blepharospasm.  The  latter  is  much  the  more  common.*®*  Hys- 
terical affections  of  the  eye  and  of  the  eye-muscles  are  very  numerous 
and  not  a  little  complicated,  and  some  of  them  may  simulate  the  eye 
symptoms  of  migraine.  This  is  true  especially  in  cases  in  which  there 
is  an  hysterical  amaurosis  combined  with  either  a  blephorospasm  or  a 
spastic  strabismus  simulating  paralysis  of  some  of  the  muscles  supplied 
by  the  third  nerve,  or  possibly  the  sixth  nerve. 

Hysterical  blindness  or  amaurosis  is  a  rare  affection;  in  this  con- 
dition there  is  loss  of  vision.  But  a  much  more  common  affection  is 
a  contraction  of  the  visual  fields,  which  might  simulate  a  scotoma  to  a 
careless  observer.  In  hysteria  there  is  also  a  reversal  of  the  normal 
color  fields,  the  field  for  red  being  larger  than  the  field  for  blue.  These 
hysterical  affections  of  the  visual  fields  are  always  associated  with  other 
hysterical  stigmata,  and  they  are  essentially  unlike  the  visual  aura  of 
ophthalmic  migraine.  In  hysteria  there  is  not  the  scintillating  scotoma, 
and  the  disorder  in  the  visual  field  is  rather  a  contraction  than  a  true 
scotoma.  In  some  very  rare  cases  (one  of  which  has  been  put  on  record 
by  the  present  writer  *^^)  a  true  hemianopsia  has,  however,  been  ob- 

'»»Char.  Ann..  1890,  Bd.  xiv. 

">'  Hinde  and  Moyer,  N.  Y.  Med.  Rec,  1887,  418. 

""Prag.  med.  Woch.,  1883,  No.   10. 

"»Am.  Journ.  Obstet.,  1876,  p.  223. 

""Gilles  de  la  Tourette,  Hysterie,  chap.  ix.  The  subject  is  discussed  at  great 
length  by  Tourette,  who  seems  to  be  sceptical  about  a  hysterical  paralysis  of  the 
interior  muscles  of  the  eye. 

'"*  Article  on  Hysteria  in  Dercum's  Text-Book  of  Nervous  Diseases,  by  American 


PSYCHICAL    MIGRAINE  723 

served  in  hysteria.  But  the  visual  aura  in  migraine,  as  we  have  seen, 
is  very  transient,  differing  in  this  respect  from  the  visual  disorders  of 
hysteria.  » 

As  Tourette  has  pointed  out  in  great  detail,  following  especially 
Borel,^"^  the  muscles  of  the  eye  in  hysteria  are  not  so  much  the;  seat  of 
paralysis  as  of  spasm.  In  fact  it  may  be  a  question  whether  there  is 
a  true  hysterical  paralysis  of  the  third  nerve,  and  whether  the  reported 
cases  have  not  really  been  instances  of  blepharospasm  and  of  spastic 
strabismus.  In  that  curious  affection  known  as  monocular  diplopia,  in 
which  the  hysterical  patient  sees  double  with  one  eye,  the  cause  is  prob- 
ably a  spasm  of  tlie  muscle  of  accommodation,  causing  irregular  con- 
traction of  the  crystalline  lens.^"'^  So  in  the  pseudo-paralysis  of  the 
third  nerve,  studied  especially  by  French  observers,  the  affection  is  in  re- 
ality a  contracture  of  the  orbicularis  muscle  causing  an  apparent  droop- 
ing of  the  upper  lid.  Blepharospasm,  or  contracture  of  the  orbicularis, 
is  indeed  the  commonest  hysterical  affection  of  the  eye-muscles.  Iso- 
lated paralysis  of  any  of  the  orbital  muscles,  and  especially  of  the  intra- 
ocular muscles,  as  of  the  iris  or  ciliary  muscle,  of  hysterical  origin,  is 
probably  never  seen,  although  apparent  cases  have  been  reported ;  but 
the  strabismus  is  probably  always  due  not  to  a  paralysis  but  to  a  spasm 
of  one  or  other  of  the  ocular  muscles.  This  seems  to  be  the  conclusion 
of  Tourette,  who  has  traversed  this  subject  with  great  care,  and  to  whose 
work  the  reader  is  referred  for  details. 

From  what  has  been  written  it  is  seen  that  the  distinction  between 
the  ophthalmoplegia  of  migraine  and  the  blepharospasm  and  spastic 
strabismus  of  hysteria  is  not  a  subject  of  great  difficulty  in  the  hands 
of  a  careful  observer. 

PSYCHICAL  MIGRAINE. 

Mental  disorders  of  various  kinds  have  been  reported  by  many 
observers  of  migraine.  The  subject  was  discussed  at  length  by  Live- 
jjjg  108  ^jjQ  gave  many  instances  which  he  had  culled  from  the  literature. 

Authors,  p.  112.  In  that  article  the  hysterical  affections  of  the  eye  are  discussed  in 
more  detail  than  there  is  room  for  in  the  present  paper.  See  Wecker  et  Landolt 
(Traits  Comp.  d'Ophthalmologie,  article  on  Amblyopic  Hysterique)  for  references 
to  a  number  of  cases  of  hysterical  hemianopsia. 

'°*  Affections  Hysteriques  des  Muscles  Oculaires,  Ann.  d'Ocul.,  Novembre,  De- 
combre,  1887. 

'"^  Tourette,  op.  cit.     Also  Parinaud,  quoted  by  Tourette. 

""Op.  cit.,  p.  107.  Liveing  was  a  most  diffuse  writer  and  a  great  compiler,  and 
some  of  his  quotations  are  perhaps  wide  of  the  mark ;  but  his  passages  on  the 
psychical  disorders  of  migraine  form  an  interesting  contribution  to  this  subject, 
and  more  complete  than  any  other  work  of  which  the  present  writer  has  knowledge. 


724  THE    EYE    AND    NERVOUS    SYSTEM 

He  divided  these  disorders  intx)  two  groups,  the  intellectual  and  the 
emotional. 

Lebert  in  describing  his  own  symptoms  said  that  his  seizures  began 
witli  some  incoherence  of  ideas  and  difficulty  in  finding  words.  Others 
have  described  confusion  of  intellect  and  impairment  of  memory.  Even 
a  partial  loss  of  consciousness  has  been  experienced.  Some  of  these 
affections  of  mind  are  accompanied  with  a  sensation  of  numbness  in  the 
extremities,  face,  and  tongue,  and  with  an  affection  of  the  speech,  which 
will  be  referred  to  again.  Some  permanent  impairment  of  mind  has 
been  observed  in  migrainous  patients  who  have  suffered  with  severe 
and  oft-repeated  attacks.  Elaborate  descriptions  have  been  indulged  in 
by  some  of  these  patients,  as  one  old  man,  referred  to  by  Liveing,  who 
said  that  as  the  visual  phenomena  passed  off,  his  memory  usually  failed 
so  much  that  for  a  time  he  was  mentally  incapacitated,  and  whatever 
he  read  or  did  during  that  period  left  no  impression ;  he  experienced  a 
singular  disorder  of  ideation ;  circumstances  and  events  of  long  before 
were  brought  back  to  him  as  if  actually  present ;  his  consciousness 
appeared  to  be  doubled,  and  the  past  and  present  were  confused. 

Emotional  disorders  are  not  uncommon  during  the  first  stage  of 
migraine.  The  state  of  mind  in  some  patients  is  one  of  fright  or  great 
dread.  They  know  what  is  in  store  for  them  and  fear  it;  but  the 
apprehension  or  demoralization  seems  in  some  cases  out  of  proportion 
to  the  cause.  This  has  been  acknowledged  by  migrainous  patients  them- 
selves.^"^ The  visual  aura,  with  its  ugly  scintillating  scotoma,  is  par- 
ticularly disturbing,  and  quite  unnerves  some  individuals ;  so  much  so 
indeed  that  they  avoid  speaking  or  even  thinking  of  it  during  the  inter- 
vals, as  in  a  case  referred  to  by  Airy.  This  vague  and  unaccountable 
fear  supplies  another  analogy  with  epilepsy,  in  which,  as  is  well  known, 
a  sense  of  terror  is  often  experienced  during  the  brief  aura. 

As  we  have  said  before,  this  mental  state  of  the  migrainous  patient 
is  well  adapted  to  excite  hysterical  symptoms,  and  it  is  rather  remarka- 
ble that  more  has  not  been  written  on  this  topic.  Possibly  some  of  the 
anaesthesias,  tinglings,  and  speech  defects  are  truly  hysterical,  but  there 
is  a  dearth  of  cases  in  which  the  subject  of  hysterical  stigmata  has  been 
put  to  the  test.  The  severe  suffering  of  the  patient,  following  so  soon 
on  the  aura,  may  mask  such  symptoms,  and  does  not  invite  to  a  close 
examination  for  them. 

Mental  depression,  not  only  during  the  aura,  but  even  between  the 


'"An  early  writer  who  reeon;nized  this  inordinate  dread  was  Dwight,  who  gave 
a  good  description.     (Medical  Repository,  vol.  ii.  p.  16,  New  York,  1800.) 


PSYCHICAL    MIGEAIXE  725 

paroxysms,  is  present  in  some  patients.  It  may  be  explained  by  the 
great  suffering  and  the  frequent  incapacity  which  results  from  the 
attack. 

In  endeavoring  to  explain  the  mental  symptoms  in  migraine  two 
points  of  view  present  themselves.  First,  the  psychical  disorder  may 
be  due  to  the  dread  of  the  approaching  suffering  and  to  the  suffering 
itself.  Great  pain,  frequently  repeated,  is  demoralizing  to  the  human 
soul.  The  ancients  said  that  physical  pain  w^as  the  greatest  evil  that 
man  could  endure.  Second,  the  mental  symptoms  may  be  looked  upon 
as  an  integral  part  of  the  nerve  storm.  This  seems  to  be  true  from 
the  fact  that  strong-minded  men  have  endeavored  in  vain  to  control  the 
emotional  depression,  the  fear  and  anxiety,  which  sometimes  precede 
the  onset  of  the  pain.  In  other  words,  there  is  a  true  psychosis  attend- 
ant upon  the  attack  of  migraine.^ ^° 

Disorders  of  speech  have  also  been  noted  by  not  a  few  observers  as 
occurring  in  migraine.  Liveing  ^^^  has  collected  much  curious  informa- 
tion on  this  subject.  Oppenheim  ^^^  says  that  he  has  observed  aphasia, 
combined  with  agraphia.  Word-deafness  has  been  observed  in  only  a 
few  cases.  The  disturbance  lasts  from  a  few  minutes  to  half  an  hour, 
and  is  succeeded  by  the  headache,  which  is  always  upon  the  left  half 
of  the  head.  This  fact,  of  course,  suggests  that  the  speech  defects 
are  due  to  a  temporary  affection  of  the  speech  centres  in  the  left  cere- 
brum. What  this  affection  is  must  be  as  yet  a  mere  matter  of  conjec- 
ture; but  it  seems  necessary  to  suppose  that  it  is  some  material  affec- 
tion, something  acting  to  disturb  possibly  the  blood  supply  or  the  nutri- 
tion of  the  brain  cells  of  the  speech  centres  in  the  cortex.  Liveing, 
although  he  wrote  before  much  was  definitely  known  about  the  speech 
centres,  saw  the  possible  significance  of  these  speech  disorders,  and  col- 
lected a  number  of  cases  which  tended  to  show  that  the  migraine  in  these 
instances  was  on  the  left  side  of  the  brain.  He  noted  that  migraine  is 
often  a  one-sided  affection,  and  the  thought  occurred  to  him  to  investi- 
gate whether  the  left  side  was  most  often  affected  in  these  cases.  Thus 
he  found  that  in  Lebert's  own  case  the  speech  disorder  was  accompanied 
with  a  numbness  in  the  tongue  and  fingers  of  the  right  hand.  In  sev- 
eral other  cases  the  phenomena  were  similar ;  there  was  speech  disorder, 
with  numbness  on  the  right  side,  as  in  the  hand  and  arm,  neck  and  face. 

"""Oppenheim,  Dis.  Nerv.  Syst.,  p.  751.  The  subject  has  been  recognized  also 
by  Griesinger  and  Krafft-Ebing.  The  psychosis  according  to  these  observers  is  one 
of  excitement,  confusion,  or  stupor. 

'"  Op.  cit.,  p.  93.  Localizing  data  are  still  wanting  in  migraine.  See  ante, 
p.   10. 

"'  Op.  cit.,  p.  751. 


726  THE    EYE    AND    NERVOUS    SYSTEM 

In  Abercrombie's  case  the  numbness  (aura)  extended  upwards  from  the 
fingers  of  the  right  hand,  and  was  followed  by  impairment  of  speech. 
Some  of  these  cases  were  very  striking,  and  seem  to  he  more  than  mere 
coincidences.  Of  15  cases  thus  analyzed  by  Liveing,  in  7  the  numbness 
was  on  the  right  side ;  in  4  it  was  bilateral,  while  in  the  remaining  cases 
the  report  was  defective.  But  in  no  single  instance  was  the  left  side 
of  the  body  alone  affected.  The  inference  seems  clear  that  the 
speech  defects  in  migraine  are  often  caused  by  a  left-sided  lesion  in 
the  brain. 

In  some  cases  reported  as  migraine  with  aphasia  it  is  possible  that 
Bright's  disease  may  have  been  a  factor,  for  this  affection  may  cause 
temporary  aphasia  with  headache.  The  writer  has  seen  a  well-marked 
example,  simulating  tumor  of  the  brain,  in  his  service  at  the  Philadel- 
phia Hospital. 

But  if  v.'e  are  to  follow  out  the  analogy  of  migraine  with  epilepsy, 
we  should  seek  for  a  true  psychical  migraine,  not  in  the  mere  presence 
of  psychical  symptoms,  but  in  the  substitution  of  such  symptoms  for 
the  classical  migrainous  attack.  Are  there  such  cases  of  pure  psychical 
migraine  ?  The  answer  seems  from  a  few  recorded  cases  to  be  in  the 
affirmative.  Sir  George  Airy^^^  observed  in  his  own  person  the  usual 
attack  complicated  with  transient  impairment  of  speech  and  memory 
without  either  headache  or  numbness.  Parry  ^^*  advanced  the  theory 
of  a  compensation  between  the  different  phenomena  of  the  seizure;  if 
the  disorder  of  sight  proved  abortive,  the  higher  cerebral  faculties  were 
more  likely  to  be  involved.  Tissot,  quoted  by  Liveing,  related  a  case  in 
which  attacks  of  habitual  migraine  were  at  length  completely  replaced 
by  fits  of  disordered  ideation — a  true  substitutional  attack,  or  pure  psy- 
chical migraine.  We  are  indebted  to  Liveing  for  more  information 
on  this  obscure  subject  than  is  supplied  by  most  later  writers. 

Among  other  mental  changes  is  the  state  known  as  double-conscious- 
ness."^ Vertigo  is  also  sometimes  experienced.  These  symptoms  indi- 
cate how  profoundly  the  mental  or  cerebral  functions  may  be  affected 
in  migraine.^ *^ 

Treatment. — The  treatment  of  migraine  is  entirely  symptomatic 
and  expectant.  There  is  no  specific  for  the  disease.  In  our  ignorance 
of  the  pathology  of  the  affection,  our  treatment  can  be  nothing  more 


"'  Philosophical  Magazine,  July,  1865. 

"*  Pathology  and  Therapeutics,  vol.  i.  p.  357,  quoted  by  Liveing. 

'"  Gowers,  Dis.  Nerv.  Syst.,  vol.  ii.  p.  842. 

"'  See  also  Bounal  for  a  case  with  epileptoid  symptoms,  Rev,  Mens,  de  M6d.  et 
Chir.,  1878,  ii.  279;  Gray,  The  Correlation  and  Interconvertibility  of  Migraine  and 
Epilepsy,  Pathologist,  1881,  i.  4. 


PSYCHICAL    MIGRAI:N^E  727 

than  empirical ;  and  this  fact  is  proved,  as  in  all  similar  cases,  by  the 
large  number  of  remedies  that  are  recommended  and  even  lauded  in 
excess.  From  quack  nostrums  to  spectacles  the  list  is  a  dreary  one,  and 
will  not  be  repeated  here. 

We  may,  however,  consider  briefly  a  few  of  the  more  approved  rem- 
edies. From  the  writer's  personal  experience  in  his  own  case  it  has 
seemed  that  an  abortive  treatment  is  always  worth  a  trial.  This  treat- 
ment consists  in  prompt  action  on  the  gastro-intestinal  tract.  A  saline 
laxative,  such  as  Rochelle  or  Epsom  salts,  taken  at  the  first  appearance 
of  the  visual  aura,  will  sometimes  cut  short  an  attack.  If  free  purgation 
is  obtained  in  an  hour  or  two,  the  attack  may  be  aborted  or  its  severity 
diminished.  The  same  is  true  of  an  emetic,  especially  if  the  attack 
comes  on  soon  after  the  patient  has  taken  a  hearty  meal.  Under  such 
circumstances  I  have  known  a  full  dose  of  ipecac  promptly  to  abort  the 
attack.  As  soon  as  the  stomach  is  emptied  the  hemicrania  diminishes 
and  soon  ends.  Although  the  remedy  is  not  pleasant,  it  is  pleasanter 
than  a  long  period  of  sick  headache  with  the  inevitable  emesis  that 
comes  anyhow  at  the  termination  of  the  attack.  It  is  but  proper  to  say, 
however,  that  in  some  of  the  more  severe  cases  these  remedies  addressed 
to  the  stomach  and  bowels  will  not  bring  entire  relief. 

The  symptomatic  treatment  is  usually  addressed  to  the  relief  of 
pain ;  and  here  the  extensive  list  of  anodyne  drugs  comes  into  play.  A 
popular  migraine  powder  at  present  is  composed  of  citrate  of  caffeine, 
monobromide  of  camphor,  and  acetanilid.  The  various  coal-tar  products 
are  often  appealed  to,  but  seldom  do  more  than  assuage  the  pain.  They 
are  not  curative,  and  they  are  therefore  often  disappointing.  Among 
them  are  antipyrine,  antikamnia,  and  phenacetine. 

Cannabis  indica  was  long  regarded  with  favor  in  migraine,  and 
it  is  extolled  by  Sinkler^^^  in  Ms  excellent  discussion  of  treatment. 
Of  recent  years,  however,  we  |||j^  little  of  it.  Remedies  which  are 
supposed  to  control  the  vasomotors,  such  as  ergot,  have  also  been  com- 
mended, but  on  purely  theoretical  grounds.  The  victim  of  migraine 
who  puts  his  trust  in  them  will  suffer  for  his  faith.  Nitrite  of  amyl  is 
said  to  give  relief  in  the  early  stage.  The  bromides  have  been  used 
extensively,  not  without  temporary  relief.^ ^®  So,  too,  of  chloral,  and 
croton  chloral.     Opiates,  and  especially  the  hypodermic  use  of  morphia, 

'"  Article,  Migraine,  in  Pepper's  Syst.  of  Med.,  vol.  v.  p.  413.  Also,  by  the  same 
author,  in  Hare's  Syst.  of  Pract.  Therapeutics,  vol.  iii.  p.  375. 

"*Tourette  et  Blocq  (Prog.  Med.,  xv.  476),  in  a  case  of  ophthalmic  migraine 
■with  transitory  aphasia,  obtained  good  results  with  bromides,  which  they  used  in 
doses  as  high  as  5  grammes  a  day. 


728  THE    EYE    AND    NERVOUS    SYSTEM 

will  deaden  the  pain,  but  the  practice  of  giving  morphia  in  this  way  to 
patients  who  have  frequent  attacks,  is  most  objectionable.  The  risk  of 
forming  the  habit  is  not  small.  Local  applications  of  all  sorts  have  been 
used ;   heat,  or  its  converse,  cold,  is  probably  among  the  best. 

The  expectant  treatment,  or  that  which  is  applied  to  warding  off 
the  attacks,  consists  merely  in  a  tonic,  hygienic,  and  restorative  regime. 
The  usual,  and  sometimes  useful,  drugs  for  this  purpose  are  quinine, 
iron,  nux  vomica,  arsenic,  and  phosphorus ;  but  it  is  the  irony  of 
therapeutics  to  recommend  these  drugs  as  in  any  sense  curative. 

Some  patients,  from  long  experience,  find  some  particular  drug  or 
method  best  suited  to  their  owti  cases,  but  the  fact  that  such  approved 
remedies  are  not  universally  effective  proves  how  much  the  personal 
equation  rules  in  migraine  as  in  other  neuroses.  It  is  still  a  disease 
that  favors  experimental  therapeutics,  and  the  stage  of  scientific  achieve- 
ment has  yet  to  be  reached. 

TETANUS. 

The  eye-symptoms  in  tetanus  are  few  in  number.  In  the  ordinary 
form  of  the  disease  these  symptoms  are  practically  nil,  but  in  that  rare 
and  interesting  kind  of  lockjaw  known  as  cephalic  tetanus  (the  Jcopf- 
tetanus  of  the  Germans)  there  is  occasionally  seen  a  paralysis  of  one 
or  more  ocular  muscles. 

In  cephalic  tetanus  the  disease  usually  takes  its  origin  in  a  wound 
situated  in  the  distribution  of  the  fifth  nerve.  The  tetanic  symptoms 
may  be  limited  to  the  head,  throat  and  neck,  although  in  some  cases 
there  is  also  opisthotonos,  and  even  general  tetanus.  The  peculiarity  of 
this  form  of  tetanus  is  that  there  is  an  associated  paralysis  of  the  seventh 
nerve.  The  muscles  of  deglutition  are  involved,  a  fact  which  led  Rose 
to  call  the  disease  tetanus  hydrophohicus.  But  this  is  an  unfortunate 
nam6,  for  the  affection  has  no  relati^fctovhatever  to  hydrophobia. 

Cephalic  tetanus  is  a  rare  affeoRon.  But  few  cases  have  been 
recorded  in  America.  The  most  complete  account  of  the  disease  ever 
given  in  this  country  was  putlished  by  Willard  ^^®  in  1895.  That 
report  was  based  upon  the  history  of  a  case  in  a  boy,  whose  attack  was 
caused  by  a  wound,  from  a  dirty  stick,  in  the  neighborhood  of  the  orbit, 
and  who  made  a  good  recovery. 

Willard  collected  the  references  to  74  cases  of  cephalic  tetanus,  his 
own  being  the  seventy-fifth  case.  There  were  57  males  and  15  females 
noted.     In  45  early  cases,  in  which  the  tetanus  developed  within  the 

'"Tetanus  Cephalic,  etc.,  Trans.  Col.  Phys.  of  Philada.,  1895,  3d  Series,  vol. 
xvii.  p.  27. 


TETANUS 


729 


first  week,  39  died  and  4  recovered.  In  32  later  cases,  in  which  the  symp- 
toms developed  after  the  first  week,  8  died  and  24:  recovered.  These 
figures  illustrate  the  fact  that  the  mortality  from  cephalic  tetanus  is 
rather  less  than  from  the  ordinary  form,  and  that  the  prognosis  is  much 
more  favorable  in  the  late  cases. 

The  ocular  muscles  have  been  involved  in  a  very  few  of  these  cases 
of  cephalic  tetanus.  In  the  first  place,  of  course,  the  orbicularis  muscle, 
which  is  supplied  by  the  seventh  nerve  and  is  not  properly  an  ocular,  or 
at  least  an  orbital,  muscle,  is  involved  along  with  the  facial  muscles 
supplied  by  the  facial  nerve.     The  eye  is  open,  as  in  all  cases  of  periph- 


FiG.  3.— Cephalic  tetanus,  with  paralysis  of  both  seventh  nerves.  This  picture  shows  the  patient 
in  the  attempt  to  close  her  eyes.  Trismus.  Ironed-out  expression.  Wound  on  nose.  The  eye  muscles 
were  not  involved,  although  the  right  eyeball  rolled  unduly  inward  in  the  attempt  to  close  the  eyes. 


eral  palsy  of  the  seventh  nerve,  a  fact  which  shows  that  the  paralysis 
is  peripheral.  There  has  been  some  discussion  as  to  the  exact  pathology 
of  this  peripheral  palsy ;  in  some  cases  examined  post-mortem  the  evi- 
dences of  neuritis  have  not  been  found  ;  and  during  life  the  reactions  of 
degeneration  have  not  been  marked.  In  Willard's  case  the  present 
writer  studied  the  electrotonus,  and  found  no  reactions  of  degenera- 
tion, although  the  examination  was  incomplete  owing  to  the  patient's 
excitement  and  resistance. 

In  a  very  few  cases  of  cephalic  tetanus  some  of  the  orbital  muscles 


730  THE    EYE    AND    NERVOUS    SYSTEM 

have  been  involved  along  with  the  facial  muscles.  Tn  one  case  ptosis 
was  observed.^^^  In  another  case^^^  paralysis  of  the  external  and 
internal  ocular  muscles  of  one  side,  with  facial  paralysis  of  the  oppo- 
site side,  was  seen.  Willard  makes  no  reference  to  this  complication, 
so  it  is  probable  that  it  has  existed  in  rare  instances  only.  V. 
Schrotter  ^^^  has  put  on  record  a  case  of  kopf  tetanus  in  which  there 
was  paralysis  of  the  facial,  the  ocular,  and  the  hypoglossal  nerves. 
Neumann  ^^^  says  that  the  facial  nerve  is  paralyzed  first ;  then  the 
oculomotor,  abducens,  trochlear  and  hypoglossal  are  affected  in  the 
order  named. 

The  pathology  of  this  affection,  as  already  said,  is  obscure.  Tetanus 
is  caused  by  a  bacillus,  and  this  is  no  doubt  so  also  in  the  cephalic  form. 
But  ordinary  tetanus  does  not  cause  paralysis;  quite  the  reverse.  We 
are  driven  to  the  conclusion,  entirely  speculative,  that  for  some  reason 
the  poison  of  tetanus,  especially  when  introduced  in  the  area  of  the  fifth 
nerve,  has  the  power  of  paralyzing  some  few  of  the  cranial  nerves, 
especially  those  supplying  the  face  and  the  orbit,  but  not  exclusively 
these,  for  the  hypoglossus  also  may  be  involved.  It  is  possible,  as 
has  been  suggested  to  the  minds  of  several  observers,  and  has  been 
voiced  by  Neumann,  that  the  poison  acts  upon  the  nuclei  of  the 
nerves  involved.  This  seems  to  be  shown  by  the  absence  of  the  post- 
mortem evidences  of  neuritis,  as  well  as  the  absence  of  the  reactions  of 
degeneration. 

TETANY. 

In  this  disease,  which  is  not  common  in  America,  eye  symptoms 
seem  to  be  rare.  Of  recent  European  wTiters  Filia,^^*  who  reports  44 
cases,  makes  no  mention  of  such  symptoms.  The  same  is  true  of 
Pons.^^**  Another  observer,  Loos,^^*  has  observed  nystagmus  in  a  child. 
The  fundus  oculi  is  usually  normal,  although  Miiller  ^^^  described  a 
case  with  neuro-retinitis  and  later  beginning  atrophy ;  but  the  case  was 
complicated  with  scarlet  fever.  V.  Jaksch  ^^*  had  a  case  with  double 
choked  disk  and  diplopia,  but  other  symptoms  pointed  to  an  organic 


'»  Serein,  L'Union  M^d.,  1886,  No.  173. 

'='  Roberts,  Lancet,  July  11,  1891. 

"Tall  von  Kopftetanus,  in  Wien.  klin.  Woch.,  1902,  No.  2,  p.  56. 

"*  Ein  Fall  von  Kopftetanus,  in  Zeit.  f.  Heilkunde,  1902,  p.  344. 

"♦La  Tetanie  chez  les  Enfants,  in  Rev.  d'Hyg.,  1903,  ii.  505-537. 

'^  Sur  Quelques  Points  de  la  Tetanie  Infantile,  Toulouse,  1903. 

'»  Deut.  Arch.  f.  klin.  Med.,  Bd.  1.  S.  169. 

'"  Deut.  Milit.  Arzt.  Zeits.,  1884,  xiii,  S.  439. 

"« Zeit.  f.  klin.  Med.,  1890,  xvii.  144. 


EPILEPSY  731 

brain  lesion.  Marschner  ^^^  describes  a  case  of  neuroretinitis  in  tetany, 
but  the  patient  had  also  pleuro-pnenmonia.  Cassel  ^^^  describes  a  case 
of  the  disease  with  optic  atrophy,  but  gives  no  other  details  regarding  it. 

Kursmal  ^^^  observed  a  case  in  which  the  pupils  were  unequal. 
There  is  sometimes  a  cramp  in  the  eye  muscles.  This  is  usually  a 
blepharospasm.  There  may  be  also  a  sudden  convergent  strabismus 
with  diplopia.  The  tetanic  spasm  of  the  eye  muscles  may  be  accompa- 
nied with  pain,  reddening  of  the  conjunctivae,  and  lachrymation.^^^ 
Cases  have  been  described  in  which  the  eye  symptoms  appeared  alone, 
or  alternating  with  the  other  symptoms.  The  pupil  is  seldom  dilated, 
and  even  more  rarely  contracted.^ ^^ 

These  few  references  to  the  disease,  by  recent  European  writers  who 
see  much  of  it  (and  much  more  than  is  seen  in  America)  seem  to  indi- 
cate that  the  ocular  symptoms,  although  not  lacking,  are  rather  rare. 
American  and  English  writers  (Osier,  Dana,  Gowei-s)  make  but  little 
if  any  reference  to  these  symptoms,  and  only  to  the  contractions  in  tlie 
eye  muscles. 

EPILEPSY. 

The  ocular  symptoms  of  epilepsy  are  important  from  a  diagnostic 
stand-point.  They  have  also  in  some  cases  not  a  little  medicolegal  sig- 
nificance. In  considering  them  it  is  necessary  to  remember  that  they 
are  usually  of  a  transient  character,  and  that  to  detect  them  requires 
alertness  and  some  skill  in  observation.     (See  Chapter  XVIII,  p.  782.) 

These  symptoms  may  be  divided  conveniently  into  groups.  Thus 
they  are  visual,  muscular,  and  vascular.  It  is  desirable  to  state  at  once 
that  it  is  not  the  object  of  this  paper  to  describe  epilepsy  itself,  but 
merely  its  ocular  symptoms.  Only  so  much  of  the  disease  will  be 
referred  to  as  is  necessary  for  an  understanding  of  these  symptoms. 
Epilepsy,  like  migraine,  is  an  explosive  neurosis,  preceded  in  the  vast 
majority  of  cases  by  an  aura.  Its  ocular  manifestations  are  sometimes 
a  part  or  even  the  whole  of  the  aura;  while  in  other  cases  the  involve- 
ment of  the  eye  is  in  the  nature  of  a  sequel.  In  the  majority  of  cases, 
however,  it  is  either  as  an  aura,  or  as  a  manifestation  of  the  early  stage 
of  the  fit,  that  the  eye  symptoms  are  prominent. 

The  purely  visual  symptoms  of  epilepsy  are  of  course  entirely  sub- 
jective, and  we  must  therefore  rely  entirely  on  the  patient's  own  state- 

>»  Deut.  Arch.  f.  klin.  Med.,  1896,  Bd.  Ivi.  501. 

^"  Bed.  klin.  Woch.,  1896,  69. 

"^  Deut.  Arch.  f.  klin.  Med.,  1869,  vi.  481. 

'"  Hochwart,  Tetanie,  in  Nothnagel's  Spec.  Path.  u.  Ther.,  Bd.  xi.  p.   134. 

»"Verein  f.  Psych,  in  Wein,  1895. 


732  THE    EYE    AND    NERVOUS    SYSTEM 

ments  for  a  knowledge  of  them.^^^  They  may  appear  as  an  aura  or  as 
a  sequel.  It  is  in  the  nature  of  the  aursc  of  epilepsy,  unlike  those  of 
migraine,  to  be  very  fleeting,  even  instantaneous ;  and  when  these  aurse 
are  visual  they  consist  usually  of  mere  momentary  affections  of  sight. 
Some  of  them  are  not  unlike  the  visual  aune  of  migraine ;  a  fact  which 
has  led  some  observers  to  see  a  more  intimate  relationship  between  these 
two  diseases  than  perhaps  the  facts  warrant. 

Mere  obscuration  of  vision,  something  like  the  amblyopia  of  mi- 
graine, is  an  epileptic  aura  in  some  cases.^^'  A  true  scintillating 
scotoma,  somewhat  like  that  seen  in  migraine,  is  claimed  by  Bens- 
wanger  ^^^  to  appear  sometimes  in  epilepsy,  but  this  is  rare;  and  it  is 
difficult  to  understand  how^  an  epileptic  patient  would  have  time  ordi- 
narily to  note  the  characteristics  of  such  a  sign.  Fere  '^^  also  refers  to 
obscuration  of  vision  as  a  symptom  of  epilepsy.  Some  patients  say 
that  everything  becomes  black  before  them  just  before  the  seizure ;  by 
this  they  probably  indicate  a  visual  aura,  or  amblyopia.  Ilerpin,*^* 
who  has  reviewed  the  history  of  epileptic  aurae  from  the  time  of  Hip- 
pocrates, gives  no  instance  of  a  true  visual  aura,  except  in  a  case  re- 
ported by  Maissonneuve,^^^  in  which  the  attack  began^with  a  dazzling 
sensation  (eblouissement)  in  the  right  eye,  which  continued  for  some 
minutes  after  the  return  of  consciousness.  The  original  idea  of  an 
epileptic  aura  was  that  it  was  caused  by  a  '"  vapor"  extending  up  the 
vessels  of  a  limb ;  and  indeed  the  word  "  aura"  is  derived  from  the 
Greek  word  meaning  "  a  vapor."  This  idea  was  so  firmly  fixed  that  it 
dominated  the  medical  world,  as  Hei^^jin  shows,  even  imtil  very  modern 
times,  and  to  it  may  be  ascribed  the  fact  that  the  older  observers  were 
not  on  the  lookout  for  any  kind  of  aura  except  the  sensory  warning 
beginning  at  an  extremity.  Hence  aura;  of  the  special  senses  were  not 
recognized  by  them,  because,  forsooth,  they  were  not  mentioned  by 
Galen  and  the  ancients.  But  in  recent  times  aura  of  the  special  senses 
have  been  fully  recognized,  and,  as  Gowers  ^^*^  remarks,  a  visual  warn- 
ing is  twice  as  common  as  all  the  other  special  sense  aurse  together. 
This  visual  aura  may  be  a  sudden  loss  of  sight,  or  it  may  be  a  flash  of 
colored  light  or  other  chromatic  appearance. 


"*  Spratling    (Epilepsy   and   its   Treatment,   p.   226)    gives  a   long  list   of  the 
visual  aurae  which  he  has  noted  in  his  patients. 
'"  Voisin,  Les  Epilepsies,   1896. 

'"  Die  Epilepsie,  in  Nothnagel's  Spec.  Path.  u.  Therap.,  Bd.  xii. 
"'  Les  Epilepsies,  Paris,  1890. 
"*  L'Epilepsie,  Paris,   1852,  p.  389. 
'"Cited  by  Herpin,  op.  cit.,  p.  411. 
»"Dis.  Nerv.  Syst.,  vol.  ii.  p.  739. 


EPILEPSY  733 

Flashes  of  light  or  balls  of  colored  or  uncolored  light  are  among 
the  commonest  visual  aurae  of  epilepsy.  They  are  variously  described 
by  patients,  and  doubtless  they  vary  in  different  cases.  There  may  be 
merely  a  glare,  a  flash,  or  a  dazzling  sensation  of  light,  and  this  may 
be  colorless  or  it  may  be  distinctly  chromatic.  Red  seems  to  be  the 
commonest  color.  In  some  cases  these  brilliant  aura?  take  distinct 
shapes,  especially  that  of  a  ball,  and  this  may  be  seen  approaching  or 
receding,  growing  larger  or  smaller.  This  tendency  to  increase  or 
diminish  in  size,  to  advance  or  recede,  is  sometimes  seen  in  other  objects, 
as  will  be  referred  to  presently.  Gowers,^^^  who  seems  to  have  had  the 
fortune  to  observe  more  complicated  cases  of  visual  aura)  than  most 
other  observers,  describes  instances  in  which  these  visual  warnings  were 
associated  with  other  aurae,  such  as  of  taste,  smell,  and  common  sensa- 
tion. He  also  calls  attention  to  the  fact  that  the  various  colors  of  the 
spectrum  are  not  seen  in  the  same  frequency.  Thus  they  appear  in 
the  following  order,  red,  blue,  green,  yellow,  and  purple,  which  is  not 
quite  the  physiological  order  in  which  the  colors  are  represented  in 
the  normal  visual  fields,  for  blue  occupies  the  larger  field  in  the  normal 
eye.  There  is  some  suggestion  here  of  what  occurs  in  hysteria — that 
is,  a  reversal  of  the  color-fields — ^red  occupying  the  most  prominent 
place  instead  of  blue.  Voisin  also  says  that  red  is  the  color  most  fre- 
quently seen  in  the  epileptic  aura. 

A  curious  form  of  epileptic  aura  is  that  in  which  objects  appear  to 
grow  larger  or  smaller,  as  the  case  may  be.  In  the  one  case  they  may 
appear  to  approach,  in  the  other  to  recede,  or  even  to  grow  thicker  or 
thinner.  These  phenomena  have  been  called  respectively  macropsia 
and  micropsia}^^  Patients  have  described  the  objects  in  a  room  as 
receding  to  a  great  distance.  In  other  cases  they  appear  to  approach 
and  enlarge  until  consciousness  is  lost  in  the  fit. 

Sometimes,  as  Eere  points  out,^^'^  there  is  a  photophobia  or  dysces- 
thesia  of  the  retina:  the  patient  cannot  bear  the  light,  and  there  is  a 
sense  of  pain  in  the  eye  with  a  forcible  reaction  of  some  of  the  ocular 
muscles,  especially  a  blepharospasm.  Fere  and  others  also  refer  to  a 
dyschromatopsia,  or  inability  to  distinguish  colors;  but  it  is  difficult 
to  understand  how  such  fine  tests  can  be  made  in  the  brief  period  of 
an  epileptic  aura.  Dyschromatopsia  and  changes  in  the  visual  field, 
such  as  contraction  and  reversal  of  the  color-fields,  may  persist  as  post- 


'"  Epilepsy  and  Other  Chronic  Convulsive  Diseases,  2d  Ed.,  p.  74  et  infra. 
""  Veraguth,  Ueber  Mikropsia  und  Makropsia,  Deut.  Zeit.  f.  Nervenheilk.,  Bd. 
xxiv..  H.  5,  6. 

•"  Op.  cit.,  p.  78. 


734  THE    EYE    AND    NERVOUS    SYSTEM 

epileptic  phenomena,  and  these  must  carefully  be  distinguished  from 
hysteria.  In  fact,  various  hysterical  stigmata  may  complicate  cases  of 
genuine  epilepsy,  as  the  present  writer  has  noted  in  the  Philadelphia 
Hospital  more  than  once. 

The  visual  aura  is  sometimes  located  by  the  patient  on  one  side,  as 
in  a  case  reported  by  Fere,^"*^  in  which  the  patient  saw  a  red  mist  before 
the  right  eye.  In  such  a  case  it  is  a  question  whether  the  phenomenon 
is  not  hemiopic, — that  is,  seen  in  the  corresponding  halves  of  both 
retiniv.  For  instance,  when  the  object  is  seen  apparently  only  by  the 
ri^t  eye,  it  is  really  perceived  by  the  left  half  of  the  retina  in  each 
eye.^^^ 

Spratling^*^  speaks  of  a  retinal  epilepsy,  in  which  there  is  sudden 
blindness  of  brief  duration  without  convulsion ;  in  other  words,  a  sort 
of  substitutional  attack.  The  term  "  epileptic  amaurosis,"  used  by 
Hughlings-Jackson,  has  also  been  used  by  some  writers  in  this  sense. 

Of  all  visual  aura?  the  most  complex  are  the  true  hallucinations,  in 
which,  as  it  were,  a  panorama  is  unfolded  before  the  patient's  gaze. 
A  few  such  cases  have  been  reported.  Gowers  ^^^  describes  minutely 
a  complicated  aura  in  which  the  patient  saw  an  old  woman  in  a  brown 
dress,  and  Gregory's  patient  saw^  an  old  woman,  too,  but  she  was  dressed 
in  red.^^^  These  visions  are  sometimes  of  disagreeable  or  ugly  objects 
or  animals ;  but  occasionally  the  scene  recalls  events  of  long  ago,  as  is 
sometimes  seen  in  migraine  also.  There  may  be  hemianopic  hallu- 
cinations.^^® 

The  muscular  affections  of  the  eye  in  epilepsy  may  be  observed  in 
either  the  extra-  or  intraocular  muscles  or  in  both.  It  is  not  unusual 
in  the  epileptic  fit  for  the  eyes  to  be  turned  forcibly  in  one  direction, 
as,  for  instance,  upward  or  to  one  side.  When  the  eyes  are  strongly 
rotated  to  one  side  it  is  usually  in  cases  in  which  there  is  forced  rotation 
of  the  head  also  to  one  side,  and  the  eyes  are  usually  rolled  towards  the 
same  side  as  the  head.  Onuf  ^^^  claims  that  there  are  exceptions  to 
this  rule,  and  that  the  eyes  may  be  rolled  to  the  opposite  side  from 
that  towards  which  the  head  is  rotated,  but  such  exceptions  must  be 
rare.     This  lateral  deviation  of  the  head  and  eyes  in  epilepsy  may  pos- 

'**0p.  cit.,  p.  464. 

'**  Harris  ( Hemianopsia,  in  Brain,  20,  307 )  refers  to  temporary  hemianoiMa 
in  epilepsy. 

"*  Epilepsy  and  its  Treatment. 
'"  Epilepsy,  etc.,  2d  Ed.,  p  .78. 
'"  Cited  by  Benswanger,  op.  cit. 
'"Harris,  op.  cit.     See  also  ante,  p.  11. 
'"New  York  State  Med.  .Tourn.,  1904,  iv.  71-74. 


EPILEPSY  735 

sibly  indicate  that  one  cerebral  hemisphere  is  more  involved  in  the 
epileptic  discharge  than  the  other,  and  it  is  the  opposite  of  what  is  seen 
in  gross  cerebral  hemorrhage  in  which  there  is  also  lateral  deviation 
of  the  head  and  eyes,  but  towards  the  lesion, — i.e.,  away  from  the  para- 
lyzed side.  In  epilepsy  the  deviation  is  usually  towards  the  more  con- 
vulsed side.^^^  This  is  showTi  in  cases  of  post-hem^plegic  seizures  in 
which  the  paralyzed  side  is  the  seat  of  the  most  active  convulsions. 
Lateral  deviation  of  the  eyes,  however,  is  not  a  localizing  symptom  of 
any  great  value  in  cerebral  pathology.  It  occurs  in  too  many  conditions 
to  be  of  special  significance. 

Diplopia  has  been  reported  by  some  writers  as  occurring  in  the  early 
stage  of  epilepsy.  Fere  and  Gowers  both  speak  of  it.  Strabismus  and 
nystagmus  have  also  been  noted,  both  as  accompaniments  of  the  attacks 
and  as  sequellae  of  them.  Diplopia  is  doubtless  caused  by  a  temporary 
strabismus  which  results  from  either  a  spasm  or  paresis  of  one  or  other 
ocular  muscle.  When  it  occurs  as  a  part  of  the  epileptic  aura  it  is 
probably  due  to  a  spasm  of  some  of  the  ocular  muscles  causing  the  two 
eyeballs  to  roll  in  different  axes.  Fere  ^^^  relates  a  case  in  which  an 
epileptic  boy  became  suddenly  cross-eyed  in  the  interval  between  fits; 
this  convergent  strabismus,  in  the  left  eye,  was  increased  after  each 
seizure,  at  which  time  the  patient  saw  double.  The  same  author  has 
noted  permanent  lateral  nystagmus,  also  permanent  blepharospasm  fol- 
lowing epileptic  seizures.  Such  permanent  affections  of  the  ocular  mus- 
cles point  to  organic  lesions,  whether  in  the  nature  of  an  accident  of 
the  fit  or  as  a  cause  of  the  fit,  such  as  a  tumor  or  a  basilar  meningitis. 
Speaking  of  nystagmus,  Fere  says  it  occurs  towards  the  affected  hemi- 
sphere in  hemiplegic  epilepsy  in  the  initial  stage. 

Much  has  been  written  about  the  state  of  the  pupils  during  the  epi- 
leptic fit.^^^  It  is  commonly  said  that  the  pupil  is  dilated  and  unre- 
sponsive to  light.  Onuf  says  that  there  is  extreme  contraction  of  the 
pupil  just  as  the  attack  begins,  and  that  it  is  also  contracted  in  the  post- 
convulsive stage.  Gowers  confirms  this  statement  with  respect  to  the 
initial  contraction,  but  says  that  dilatation  quickly  supervenes  and  is 
present  during  the  tonic  stage.  He  found,  however,  that  in  mild  attacks 
the  pupil  is  not  always  unresponsive  to  light.  Reynolds  ^^^  points  out 
that  at  the  end  of  the  fit  the  pupils  often  exhibit  marked  oscillations. 
These  oscillations  consist  of  alternate  contraction  and  dilatation,  but  are 


'^'  Jere,  op.  cit.,  p.  387. 
"''Op.  cit.,  129,  also  p.  177  et  infra. 
"^Fere,  op.  cit..  pp.  89,  178,  391,  417,473. 
'"Epilepsy,   1861,  p.   112. 


736  THE    EYE    AND    NERVOUS    SYSTEM 

not  present  in  every  case.  The  conjunctival  reflex  is  also  abolished  dur- 
ing the  profound  unconsciousness  of  epilepsy :  the  eye  may  be  touched 
or  irritated  with  the  finger  without  causing  the  usual  reflex  contraction 
of  the  orbicularis.  In  very  mild  cases  of  petit  mal  this  may  not  always 
be  so,  doubtless  because  consciousness  is  not  lost,  or  only  momentarily 

80.^" 

The  condition  of  the  pupil  has  been  appealed  to  in  some  cases  as  a 
medicolegal  test.  Evidently,  in  simulated  epilepsy  this  state  of  dila- 
tation could  not  be  feigned  by  an  act  of  the  will;  but,  as  Voisin  has 
pointed  out,  dilatation  can  be  secured  by  the  use  of  belladonna,  and 
deception  might  thus  be  practised.  Still,  the  action  of  belladonna  or 
its  alkaloid  atropia  is  very  persistent  on  the  pupil,  lasting  for  days, 
and  this  should  be  enough  to  expose  the  deception.^ ^^  The  dilatation 
in  epilepsy,  with  abolition  of  the  light-reflex,  does  not  endure  after  the 
fit.  But  in  a  suspected  case  the  dilatation  with  immobility  of  the  pupil 
during  the  fit,  speaks  for  epilepsy.^  ^'^  Fere  speaks  of  asymmetry  of  the 
pupil  in  the  epileptic  fit;  also  of  inequality  of  the  pupils.^^*  It  is 
important  to  bear  in  mind  that  we  are  here  discussing  essential  or  idio- 
patliic  epilepsy, — i.e.,  epilepsy  without  gross  organic  lesion.  In  cases 
of  symptomatic  epilepsy,  as,  for  instance,  in  cases  of  tumors,  hemor- 
rhage, or  meningitis,  the  state  of  the  pupils  might  be  affected  by  such 
lesion  more  or  less  permanently  and  independently  of  the  seizures. 

Vascular  lesions  in  the  eye  are  not  common  in  epilepsy.  Ecchy- 
moses  or  extravasations  of  blood  beneath  the  conjunctiva  are  occasion- 
ally seen  as  results  of  the  violence  of  the  fit.^''^  I  have  seen  such  a  case 
in  which  a  large  part  of  the  exposed  surface  of  the  eyeball  was  covered 
with  extravasated  blood.  It  is  not  a  symptom  of  special  importance, 
and  it  usually  disappears  in  a  few  days.  Congestion  of  the  conjunctiva 
may  be  present  during  the  seizure,  but  it  soon  subsides.     These  ocular 

"*Spratling  (Epilepsy  and  its  Treatment,  1904,  p.  268.)  does  not  recall  a 
single  instance  in  which  the  pupils  were  not  more  or  less  dilated — in  some  cases 
extremely  so.  He  does  not  believe  that  this  is  due  to  asphyxia,  as  some  have  held, 
because  he  has  seen  it  in  light  attacks  as  a  very  early  phenomenon.  He  thinks 
that  inequality  of  the  pupils  points  to  an  organic  lesion,  and  thus  it  may  persist 
during  the  intervals. 

'"  See  the  Trial  of  Allen  C.  Laros,  which  took  place  at  Easton,  Pa.,  in  August, 
1876  (reported  by  P'dgar),  for  an  example  of  alleged  feigned  epilepsy  in  which  the 
state  of  the  pupils  wa§  discussed. 

'"  Max  Bire,  Ueber  Epilepsie,  Deut.  Zeit.  f.  Nervenheilk.,  Bd.  xxiii.,  H.  1,  2, 
p.  30.  See  also  Hoche,  Die  differential  Diagnose  Zuischen  Epilepsie  und  Hys- 
teric, Berl.  Augen.  Huschweld,  1902. 

•»0p.  cit.,  391. 

""Voisin,  op.  cit. 


EPILEPSY  737 

lieniorrliages  are  sometimes  associated  with  hemorrhages  in  other  parts 
of  the  body.^^**  Retinal  hemorrhage  is  rare.  Gowers  thinks  it  is  pre- 
vented by  the  intra-ocular  tension.  The  appearance  of  the  fundus  is 
not  significant  in  ordinary  essential  epilepsy.  Between  attacks  the 
fundus  is  normal,  and  during  attacks  the  only  marked  change  is  in- 
creased fulness  of  the  retinal  veins ;  this  is  in  unison  with  the  general 
congestion  of  the  vessels  of  the  head.  When  traces  of  old  optic  neuritis 
are  found  in  so-called  idiopathic  epilepsy,  they  may  be  due  to  an  old 
syphilitic  lesion  wliicli  may  have  acted  as  the  original  cause  of  the  epi- 
lepsy, as  Gowers  has  pointed  out.  Epilepsy  itself  does  not  cause  per- 
manent changes  in  the  fundus  of  the  eye,  as  is  the  universal  verdict. 
It  is  a  rather  curious  fact  that,  as  Fere  points  out,  an  ophthalmoscopic 
examination  in  an  epileptic  will  sometimes  excite  a  seizure. 

In  convulsive  attacks  due  to  uraemia  and  to  chronic  lead-poisoning 
(encephalopathy)  there  may  be  well-known  changes  in  the  fundus  due 
to  those  conditions,  but  such  changes  are  in  nowise  either  the  causes 
or  results  of  the  fits. 

There  are  but  few  ocular  sequellce  of  epilepsy.  Mention  has  already 
been  made  of  ocular  palsies  which  may  persist  after  the  fit,  but  they 
are  rare.  In  genuine  psychical  epilepsy,  in  which  the  convulsion  is 
replaced  by  a  psychosis,  hallucinations  of  sight  may  occur ;  also  in  the 
post-paroxysmal  stage.^®^  These  hallucinations  are  usually  of  alarming 
objects,  as  ugly  faces,  devils,  etc.^^^  In  testing  the  visual  fields  after 
an  epileptic  attack,  it  is  important  to  remember,  as  already  said,  that 
such  symptoms  as  dyscromatopsia,  contraction  of  the  visual  fields,  re- 
versal of  the  color  fields,  etc.,  may  be  of  hysterical  character,  for  it  is 
not  rare  for  epilepsy  to  be  complicated  with  hysteria.  The  error  must 
not  be  made,  however,  of  supposing  that  because  a  patient  has  a  few 
hysterical  stigmata  after  a  fit,  therefore  his  attack  is  not  genuine  epi-' 
lepsy.  The  two  diseases  may  co-exist  in  one  and  the  same  patient.  So, 
too,  of  migraine.  The  fact  that  a  patient  has  visual  aurse  resembling 
those  of  migraine,  or  even  the  still  more  striking  fact  that  a  patient 
has  both  migrainous  and  epileptic  seizures,  is  no  reason  for  claiming 
that  those  two  diseases  are  so  intimately  related  as  is  done  by  some 
authors,  conspicuous  among  whom  are  both  Gowers  and  Fere.^®^ 

""  Spiller,  Uncinate  Group  of  Fits,  Am.  Med.,  1904,  vii.  474. 

"•  Sachmunde,  Ueber  vereinzelt  auftretende  Halluzinationen  bei  Epileptiken, 
Monat.  f.  Psych,  u.  Nerv.,  1904,  xv.  434. 

"^  Voisin,  op.  cit. 

'"H.  C.  Wood  (Medical  News,  1894,  p.  707)  has  reported  a  case  of  epilepsy  with 
migrainous  symptoms.  Such  a  form  might  appropriately  be  called  migrainoid  epi- 
lepsy.     Hughlings-Jackson    (Lancet,    1875,   vol.   ii.    p.   244)    spoke   of   migraine   as 

47 


738  THE    EYE    AND    NERVOUS    SYSTEM 


THE   BLINDNESS    DUE    TO    UREMIA    AND    OTHER 
BLOOD-STATES. 

Under  this  heading  will  be  described  those  attacks  of  blindness, 
usually  sudden  and  sometimes  transient,  which  are  observed  occasion- 
ally in  uraemic  patients,  also  in  anaemic  and  other  blood-states^  and 
likewise  those  due  to  embolus  of  the  retinal  artery.  This  chapter,  how- 
ever, does  not  include  in  its  scope  an  account  of  the  permanent  changes 
in  the  fundus  oculi  due  to  Bright's  disease  or  to  other  organic  diseases 
in  the  optic  nerves  and  brain. ^^* 

Uraemic  Blindness. — Sudden  blindness  sometimes  occurs  in  ursemic 
patients.  It  is  usually  seen  in  cases  of  kidney  disease  that  are  well 
advanced,  although  cases  have  been  reported  in  which  the  amaurosis 
was  an  early  symptom.  But,  whether  early  or  late,  the  amaurosis  is 
usually  a  sign  that  the  renal  disease  is  severe,  and  the  inference  is  that 
the  system  is  profoundly  poisoned.  The  characteristics  of  this  blind- 
ness are  its  sudden  onset,  its  completeness,  the  preservation  of  the  reac- 
tions of  the  pupil,  the  sudden  disappearance  of  the  blindness  when  the 
blood-state  is  relieved  by  active  purgation  or  sweating,  and  the  absence 
of  ophthalmoscopic  changes,  excepting  such  as  may  previously  have 
existed.^  °^ 

The  other  symptoms  associated  with  this  uremic  blindness  may 
or  may  not  be  marked.  In  many  cases  there  are  no  prominent  brain 
symptoms  of  uraemia,  no  coma,  no  convulsions,  or  any  special  signs  of 
uraemic  encephalopathy.  In  other  cases  the  visual  phenomena  precede 
a  more  active  and  extensive  attack  of  ura^mic-poisoniiig.  There  may 
be  some  headache  and  mental  confusion,  which  pass  away  with  the 
blindness  under  active  treatment,  or  the  case  may  advance  to  convul- 
sions and  coma.  Most  of  the  cases  put  on  record  have  been  those  in 
which  the  blindness  was  the  prominent  or  only  symptom.  It  is  proba- 
ble that  some  affection  of  vision  precedes  most  attacks  of  uraemic  con- 
vulsions and  coma,  but  is  overlooked  in  the  gravity  of  the  succeeding 
symptoms. 

There  has  been  much  speculation  about  the  causes  of  the  sudden 
and  transient  blindness  of  uraemia.  Most  observers  are  agreed  that 
there  are  no  ophthalmoscopic  changes  to  account  for  it,  or  that  such 

"  sensory  epilepsy,"  and  he  had  a  rather  fanciful  theory  that  it  is  due  to  a  "  dis- 
charging lesion  of  the  occipital  lobe."  There  is  no  scientific  warrant  for  such  a 
pathology  or  such  a  terminology. 

'"  Antonalli,  L'Amblyopie  Transitoire,  in  Arch,  de  Neurolog.,  1892,  pp.  202,  423. 

"*  Gowers,  Manual  of  Medical  Ophthalmoscopy,  1904. 


URzEMIC  BLINDNESS  739 

changes,  if  present,  are  slight.  Gowers  says  that  slight,  transient  oedema 
of  the  papilla  has  been  noted  to  coincide  with  the  symptom  and  to 
pass  away  with  it.  This  is  also  noted  by  Orters.^^®  In  some  cases, 
however,  even  this  oedema  of  the  nerve-head  has  not  been  seen.^®^  Con- 
sidering the  frequency  of  retinal  changes  in  chronic  Bright's  disease, 
it  is  rather  remarkable  that  no  alterations  are  reported  in  so  many 
cases  of  sudden  blindness.  Gowers  does,  indeed,  say  that  permanent 
retinal  changes  are  present  in  some  cases,  but  that  these,  while,  of 
course,  they  persist  after  the  attack,  are  not  changed  by  it.  It  might 
well  be  difficult  to  detect  transient  changes  under  such  circumstances. 

It  is  natural  to  fall  back  upon  the  theory  of  an  acute  poisoning  of 
the  nerve  fibres,  without  visible  organic  changes.  This  is  a  reasonable 
theory,  in  accord  with  the  condition  of  the  patient,  and  in  accord  also 
with  what  we  know  about  acute  poisoning  of  nervous  tissue.  A  toxic 
substance  in  the  blood  might  well  affect  the  nutrition  and  function  of 
the  optic  nerve  and  retina  without  giving  signs  that  could  always  be 
recognized  with  the  ophthalmoscope.  Thus  the  blindness  might  be  due 
to  the  overwhelming  of  the  nerve  with  the  ura?mic  poison.  ^^®  This  is 
the  chemical  theory,^  ®^  according  to  which  deleterious  substances  are 
circulating  in  the  blood,  though  what  the  exact  chemical  composition 
of  them  is  remains  unknown. 

Another  theory  is  the  mechanical  one,  according  to  which  the  blind- 
ness is  due  to  an  oedema  of  the  brain  and  of  the  optic  nerve  and 
retina.^ ^^  We  have  already  referred  to  the  fact  that  oedema  of  the 
nerve-head  has  been  noted  by  various  observers.  It  has  been  argued 
by  Hauenschild  that  oedema  of  the  nerve-head  alone  could  not  cause 
the  symptoms,  for  the  reason  that  the  pupillary  reflexes  are  preserved; 
therefore,  the  lesion  must  be  located  back  of  the  primary  optic  ganglia, 
possibly  in  the  brain  cortex.  This  is  an  ingenious  argument,  and  not 
easily  answered.  Certainly,  if  the  pupils  react  to  light,  the  retinal 
cells  must  be  capable  of  taking  impressions  and  sending  them  on  to  the 
ganglia;  in  other  words,  the  functions  of  the  optic  nerves  are  not 
entirely  gone,  as  the  total  blindness  would  seem  to  indicate. 

This  mechanical  theory,  or  theory  of  pressure  due  to  oedema,  is 
much  the  same  as  the  vasomotor  theory,  which  here,  as  elsewhere,  has 
played  its  part.  This  is,  however,  a  most  unsatisfactory  theory  by 
which  to  account  for  anything  in  pathology,  for  although  the  vasomotors 

^•^  Dobrowolsky,  Klin.  Monatsbl.  f.  Augenheilk.,  March,  1881,  p.  121. 
'"  Hauenschild,  Muench.  med.  Woch.,  1903,  No.  4. 
'"  Benschoten,  Providence  Med.  Journ.,  July,  1903. 
lee  Frerichs,  Arch.  f.  Physik.  Heilk.,  x.  34. 
""  Traube,  Schmidt's  Jahrbuch.,  cxiv,  p.  308. 


740  THE    EYE    AND    JsTERVOUS    SYSTEM 

are  undoubtedly  a  means  by  which  disease  processes  act,  they  never- 
theless must  require  some  cause  to  set  them  in  action.  It  is  this  cause, 
whatever  it  may  be,  acting  upon  the  vasomotors,  that  is  the  true  cause 
of  any  disease  process,  not  the  vasomotors  themselves.  A  poison  can 
conceivably  affect  the  vasomotors,  as  can  also  mechanical  irritation  or 
a  gross  lesion,  but  nothing  is  explained  by  merely  appealing  to  the  state 
of  the  vasomotors  so  far  as  essential  causation  is  concerned.  Pal,^'^^ 
discussing  the  pathogenesis  of  acute  transitory  amaurosis  of  uraemia, 
eclampsia,  etc.,  says  it  is  due  to  a  transitory  anaemia  of  the  brain  due 
to  vasomotor  change,  cardiac  insujfficiency,  etc.  This  seems  like  beg- 
ging the  question.  Certainly,  cardiac  insufficiency  is  not  present  in 
most  of  the  cases  of  puerperal  eclampsia,  in  which,  just  as  in  uraemia, 
some  of  these  affections  of  sight  are  observed.  Benschoten  suggests 
that  the  blindness  may  be  due  to  a  spasm  of  the  retinal  vessels.  Bull  ^^^ 
suggests  that  arteriosclerosis  of  the  ophthalmic  and  internal  carotid 
arteries  may  be  the  underlying  cause  of  some  cases  of  obscure  blind- 
ness, especially  shown  by  a  paracentral  scotoma. 

In  ura^mic  amaurosis  there  is  usually  an  obscuring  of  the  whole  field, 
and  equally  in  both  eyes.  But  Pick'^^  reports  a  case  in  which  there 
was  an  homonymous  hemianopsia  of  uraemic  origin.^ ^* 

Ura^mic  blindness  has  been  observed  in  various  forms  of  kidney 
disease.  In  Ilauenschild's  case  there  was  a  parenchymatous  nephritis. 
The  condition  may  occur  in  contracted  kidney  also.  Roscher  ^"^^  reported 
a  case  in  which  there  was  a  suddenly  developed  hemorrhagic  nephritis ; 
two  days  later  uraemic  symptoms  appeared,  with  amaurosis.  In  this 
case  there  were  normal  eye  grounds,  but  tlie  pupils  were  dilated  and 
would  not  react,  contrary  to  what  is  usually  reported.  This  case  was 
rapidly  fatal,  and  Roscher  believed  that  the  immobility  of  the  pupils 
suggested  a  bad  prognosis,  because  it  indicated  the  severity  of  the 
process. 

In  puerperal  eclampsia  there  are  eye  symptoms  exactly  like  those 
seen  in  uraemia.  Disturbance  of  vision  is  observed  in  many  cases; 
there  may  be  amblyopia  or  amaurosis.     The  late  Professor  Parvin  ^"^^ 

'"Centblt.  f.  inn.  Med.,  1903,  No.   17. 

"»Ann.  Ophthal.,  1904,  xiii.  74. 

"'  Ueber  Hemianopie  bei  Uraemie,  Munch,  med.  Woch.,  1903,  p.  2125.  Thi8 
condition  was  first  described  by  Pick  in  1896.  A  few  cases  have  been  described 
by  others. 

"*  See  also  Gill,  Transient  Recurrent  Attacks  of  Lateral  Hemianopsia,  in 
Brit.  Med.  Journ.,  Feb.  1,  1890,  p.  233. 

'"  Muench.  med  Woch.,  1903,  No.  9,  p.  382. 

""  Eclampsia,  in  Hirst's  Am.  Syst.  of  Obstetrics,  vol.  ii.  p.  73. 


UR^^MIC  BLI:N^DNESS  741 

referred  to  a  fatal  case  of  eclampsia  occurring  at  the  fourth  month  of 
pregnancy  in  which  there  was  almost  total  blindness  for  two  days  before 
the  seizure.  Dewees  ^""  many  years  ago  said  that  a  temporary  loss  of 
vision  was  among  the  premonitory  symptoms  of  puerperal  convulsions. 
Of  recent  years  this  symptom  has  been  so  generally  recognized  as  not 
to  require  us  to  quote  many  authorities.  Xeither  is  this  the  place  to 
enter  upon  a  discussion  of  the  question  w^hether  eclampsia  is  identical 
with  uraemic  convulsions.^^®  Certainly  it  resembles  the  uraemic  fit  in 
many  of  its  symptoms,  among  which  is  amaurosis. 

Anaemia  from  hemorrhage  and  other  causes  may  produce  sudden 
blindness.  This  blindness  may  be  trifling  or  complete,  transient  or 
permanent,  immediate  or  postponed,  and  more  frequent  from  so-called 
spontaneous  than  from  traumatic  hemorrhage.  The  pupils  are  usually 
dilated  and  immobile  to  light.  It  has  been  observed  after  hemorrhage 
from  the  bowels,  uterus,  nose,  as  well  as  after  venesection  and  trauma. 
The  visual  disturbance  may  be  in  the  nature  of  a  scotoma,  or  hemian- 
opsia, or  contraction  of  the  fields,  or  the  blindness  may  be  in  sectors 
or  irregular.-^ '^  There  may  be  changes  in  the  fundus,  or  this  may  be 
normal.  Benschoten  says  that  the  prognosis  is  good  if  the  blindness 
is  immediate,  but  unfavorable  if  it  is  delayed  several  days,  because  in 
the  latter  case  it  may  be  due  to  neuritis  or  atrophy  of  the  optic  nerve. 
He  believes  that  the  prognosis  is  worst  in  cases  of  hemorrhage  from 
the  stomach.  Assicot  ^^^  reports  two  cases  of  permanent  blindness  due 
to  hemorrhage  from  the  uterus.  In  transitory  blindness  due  to  anaemia 
Loewe  ^®*  supposed  that  there  was  a  spasm  of  the  blood-vessels  supplying 
the  cuneus,  a  theory  to  be  taken  for  what  it  is  worth.  Transient  loss 
of  sight  occurs  also  in  syncopal  attacks. 

In  diabetes  there  are  sometimes  observed  defects  of  sight  which  are 
similar  to  uraemic  amaurosis.^®^  Lecorche  called  attention  to  the  grave 
significance  of  diabetic  amblyopia  in  some  cases,  in  which  it  may  be 
a  terminal  symptom,  or  at  least  herald  the  end.^®^  It  should  not  be 
forgotten  that  cataract  occurs  in  some  cases  of  diabetes. 

'"Midwifery,  3d  Ed.,  Philada.,  1828,  p.  451. 

"'  Winckel,  Obstetrics,  Am.  Trans.,  p.  587  et  infra. 

^'°  Gowers,  op.  cit. 

""  Pathogenie  des  Amauroses  Posthemorrhagiques,  in  Arch,  de  Ophth.,  May, 
1902. 

'*'  Ein  Fall  von  Transitorischer  Blei  Amaurose,  in  Arch.  f.  Augenheilk.,  xlviii. 
p.  3.32. 

^*^  Gowers,  op.  cit.  See  also  Heine,  Deutsch.  med.  Woch.,  1903,  p.  348;  and 
Brunton,  in  Reynolds's  Syst.  of  Med.,  voh  iii.  p.  591. 

'*^  Traite  du  Diab&te,  1876.  Also  Laveran  et  Teissier,  Pathologic  M6dicale, 
3d  ed.  vol.  i.  p.  314. 


742  THE    EYE    AND    NERVOUS    SYSTEM 

Jacquean  has  even  written  of  amblyopia  of  hepatic  origin.^®*  Am- 
blyopia from  tobacco  and  alcohol  is  usually  due  to  a  post-orbital  optic 
neuritis,  and  is  not  to  be  described  in  this  chapter.^ ^^ 

Finally,  brief  reference  may  be  made  here  to  embolus  of  the  retinal 
artery  as  a  cause  of  sudden  blindness.  This  blindness  may  be  per- 
manent or  it  may  tend  to  improve.  The  causes  are  such  as  give  rise 
to  embolism  in  other  arteries,  especially  diseases  of  the  endocardium; 
and  there  may  be  also  arterial  sclerosis  with  temporary  stoppage  of  the 
circulation.  Emboli  and  thrombi  may  occur  in  these  cases  in  other 
arteries  in  the  brain.^^^  In  a  case  observed  by  the  present  writer  in 
an  old  lady  the  blindness  was  sudden,  complete,  and  permanent,  and 
the  embolus  was  doubtless  derived  from  the  heart,  which  was  diseased. 
The  ophthalmoscopic  changes  are  usually  well  marked,  and  consist  in 
a  diminution  in  the  size  of  the  artery  and,  in  time,  atrophy  of  the  disk 
and  retina.*®^ 


'"Bull,  et  M6m.  Soc.  Franc.  d'Ophthal.,  1902. 

'"  De  Schweinitz  and  Edsall,  Concerning  a  Possible  Etiological  Factor  in 
Tobacco  and  Alcohol  Amblyopia,  etc.,  in  Am.  Journ.  Med.  Sci.,  1903,  p.  216. 

•"Posey  (Transient  Monocular  Blindness,  in  Journ.  Am.  Med.  Asso.,  May 
31,  1902)  has  described  cases  in  which  the  blindness  was  evidently  due  to  vascular 
defects  other  than  embolic,  as  the  loss  of  sight  was  only  temporary.  In  some  cases 
there  was  actual  sclerosis. 

'"  Norris,  Medical  Ophthalmology,  in  Pepper's  Syst.  of  Med.,  vol.  iv.  pp.  740, 
741.     See  also,  Van  Duyse,  Arch.  d'Ophth.,  1902,  p.  93. 


CHAPTER    XVIII. 

NEUEOSES  AND  OTHEE  CONDITIOI^S  OCCASIONED  BY 
EEEOES  OF  EEFEACTION  AND  IMBALANCE  OF  THE 
EXTEA-OCULAE  MUSCLES,  AND  THE  EFFECT  OF  LENSES 
AND  OPEEATIVE  PEOCEDUEE  IN  EELIEVING  THE  SAME. 

Part  I. 
By  S.  D.  RISLEY,  M.D. 
HEADACHE;    NEURALGIA. 
Th-e  design  in  the  present  chapter  is  to  treat  primarily  of  headache 
and  its  relation  to  eye-strain ;   but  it  will  not  be  found  possible  to  avoid 
a  more  or  less  extended  consideration  of  a  group  of  associated  phenom- 
ena composing  the  symptom  complex  known  as  "  asthenopia." 
In  the  discussion  of  the  subject  it  will  be  maintained — 

(1)  That  in  a  large  number  of  patients  anomalies  of  the  ocular 
apparatus  are  the  sole  and  sufficient  cause  of  headache. 

(2)  That  these  anomalies  of  vision  may  be  the  unsuspected  cause 
and,  therefore,  that  the  absence  of  symptoms  obviously  referable  to  the 
eyes  does  not  exclude  them  as  an  etiologic  factor  in  headache  and  certain 
associated  symptoms, — e.g.,  insomnia,  vertigo,  petit  chorea  in  children, 
stomach  derangements,  and  impaired  general  nutrition. 

(3)  That  the  congenital  origin  of  these  ocular  anomalies  does  not 
exclude  them  from  participation  as  a  factor  in  the  sudden,  or  recent, 
development  of  symptoms  coming  on  during,  or  after,  middle  life,  after 
attacks  of  acute  disease,  or  more  or  less  acute  exacerbations  of  some 
general  dyscrasia. 

(4)  That,  therefore,  the  participation  of  the  eyes  as  a  probable  etio- 
logic factor  in  headache  and  other  neuroses  can  be  positively  excluded 
only  in  the  absence  of  ocular  disease,  or  after  the  most  painstaking  cor- 
rection of  any  existing  error  of  refraction  or  abnormality  of  binocular 
balance. 

(5)  That  in  many  cases  the  prolonged  eye-strain,  consequent  upon 
existing  anomalies  of  vision,  sets  up  pathologic  conditions  of  the  fundus 
oculi,  and  that  these  require  time  for  recovery,  therefore,  immediate 
relief  of  symptoms  is  not  always  to  be  expected. 

The  facts  set  forth  briefly  in  the  preceding  propositions  may  be 
claimed  as  among  the  most  signal  achievements  of  modern  ophthalmol- 

743 


744  THE    EYE    AND    NERVOUS    SYSTEM 

ogy.  While  it  is  true  that  a  number  of  early  observers  had  recognized 
a  more  or  less  definite  association  between  weak  eyes  and  pain  in  the 
brow,  they  failed  to  grasp  the  true  relationship  of  the  symptoms. 
Indeed,  any  historical  study  of  the  early  literature  of  headache,  or  even 
of  much  that  has  been  written  by  later  authors,  is  disappointing  when 
viewed  from  the  standpoint  of  the  ophthalmologist.  In  the  general  or 
specific  discussion  of  this  symptom  and  its  etiology  by  some  authors, 
the  eyes  as  a  probable  factor  are  noticed  in  such  a  casual  manner  that 
it  is  obvious  no  adequate  comprehension  of  the  importance  of  eye-strain 
had  been  reached.  To  the  ophthalmic  surgeon  this  practical  neglect 
of  ocular  affections  as  a  cause  of  headache  and  a  considerable  group  of 
associated  nervous  symptoms  is  unaccountable,  since  in  the  daily  rou- 
tine of  his  work  he  encounters  it  in  so  large  a  percentage  of  his  eye- 
strain patients.  In  1899,  the  writer  made  a  cursory  analysis  of  one 
thousand  consecutive  eye  patients,  and  found  that  upward  of  50  per 
cent,  of  them  complained  of  headache,  and  that  many  of  them  had  been 
sent  by  their  physicians  in  the  hope  that  the  pain  in  the  head,  which 
had  proved  rebellious  to  general  treatment,  might  be  due  to  some  ocular 
defect.  A  colleague,  Dr.  M.  W.  Zimmerman,  in  an  elaborate  study 
of  two  thousand  patients,  drawn  from  private  and  hospital  cases,  found 
that  71.3  per  cent,  of  them  suffered  from  headache.  In  ophthalmic 
practice,  therefore,  the  subject  assumes  a  place  of  commanding 
importance. 

Until  within  the  memory  of  men  now  living,  and  actively  engaged  in 
professional  work,  ophthalmic  literature  was  no  less  at  fault.  We 
are,  nevertheless,  indebted  to  many  of  the  early  writers  for  painstaking 
and  accurate  descriptions  of  the  symptom  complex  we  now  know  as 
asthenopia.  The  weak  eyes,  which  baffled  all  their  efforts  for  relief, 
were  associated  by  them  with  brow  pain,  and  were  carefully  described 
by  Taylor  in  1776;  by  Plenck  in  1792;  by  Scarpa  in  1816,  and  by 
Beer  in  1817  (Donders).  These  observers  were  followed,  prior  to  1850, 
by  Lawrence,  Tyrrel,  Sichel,  MacKenzie,  and  others.  The  variety  of 
opinions  expressed  by  these  eminent  men  and  the  many  designations 
devised  and  employed  as  either  descriptive  of  the  observed  conditions 
or  explanatory  of  their  supposed  nature  or  cause  convict  them  of  error. 
This  is  not  surprising  since  it  was  not  possible  to  form  any  adequate 
conception  of  the  essential  nature  of  the  morbid  conditions  they  observed 
until  after  the  discovery  of  the  hypermetropic  eye,  and  the  publication, 
in  1858,  of  the  justly  renowned  physiologic  researches  of  Donders  of 
Utrecht  in  his  classic  treatise  on  the  ''Anomalies  of  Refraction  and 
Accommodation."     This  remarkable  book  shed  a  flood  of  light  upon 


NEUROSES  OCCASIONED  BY  EYE-STRAIN         745 

many  ocular  problems,  and  laid  the  foundation  for  a  most  important 
part  of  the  beautiful  structure  which  modern  ophthalmology  has  reared. 

This  great  master  does  not,  however,  seem  to  have  comprehended  the 
full  clinical  significance  of  the  truths  he  had  discovered  and  established. 
The  ulterior  results  of  the  strain  upon  the  accommodative  function  of 
the  eye  in  overcoming  the  anomalies  of  refraction,  and  of  the  struggle 
required  to  maintain  binocular  single  vision  in  the  presence  of  some 
abnormality  of  the  extraocular  muscular  apparatus,  have  for  the  most 
part  been  disclosed  by  his  followers.  The  credit  for  recognizing  the  great 
significance  of  eye-strain  in  producing  both  the  pathologic  states  of  the 
fundus  oculi,  and  an  associated  group  of  general  reflex  symptoms,  often 
simulating  organic  disease,  is  largely  due  to  American  observers.  Dr. 
William  Thomson,  in  1879,^  in  a  paper  on  "Astigmatism  as  a  Cause 
for  Persistent  Headache  and  Other  Xervous  Symptoms,"  states  that 
more  than  ten  years  before  he  became  aware  that  persons  who  con- 
sulted him  for  defective  sight  presented  symptoms  which  he  enumerated 
as  pain  in  the  brow,  temples,  and  occiput,  a  sense  of  fulness  in  the  head 
amounting  to  vertigo  and  nausea,  insomnia,  loss  of  appetite,  fear  of 
impending  apoplexy  or  epilepsy,  general  nervous  prostration,  choreic 
twitching  of  the  muscles  of  the  head  and  face,  all  of  which  he  had 
seen  relieved  by  correcting  their  astigmatic  eyes.  Other  ophthalmic 
surgeons  had  also  witnessed  the  marked  relief  of  headache,  insomnia, 
and  other  reflex  symptoms,  following  the  correction  of  optical  defects 
by  glasses. 

Dr.  S.  Weir  Mitchell's  attention  was  called  to  these  cases,  and  to 
him  is  due  the  credit  of  impressing  upon  the  general  profession,  in  two 
forcible  articles,^  the  grave  significance  of  eye-strain.  Ever  since  atten- 
tion was  thus  fixed  upon  the  subject,  in  Philadelphia  at  least,  no  study 
of  a  rebellious  case  of  headache  or  of  obscure  nervous  disease  has  ever 
been  considered  complete  until  a  careful  examination  of  the  eyes  had 
excluded  them  as  a  possible  cause  of  the  disturbance. 

It  does  not  fall  within  the  scope  of  this  chapter  to  consider  in  detail 
the  nature  and  variety  of  the  ocular  anomalies  which  may  cause  an 
undue  strain  or  tension  upon  the  visual  organs,  but  it  is  important  to 
have  a  definite  conception  of  the  normal  or  essential  requirements  for 
comfortable  binocular  vision.  That  is  to  say,  a  physiologic  standard 
to  which  anomalous  states  may  be  referred  for  comparison. 

This  standard  will  be  found  in  two  healthy  emmetropic  eyes  with 


^The  Medical  News  and  Library. 

"  Medical    and    Surgical    Reporter,    1874 ;     American    Journal    of    the   Medical 
Sciences.  1876. 


746  THE    EYE    AND    NERVOUS    SYSTEM 

normal  sharpness  of  vision,  a  physiologic  range  of  accommodation  and 
a  proper  binocular  balance;  that  is  to  say,  emmetropia,  or  the  ability 
to  focus  parallel  rays  of  light  on  the  fovea  without  effort  of  the  accom- 
modation, and  with  Y=6/vi  for  each  eye ;  a  range  of  accommodation 
corresponding  to  the  age  of  the  individual,  and  the  power  to  converge 
both  eyes,  equally  and  normally  to  the  physiologic  near-point,  on  the 
same  horizontal  plane.  When  these  conditions  obtain,  a  clear  image 
of  the  approaching  object  is  maintained  upon  the  fovea  of  each  eye 
from  infinity  to  approximately  the  binocular  near-point,  without  con- 
scious effort.  In  point  of  fact  this  ideal  of  requirement  is  compara- 
tively rarely  observed;  nevertheless  the  eyes  which  most  nearly 
approach  it  enjoy  the  highest  degree  of  comfort  and  meet  the  visual 
requirements  of  civilized  life  with  a  minimum  of  pain  and  peril. 
Indeed,  under  these  harmonious  relations  it  is  extremely  rare  to  find  any 
ocular  disturbance,  even  under  the  exacting  demands  of  modern  life. 

Disturbance  of  function,  therefore,  in  the  class  of  patients  under 
discussion  is  ordinarily  produced  by  some  departure  from  this  standard. 

Any  thoughtful  analysis  of  the  standard  as  defined  in  the  preceding 
paragraph  will  detect  its  great  complexity.  To  secure  emmetropia,  two 
model  eyes  are  required.  To  enjoy  comfortable  binocular  single  vision 
the  complex  system  of  extraocular  muscles  must  be  normally  developed, 
have  a  normal  innervation  and  a  normal  origin  at  the  apex  of  a  model 
bony  orbit,  and  a  proper  attachment  to  the  globe  of  each  eye.  The 
accommodative  function  of  each  eye  must  be  equal  and  normal,  and 
must  act  harmoniously  under  the  same  innervation  impulse  which  con- 
trols the  convergence  of  the  two  eyes  to  the  finite  point  under  observa- 
tion. 

That  the  harmony  of  all  parts  of  this  intricate  apparatus  is  liable 
to  frequent  disturbance  should  not  cause  surprise.  Putting  aside  the 
liability  to  impaired  function  from  disease  of  one  or  more  of  its  parts, 
which  does  not  fall  under  discussion  in  this  chapter,  it  remains  to  con- 
sider the  fact  that  the  various  elements  entering  into  this  complex 
visual  apparatus  are  subject  to  anatomical  variations, — e.g.^  anomalies 
in  the  form  of  the  eyeball,  or  anomalous  distribution  in  the  system  of 
extra-ocular  muscles,  or  less  gross  defects  of  development  in  the  muscles 
or  their  nerve  supply,  any  one  or  all  of  which  would  of  necessity  cause 
impairment  of  binocular  vision  or  cause  undue  strain  for  its  main- 
tenance. 

That  such  anomalous  conditions  are  liable  to  befall  the  eyes  is  sug- 
gested by  the  great  frequency  of  deformity  in  other  portions  of  the 
body.     That  the  eye  is  peculiarly  liable  would  seem  to  be  indicated  by 


NEUROSES  OCCASIOXED  BY  EYE-STRAIN^ 


747 


the  frequent  distortions  of  the  human  skull,  as  shown  by  any  cursory 
study  of  a  hatter's  forms,  many  of  which  are  so  ill  shaped  as  to  appear 
grotesque  when  compared  with  the  diameters  of  a  model  skull.*  One 
need  not  go  far  to  verify  the  fact  that  these  deformities  of  the  skull 
cause,  in  many  persons,  variations  in  the  size  and  form  of  the  bony 
orbits,  and  this  leads  to  the  more  or  less  serious  deformity  of  the  devel- 
oping eyeballs,  which  the  orbits  are  designed  to  inclose  and  protect. 


Fig.  1. — Diagrams  showing  types  of  conformation  of  skulls  in  subjects  whose  eyes  were  highly  astigmatic, 
with  asymmetrical  meridians,  associated  with  binocular  imbalance. 

It  is  not  unreasonable  to  expect  that,  during  development  in  an  anoma- 
lously shaped  orbit,  not  only  certain  modifications  will  occur  in  the 
shape  of  the  eyeball  itself,  which  would  change  its  diameters  and  alter 
the  radii  of  curvature  of  the  cornea,  but  would  lead  also  to  changes  in 
the  length  and  line  of  direction  of  the  optic  nerves,  and  the  origin  and 
attachments,  the  length  and  direction  of  the  ocular  muscles, — condi- 
tions one  or  all  of  which  would  lead  to  more  or  less  marked  disturbance 
of  the  refraction  and  motility  of  the  eye,  and  in  many  complex  ways 
modify  the  conditions  of  binocular  vision  rendering  it  either  difficult 
or  impossible.     (Compare  Chapter  XIV  and  XXIII.) 


*"  Abnormalities  of  Ocular  Balance,"  by  S.   D.   Risley,  M.D.,   University  Med. 
Mag.,  Jan.   1895. 


748  THE    EYE    AND    NERVOUS    SYSTEM 

When  we  add  to  these  congenital  defects  of  structure  and  relation 
the  ravages  of  disease,  either  affecting  the  eye  itself,  or  its  essential 
anatomical  connections,  it  is  obvious  that  in  tlie  elucidation  of  the  anom- 
alies of  binocular  vision  we  have  placed  before  us  many  obscure  and 
complex  problems  which  lie  at  the  foundation  of  eye-strain  and  its 
attending  group  of  symptoms. 

These  theoretical  considerations  find  ample  demonstration  in  the 
daily  routine  work  of  the  ophthalmic  surgeon  and  also  in  the  extensive 
statistics  collated  in  the  schools.*  In  these  statistical  studies  it  has  been 
shown  that  the  standard  conditions  for  comfortable  vision  are  compara- 
tively rare;  hypermetropia,  astigmatism,  or  myopia  being  present  in 
varying  degrees  in  the  large  majority  of  children's  eyes;  the  baneful 
significance  of  these  defects  being  shown  by  the  associated  asthenopia, 
that  is  to  say,  weak  eyes,  impaired  function,  inflammation  of  the  margin 
of  the  lids,  headache,  and  a  wide  complex  of  symptoms  affecting  the 
general  health  and  rendering  school  life,  for  many  children,  both 
burdensome  and  perilous. 

The  great  conservatism  so  often  displayed  by  nature  in  adapting 
the  means  at  hand  to  the  required  end  doubtless  frequently  overcomes 
in  great  measure  the  baneful  influence  which  these  anomalous  condi- 
tions would  otherwise  exert  over  binocular  vision.  For  example,  the 
growing  child  under  the  demands  of  a  steadily  increasing  experience 
gradually  moulds,  during  the  plastic  years  of  growth,  the  existing  con- 
ditions to  meet  more  or  less  successfully  the  felt  requirements  for  accu- 
rate vision,  and  victory  is  won  in  behalf  of  single  binocular  vision 
where  defeat  would  have  seemed  inevitable.  Unfortunately  when  exist- 
ing defects  are  great,  the  final  outcome  is  frequently  a  failure,  or  at  best 
but  partially  successful,  and  the  child  passes  into  adolescence  or  adult 
life  with  defective  sight,  and  a  heritage  which,  if  unrelieved,  must  be  a 
handicap  to  any  ambitious  career.  It  is  the  resulting  conditions  which 
are  before  us  for  study,  since  they  furnish  one  of  the  most  prolific 
causes  of  headache. 

The  errors  of  refraction  as  found  in  practice  present  a  great  variety 
of  conditions,  but  group  themselves  into  certain  types  or  classes :  1. 
Simple  hypermetropia  of  equal  degree  in  both  eyes.  2.  ITvpermetropie 
astigmatism,  simple  and  compound,  with  the  most  favorable — i.e.,  least 
defective — meridian  of  the  cornea  vertical,  or,  if  inclined  from  the 
vertical,  the  inclination  is  symmetrical  for  the  two  eyes.  3.  TTvperme- 
tropic  astigmatism,  simple  or  compound,  with  asymmetrical  meridians, 


*  Article,  "  School  Hyjriene."  A  System  of  Diseases  of  the  Eye,  edited  by  Norris 
and  Oliver,  vol.  ii. 


NEUROSES   OCCASIONED  BY  EYE-STRxilN         Y49 

— i.e.,  the  meridian  of  least  defect  is  vertical  in  one  eye  and  inclined  in 
the  other  or  irregularly  placed  in  a  great  variety  of  ways.  4.  One 
eye  is  astigmatic,  the  other  emmetropic  or  has  a  simple  hypermetropia, 
or  both  are  hypermetropic,  bnt  in  different  degree  (anisometropia),  or 
both  are  myopic  with  or  without  astigmatism.  5.  One  eye  is  hyperme- 
tropic with  or  without  astigmatism,  the  other  is  myopic  with  or  with- 
out astigmatism  (antemetropia).  6.  Both  eyes  are  myopic  with  or 
without  astigmatism,  which,  as  in  hypermetropic  refraction,  may  be 
symmetrical  or  not,  or  present  in  different  degrees  between  the  two  eyes. 
7.  In  both  hypermetroi)ic  or  myopic  eyes  with  astigmatism  the  meridian 
of  least  defect  may  be  "  against  the  rule," — i.e.,  horizontal  instead  of 
vertical,  symmetrically  or  asymmetrically  inclined  from  the  horizontal. 

When  compared  to  the  emmetropic  or  model  eye  all  of  these  com- 
plex conditions  are  to  be  regarded  as  defects.  They  are  essentially  ana- 
tomical anomalies.  When  viewed  from  the  standpoint  of  physiologic 
optics  they  constitute  the  errors  of  refraction  in  the  eye. 

The  emmetropic  eye  has  an  average  length  of  about  22  millimetres 
and  its  dioptric  system  will  focus  parallel  rays  of  light  on  the  retina, 
at  the  fovea,  without  any  accommodative  effort ;  that  is  to  say,  its  far- 
point  is  infinity,  its  principal  focal  point  lies  on  the  retina.  Such  an 
eye  has  a  range  of  accurate  vision  from  infinity  to  the  physiologic  near- 
point.  The  near-point  gradually  recedes  from  the  eye  until  at  forty-two 
to  forty-five  years  of  age  presbyopia  is  reached.  Any  departure  from 
this  ideal  state  of  refraction  must  sooner  or  later  introduce  disturbance 
of  function.  In  the  routine  of  practice  it  simplifies  the  theoretical  con- 
siderations to  reduce  the  anomalous  states  of  refraction,  as  enumerated 
above,  to  the  ideal  standard  (emmetropia)  by  means  of  carefully  selected 
glasses,  which,  when  placed  before  the  eyes,  neutralize  the  defective  con- 
ditions. To  accomplish  this  would  seem  at  first  sight  a  simple  problem 
in  mathematical  optics,  but  the  human  eye  has  been  provided  with  the 
power  of  accommodation  through  the  agency  of  which  it  is  able  to 
change  the  focus  of  its  dioptric  system.  This  power  makes  it  possible 
even  in  the  presence  of  anomalies  of  refraction,  to  so  change  its  dioptric 
system  as  to  focus  more  or  less  accurately  the  image  of  surrounding 
objects  upon  the  retina.  This  power  resides  in  the  ciliary  muscle  and 
is  exercised  involuntarily.  It  follows  that  in  any  effort  to  measure  the 
static  refraction  of  the  eye,  this  ever  changing  power  of  accommodation 
becomes  an  unknown  quantity  and  must  of  necessity  vitiate  the  results 
obtained.     (Compare  Chapter  II.) 

It  is  not  difficult  to  detect  the  presence  of  the  grosser  forms  of  these 
congenital  defects,  but  they  do  not,  as  a  rule,  comprise  the  most  im- 


750  THE    EYE    AND    NERVOUS    SYSTEM 

portant  group  in  their  relation  to  headache  and  reflex  symptoms.  Wliere 
binocular  vision  is  impossible  because  of  the  gross  nature  of  the  mus- 
cular anomaly,  or  where  the  existing  error  of  refraction  is  too  great 
or  of  such  a  character  as  not  to  be  overcome  by  any  muscular  effort,  the 
afflicted  individual  perforce  resigns  to  the  inevitable,  gives  up  the 
struggle,  and  is  usually  free  from  pain.  It  is  quite  different,  however, 
when  the  existing  error  is  of  such  a  nature  that  the  efforts  of  the  indi- 
vidual can  overcome  it  in  the  interests  of  clear  vision.  It  is  this  effort 
which  constitutes  the  eye-strain  and  is  the  cause  of  intra-ocular  conges- 
tion, weak  eyes,  and  pain, — in  a  word,  of  asthenopia. 

In  the  case  of  grosser  defects  of  vision  diagnosis  is  not  diflBcult, 
since  they  are  accompanied  by  impaired  vision  or  by  a  manifest  ten- 
dency to  double  vision.  Obscurity  exists  only  in  the  case  of  defects 
of  minor  degree,  or  when  the  experience  and  habit  of  a  lifetime  has 
taught  the  individual  the  trick  of  rendering  the  congenital  ocular  defect 
latent ;  that  is  to  say,  of  covering  it  up  by  a  tonic  muscular  tension. 
In  such  cases  the  vision  with  both  eyes  or  for  each  may  be  normal,  or 
approximately  so;  the  range  of  accommodation  and  binocular  balance 
may  be  but  little  if  at  all  deranged,  the  existing  congenital  abnormality 
in  this  group  of  cases  revealing  itself  only  to  careful  analysis  by  the 
skilled  observer.  It  becomes  necessary  in  all  such  patients  to  tem- 
porarily suspend  the  action  of  the  ciliary  muscle  before  any  trustworthy 
determination  can  be  made  of  the  static  refraction  of  the  eye. 

This  is  accomplished  through  the  instillation  of  the  so-called  mydri- 
atics, or  cyclophlegics.  Notwithstanding  the  now  well-known  fact  of  the 
very  high  percentage  of  defective  eyes  and  the  very  generally  recognized 
relation  between  these  defects  and  headache,  the  discussion  of  the  subject 
should  be  approached  with  great  conservatism. 

That  eye-strain  and  certain  diseases  of  the  eye — e.g.,  chorioiditis — 
are  in  many  persons  the  sole  and  sufficient  £ause  for  headache  can  no 
longer  be  questioned  in  the  light  of  experience,  since  the  ophthalmolo- 
gist is  a  daily  witness  of  the  complete  relief  from  the  malady  afforded 
by  the  correction  of  errors  of  refraction  and  abnormalities  in  the  ocular 
muscles  or  by  the  cure  of  intra-ocular  disease.  Experience,  however, 
has  shown  that,  in  other  groups  of  patients  suffering  from  headache, 
there  may  be  no  relation  between  existing  ocular  defects  and  the  pain 
in  the  head,  since  the  most  careful  correction  of  the  existing  anomalies 
affords  no  relief.  Some  examples  of  migraine  belong  to  this  class.  All 
headaches  are  not  due  to  eye-strain.  It  is  not  a  very  unusual  experi- 
ence to  remove  the  eye-strain  by  glasses  to  the  great  relief  of  ocular 
symptoms,  without  any  appreciable  result  in  relieving  the  headache; 


NEUROSES  OCCASIONED  BY  EYE-STRAIN         751 

or,   certain  kinds  of  pain  in  the  head,  in  any  given  patient,  may  be 
relieved  and  others  still  persist. 

It  must  be  borne  in  mind  also  that  many  patients  suffer  greatly 
from  impaired  vision,  chronically  inflamed  and  painful  eyes,  who  never 
have  had  a  headache  or  other  reflex  symptoms.  It  may  be  stated  as  an 
ophthalmic  aphorism  that  when  eye-strain  manifests  itself  by  red  lids, 
recurring  styes  and  symptomatic  conjunctivitis  that  such  persons  com- 
paratively rarely  suffer  from  headache,  while  on  the  other  hand  the 
patients  who  suffer  from  reflex  symptoms  as  the  result  of  ocular  defects 
may  exhibit  no  local  external  inflammatory  or  irritative  manifestations. 
There  are  numerous  exceptions,  however,  to  this  statement.  Then,  too, 
the  subject  grows  in  complexity,  from  the  standpoint  of  diagnosis, 
because  of  the  fact  that  in  two  patients  suffering  from  similar  or 
identical  ocular  defects,  one  may  have  no  symptoms  other  than  weak 
eyes  and  a  blurring  page  after  prolonged  effort,  while  the  other  is  con- 
stantly in  the  slough  of  semi-invalidism  from  pain  in  the  head  and  other 
reflex  symptoms  which,  from  exposure  to  light  or  the  persistent  use  of 
the  eyes,  or  from  the  general  fatigue  following  some  unusual  exertion, 
culminate  in  more  or  less  frequent  and  violent  explosions  of  so-called 
"  sick  or  nervous  headache,"  and  both  of  whom  find  complete  and  last- 
ing relief  from  glasses  so  nearly  alike  that  they  might  be  used  inter- 
changeably. In  a  third  case,  with  a  similar  ocular  defect,  there  are  no 
symptoms  either  reflex  or  local,  the  strain  being  borne  with  impunity. 
While  then  it  is  unquestionably  true  that  in  many  instances  the  exist- 
ing ocular  defects  are  the  sole  and  sufiicient  cause  for  their  headache, 
the  great  difference  in  the  effects  of  eye-strain,  upon  different  individ- 
uals, in  combating  the  untoward  conditions  presented  by  these  con- 
genital anomalies  make  it  necessary  to  look  beyond  the  eye  for  some 
adequate  explanation.  Sufficient  cause  for  the  phenomena  may  often 
be  found  in  the  difference  of  vocation,  environment  or  temperament. 
For  example,  the  persistent  use  of  the  eyes  in  one  case  may  be  unnec- 
essary and  in  the  other  compulsory.  One  may  be  in  good  general  health, 
the  other  a  victim  of  hereditary  or  other  neuroses,  or  some  complicating 
diathesis  which  impairs  the  nutrition  and  introduces  a  tendency  to 
pain  and  local  pathologic  states.  Ambition  to  excel,  or  the  sanguine 
temperament,  may  drive  one  into  constant  activity  in  spite  of  the  pain 
occasioned  by  the  use  of  the  eyes,  while  the  other  rejects  the  goad  of 
ambition  unwilling  to  secure  success  or  to  court  fame  at  the  sacrifice  of 
pain.  Then,  too,  in  the  presence  of  ocular  conditions  causing  eye-strain, 
other  things  being  equal,  the  neurotic  individual  is  more  likely  to  suffer 
from  headache  and  associated  phenomena  than  those  who  cannot  be 


752  THE    EYE    AND    NERVOUS    SYSTEM 

designated  as  nervous  people.  While,  therefore,  the  general  conditions 
01  health  or  temperament  or  the  necessity  for  constant  use  of  the  eyes 
may  in  some  sense  be  regarded  as  the  cause  of  the  headache,  the  primary 
factor  in  its  etiology  is  the  eye-strain,  and  the  pain  will  not  be  pre- 
vented until  the  exciting  cause  is  removed,  although  it  may  be  partially 
relieved  or  modified  by  improvement  in  general  conditions. 

An  extremely  interesting  group  of  patients  are  those  who  suffer  from 
sick  or  nervous  lieadache  and  general  nervous  symptoms, — e.g.,  nausea, 
insomnia,  irritability  of  temper,  facial  chorea  in  children,  etc.,  and  are 
completely  and  permanently  relieved  of  their  symptom  complex  by  the 
correction  of  some  ocular  defect  which  throughout  life  has  been  the 
unsuspected  cause  of  their  suffering. 

Many  patients  who  suffer  severely  from  headache  demonstrably  due 
to  ocular  defects  or  disease,  are  often  quite  free  from  local  symptoms. 
If  ocular  symptoms  are  present  during  acute  exacerbations  of  headache, 
they  occur  pari  passu,  or  come  on  after  the  reflexes,  and  are  regarded 
by  the  patient  as  due  to  headache.  Patients  often  claim  that  their 
"  eyes  are  weak  because  they  have  a  bad  head"  and  submit  to  examina- 
tion of  tlie  eyes  reluctantly  and  only  on  the  advice  of  their  physician, 
deeming  it  unnecessary  since  there  are  no  obvious  eye  symptoms  and 
therefore  apparently  nothing  wrong  with  their  eyes.  In  these  patients 
the  location  of  pain  is  often  suggestive  of  the  ocular  origin  of  the  symp- 
toms. Vertex  pain  is  comparatively  rarely  due  to  eye-strain.  In 
optic-nerve  disease,  as  in  post-ocular  neuritis  and  atrophy,  there  is  in 
many  cases  pain  in  the  front  part  of  the  head  on  top,  but  it  is  not  a 
vertex  pain.  The  pain  when  associated  with  the  class  of  eye  affections 
under  discussion  occurs  in  the  brow,  occiput  or  nape  of  the  neck,  back 
of  the  eyes  and  shooting  thence  to  the  occiput,  as  a  hemicrania,  or  in 
the  temples.  In  point  of  frequency  the  pain  occurs  probably  in  the 
order  named.  Simple  eye-strain  due  to  some  error  of  refraction, — e.g., 
hypermetropic  astigmatism,  particularly  in  children, — will  usually  give 
rise  to  brow  pain.  If  the  refraction  error  is  associated  with  an  imbal- 
ance of  the  ocular  muscles  so  that  constant  muscular  strain  is  required  to 
maintain  single  vision,  as  in  exophoria,  a  tendency  for  the  eyes  to 
diverge ;  or  in  esophoria,  a  tendency  to  converge ;  or  in  hyperphoria,  a 
tendency  of  one  eye  to  turn  upward,  there  will  usually  be  occipital  pain 
also.  If  at  the  same  time  marked  turgidity  or  inflammation  of  the 
chorioid,  retinal  irritation  or  disease  of  the  retina  and  chorioid  in  the 
region  of  tlic  fovea  have  been  set  up  as  the  immediate  result  of  the 
constant  struggle  for  accurate  vision,  in  tlie  majority  of  patients  there 
will  be  a  tense  post-ocular  pain  which  shoots  to  the  occiput,  nape  of 


N^EUROSES  OCCASIONED  BY   EYE-STRAm         753 

the  neck,  or  even  down  the  spine,  not  infrequently  radiating  to  the 
shoulders  and  arms.  In  two  notable  instances  which  caused  great 
anxiety,  one  a  busy  attorney,  the  other  an  eminent  physician,  the  writer 
witnessed  complete  relief  from  pain  in  the  region  of  the  heart,  which 
shot  backward  to  the  left  scapula,  follow  the  correction  of  an  astigma- 
tism against  the  rule  in  the  one,  and  correction  of  hyperphoria  by 
tenotomy  in  the  other. 

Many  of  these  patients,  although  unwilling  to  ascribe  their  suffer- 
ing primarily  to  their  eyes,  nevertheless  recognize  the  harmful  influ- 
ence of  exposure  to  strong  light,  or,  that  exacerbations  of  pain  come  on 
after  extraordinary  use  of  the  eyes,  but  others  never  do  and  ascribe 
their  eye  symptoms  to  "  biliousness,"  or  to  overfatigue,  hunger,  and 
many  other  things. 

Others  regard  their  recurring  sick  headaches  as  hereditary  and  relate 
that  one  or  both  parents  and  other  members  of  the  family  are  victims 
to  the  same  malady.  They  think,  therefore,  that  they  are  born  to  a 
heritage  of  pain  from  which  it  is  useless  to  seek  permanent  relief.  I 
have  seen  many  of  these  lifelong  sufferers  from  recurring  sick  headache 
entirely  and  permanently  relieved  by  the  correction  of  their  faulty  ocu- 
lar conditions.  It  is  doubtless  true  that  the  neurotic  tendency  may  be  a 
family  trait,  but  it  is  equally  true,  even  more  probable,  that  the  ana- 
tomic defects  already  noticed,  upon  which  the  errors  of  refraction  or 
the  ocular  imbalance  depend,  are  hereditary.  The  regularity  with  which 
defective  eyes, — e.g.,  liypermetropia  with  astigmatism, — are  handed 
down  from  parent  to  child  is  one  of  the  most  striking  facts  in  ophthal- 
mology. It  should  be  noted  in  this  connection  how  frequently  it  is  to  the 
form  of  the  skull  that  the  characteristic  family  likeness  is  largely  due. 
It  is  highly  probable  that  the  defective  form  of  the  eyeball  which  pro- 
duces the  ametropia,  and  the  faulty  attachment  of  the  muscles  to  the 
globe  which  causes  the  ocular  imbalance,  are  the  direct  results  of  the 
defects  in  the  shape  of  the  bony  orbit,  and  this  in  turn  is  due  to  distor- 
tion of  the  skull.  It  follows,  therefore,  that  the  possibility  of  hereditary 
anatomical  malformations  should  be  excluded  in  every  case  before 
accepting  the  theory  of  hereditary  headache.     (See  Page  954.) 

There  is  much  variety  of  manifestation  in  different  patients  even 
in  the  presence  of  similar  ocular  defects.  Many  suffer  almost  con- 
stantly from  brow  pain  and  pain  in  the  occii:)ut.  It  is  quite  common  for 
patients  afflicted  with  muscular  anomalies  to  have  their  rest  disturbed  at 
night  by  a  dull,  dragging  pain  in  the  back  of  the  head  and  neck.  Many 
patients  complain  that  they  are  often  awakened  in  the  night  by  this  dis- 
tressing symptom  and  get  relief  only  by  shifting  the  position  of  the 
48 


754  THE    EYE    AND    NERVOUS    SYSTEM 

head.  One  patient  remarked  that  he  always  awoke  in  the  morning 
feeling  as  though  he  had  had  a  billet  of  wood  for  a  pillow.  He  had 
in  addition  to  his  astigmatic  eyes  a  hyperphoria  and  was  not  relieved 
of  his  nocturnal  headache  and  insomnia  until  vertical  balance  between 
tlie  eyes  was  restored  by  tenotomy,  notwithstanding  the  fact  that  his 
weak  eyes  and  headache  had  been  relieved  during  his  waking  hours  by 
glasses  containing  a  prism  which  corrected  the  hyperphoria. 

The  complaints  of  a  busy  physician  who  consulted  the  writer  in  the 
hope  that  his  eyes  might  afford  some  clue  to  the  origin  of  his  symptoms 
furnish  a  vivid  illustration  of  the  unsuspected  participation  of  the  eyes. 
He  suffered  from  almost  constant  occipital  pain,  considered  himself  a 
chronic  dyspeptic,  was  in  despair  over  the  insomnia  which  for  many 
years  had  been  his  constant  terror.  He  had  consulted  many  colleagues 
in  different  cities  without  lasting  relief,  and  finally  was  advised  to  aban- 
don his  work  for  a  year' and  take  a  long  sea  voyage  on  a  slow  sailing 
merchantman.  It  was  on  his  .return  from  this  trip,  which  had  con- 
sumed more  than  six  months,  that  he  consulted  me.  He  had  left  home 
"  a  nervous  wreck,"  had  returned  much  improved  in  many  ways,  but  no 
sooner  had  he  entered  upon  his  work  as  a  rhinologist  than  he  suffered  a 
speedy  return  of  all  his  symptoms.  He  enjoyed  normal  acuity  of  vision 
in  each  eye,  and  had  no  subjective  eye  symptoms.  The  ophthalmoscope 
revealed  marked  retinal  irritation,  however,  the  fundus  being  uniformly 
red  and  fluffy.  There  was  a  low  grade  of  heteronymous  astigmatism,  a 
tendency  for  the  two  eyes  to  converge,  and  a  right  hyperphoria  of  3°. 
The  refraction  error  was  carefully  corrected  under  prolonged  mydriasis, 
and  subsequently  the  hyperphoria  relieved  by  tenotomy.  Very  little 
relief  followed  the  use  of  the  correcting  glasses,  but  as  soon  as  the 
vertical  deviation  between  the  eyes  was  relieved  by  operation  his  symp- 
toms promptly  vanished,  not  only  the  headache  and  insomnia,  but  the 
dyspepsia.  He  ate  with  impunity,  the  morning  following  the  operation, 
"  a  hearty  breakfast  of  sausage,  buckwheat  cakes,  and  coffee,"  and  con- 
tinued for  years  to  eat,  without  subsequent  harm,  anything  he  wished. 
The  general  health  improved  and  he  has  continued  his  work  with  satis- 
faction. 

Many  persons,  although  rarely  free  from  dull  pain  in  the  head, 
suffer  more  or  less  acute  exacerbations  after  exposure  to  light,  from 
prolonged  use  of  the  eyes  at  near-work,  after  an  evening  spent  in  the 
theatre,  or  by  general  fatigue,  but  their  symptoms  are  never  violent. 
Others,  while  like  these,  are  rarely  free  from  pain,  are  subject  to  stead- 
ily progressive  aggravation  of  all  the  symptoms  until  they  culminate  in 
violent  attacks  which  drive  them  to  bed  in  a  darkened  room,  from  which 


NEUKOSES  OCCASIONED  BY  EYE-STRAIN         755 

they  emerge  after  twelve  or  twenty-four  hours  with  a  dull  sore  head,  and 
weak  eyes,  which  are  tender  to  palpation  and  unduly  sensitive  to  light. 

The  explanation  for  the  advancing  symptoms  up  to  the  point  of 
crisis  is  found,  not  only  in  the  muscular  fatigue  and  exhaustion  of  the 
nerve-centres,  hut  in  great  measure  is  due  to  the  steadily  increasing 
hyperemia  and  turgescence  of  the  uveal  tract  of  the  eyes  coming  on 
under  the  strain  of  near-work  or  by  exposure  to  light.  It  is  an  interest- 
ing fact  that  the  acute  symptoms  often  present  themselves  many  hours 
after  the  culminating  cause.  For  example,  an  evening  at  the  opera  will 
be  followed  by  the  acute  attack  the  following  morning  after  a  restless 
or  sleepless  night,  the  insomnia  being  due  to  the  ocular  turgescence. 
The  relief  comes  after  the  subsidence  of  the  ocular  congestion,  which 
results  from  the  confinement  in  a  darkened  room,  the  emesis,  the 
administration  of  a  saline  cathartic,  and  the  cold  compresses  over  eye 
and  brow,  which  are  the  forms  of  treatment  ordinarily  adopted  by  the 
patient,  and  which  experience  has  taught  are  the  most  efiicient  means 
of  relief.  They  return  to  their  usual  vocation  only  to  pass  through  the 
inevitable  cycle  of  suffering.  Many  patients  expect  these  attacks  of 
headache  once  a  week  at  least,  and  return  from  a  journey  or  shopping 
expedition  in  the  full  expectation  of  spending  the  next  twenty-four 
hours  in  bed. 

A  striking  illustration  of  this  group  of  patients  was  afforded  by 
a  young  lady,  a  guest  in  the  writer's  house.  She  arrived  worn  out  by 
a  long  journey.  The  following  morning  she  was  unable  to  appear 
at  breakfast,  and  was  found  in  the  throes  of  a  violent  sick  headache. 
The  eyes  were  red,  the  lids  swollen,  she  had  photopsies  which  she  de- 
scribed as  wheels  of  fire  and  circling  balls  of  red  light.  The  pain  was 
more  severe  in  the  right  side  of  the  head.  She  related  that  she  had 
suffered  these  attacks  two  or  three  times  every  month  since  adolescence ; 
that  when  the  attack  was  imminent  she  usually  awoke  in  the  morning 
with  dim  vision,  scintillating  flashes  of  light  and  zigzag  "  wall  of  troy" 
lights  in  the  periphery  of  the  field  of  vision,  which  at  times  would 
present  all  the  colors  of  the  rainbow.  These  would  repeatedly  narrow 
down  to  near  the  centre  of  the  field,  then  widen  out  into  broader  cir- 
cles, grow  fainter  and  finally  disappear,  leaving  her  with  obscured  cen- 
tral vision,  objects  being  seen  as  through  a  veil.  This  obscuration  would 
last  but  a  brief  time,  but  was  always  followed  by  nausea  and  vomiting, 
and  the  onset  of  violent  headache,  usually  a  right  hemicrania  at  first, 
which  later  spread  to  the  entire  head.  Every  noise  or  ray  of  light  added 
to  her  suffering,  the  slamming  of  a  door  being  especially  excruciating, 
as  it  seemed  to  her  to  shake  the  entire  house.      The  attacks  usually 


756  THE    EYE    AND    NERVOUS    SYSTEM 

forced  her  to  remain  in  bed  for  the  entire  day,  and  her  "  head  felt  tired 
and  tender"  for  a  day  and  often  two  days  after  the  subsidence  of  the 
acute  symptoms.  Her  mother  and  an  older  brother  had  suffered  in  the 
same  manner  since  her  earliest  recollection. 

A  solution  of  atropia  sulphate  was  instilled  immediately  into  both 
eyes  and  repeated  at  brief  intervals.  All  other  means  of  relief  were 
omitted.  In  an  hour  the  pain  subsided,  and  in  two  hours  she  was  able 
to  come  down  stairs  to  my  consulting  room.  I  there  found  a  hyperme- 
tropic astigmatism,  not  in  high  degree,  but  with  the  axis  of  the  required 
correcting  cylinder  against  the  rule  in  the  left  eye,  and  diagonal  in  the 
right.  There  were  absorption  changes  in  the  fundus  of  both  eyes,  and 
macular  retinochorioiditis  in  the  right,  a  fact  which,  together  with  the 
diagonal  corneal  meridians,  explained  the  right  hemicrania.  After  a 
few  days  of  treatment  she  received  a  pair  of  correcting  glasses.  These 
she  has  worn  for  twenty  years  with  no  recurrence  of  her  attacks  of 
"  migraine ;"  she  has  suffered  rarely  from  a  dull  headache,  but  then  only 
after  special  stress  of  circumstances  or  when  her  "  glasses  were  fitting 
badly." 

Another  interesting  example  of  this  group  of  cases  was  furnished 
by  a  lady  aged  49  years,  who  Avrote  from  a  distant  city  for  an  appoint- 
ment. She  desired  a  consultation  at  her  hotel,  as  she  wrote  that  she 
would  be  suffering  too  severely  from  "  sick  headache"  after  her  journey 
to  be  able  to  leave  her  bed  for  a  day  or  possibly  two  days  thereafter. 
^^^len  the  writer  called  at  the  appointed  time  he  found  her,  as  she  had 
predicted,  in  the  throes  of  a  violent  headache ;  nervous,  exhausted,  suf- 
fering from  nausea  and  fruitless  attempts  at  emesis.  A  mydriatic  was 
immediately  instilled,  and  cold  compresses  applied  to  the  closed  lids, 
and  potassium  bromide  administered.  The  patient  was  completely 
relieved  in  an  hour,  rose,  ate  a  hearty  meal,  and  the  following  morning 
was  able  to  go  to  the  office  of  the  writer.  Here  it  was  ascertained  that 
she  had  a  high  grade  of  hypermetropic  astigmatism.  After  this  had 
been  corrected  by  glasses  she  was  permitted  to  return  to  her  home.  Upon 
her  arrival  she  wrote  that  for  the  first  time  in  her  experience  she  had 
been  able  to  enjoy  a  railroad  journey  and  had  reached  her  home  in  per- 
fect comfort.  Ocular  defects  are  often  the  cause  of  headache  after  a 
journey  by  rail,  in  a  carriage,  or  after  shopping  or  mingling  witli 
crowds  of  people. 

riosely  allied  to  this  group  of  patients  are  those  who  claim  to  be 
iiervous  and  suffer  from  what  they  call  nervous  headache.  They  are 
rarely  nauseated,  but  have  violent  pains  with  muscular  twitchings  and 
hysterical  crises.    They  usually  seek  for  relief  in  opiates  and  analgesics. 


Is^EUROSES  OCCASIONED  BY  EYE-STRAI:N^         757 

Such  patients  are  generally  women,  but  the  writer  recalls  the  case  of  a 
merchant,  the  father  of  a  numerous  family  of  children,  and  the  head  of 
a  large  mercantile  house,  who  had  high  hypermetropic  astigmatism  and 
right  hyperphoria.  The  right  side  of  the  face  was  smaller  than  the  left, 
the  right  zygoma  being  flat,  while  the  left  formed  an  arch.  The  hyper- 
metropia  was  higher  in  the  right  eye  than  in  the  left,  and  the  principal 
meridians  of  the  cornea  were  inclined  45°  from  the  horizontal  plane. 
The  refraction  defect  had  been  partially  corrected  by  glasses,  but  the 
ocular  imbalance  neglected.  Suddenly,  while  at  his  place  of  business, 
he  would  have  some  obscuration  of  vision  accompanied  by  shimmering, 
rainbow-colored  phosphenes  in  the  periphery  of  his  field  of  vision. 
Taught  by  many  experiences  he  would  hastily  summon  a  cab  to  take 
him  to  his  home,  for  he  recognized  the  phosphenes  as  an  invariable 
prodrome  of  an  attack  of  severe  headache.  His  wife  described  to  me 
one  of  these  exacerbations. 

Arrived  at  home,  his  fortitude  entirely  exhausted,  he  would  ring 
the  doorbell  violently,  enter  the  house  holding  his  head  between  his 
hands,  and  often  unable  to  reach  his  bedroom,  would  throw  himself 
upon  the  hall  or  parlor  floor  in  despair,  and  entirely  beside  himself  in 
an  agony  of  suffering,  burst  into  a  flood  of  tears,  roar  with  pain,  making 
the  most  violent  manifestations,  alarming  his  wife,  children,  and  ser- 
vants, until  the  doctor  came  and  relieved  him  by  a  hypodermic  injec- 
tion of  morphia.  These  violent  attacks  were  relieved  by  careful  correc- 
tion of  his  refraction  error  and  a  subsequent  tenotomy  of  the  right 
superior  rectus  muscle;  but  he  still  suffers  from  a  dull  occipital  pain 
after  a  "  hard  day."  Since  these  attacks  often  came  on  after  some 
fancied  indulgence  at  the  table,  or  after  an  evening  spent  at  the  theatre 
followed  by  a  supper,  he  ascribed  them  to  deranged  stomach  and  liver, 
and  was  in  consequence  a  victim  to  the  cathartic  habit. 

These  violent  onsets  of  pain  in  the  head  are  occasionally  associated 
with  more  or  less  violent  storms  of  the  nervous  system,  hysterical  crises, 
cataleptoid  seizures,  and  in  one  case  which  came  under  my  eye  the 
attacks  were  followed  by  what  had  at  least  been  diagnosticated  by  an 
eminent  authority  as  epilepsy. 

A  man  thirty-five  years  of  age  had  for  several  years  been  under 
treatment  for  epilepsy,  but  suffered  almost  constantly  from  headache, 
which  culminated  in  paroxysms  of  pain  preceded  by  phosphenes,  which 
had  come  to  be  regarded  as  the  aura  of  his  epileptic  seizures.  He  was 
sent  to  me  for  blurring  page,  and  in  the  hope  that  his  headache  might 
be  relieved  by  glasses.  I  found  a  moderate  degree  of  refraction  error, 
corrected  by  -|-1.50*-f-.50^  ax.  180°  in  each  eye,  and  a  marked  abso- 
lute insufficiency  of  the  internal  recti.     He  received  correcting  glasses 


758  THE    EYE    AND    NERVOUS    SYSTEM 

for  constant  wear  and  a  second  pair  combined  with  prisms,  base  in,  for 
all  continuous  near-work.  I  did  not  hear  from  him  again  for  eight 
years,  when  he  returned  from  his  distant  home  for  some  change  in  his 
glasses,  and  much  to  my  surprise  said  that  he  had  not  had  a  single 
return  of  his  headache  or  convulsions  after  the  treatment  of  his  eyes, 
lie  then  described  to  me  his  former  attacks.  Hurrying  to  his  home 
with  the  first  appearance  of  the  phosphenes,  he  would  soon  be  in  the 
throes  of  a  violent  headache.  The  paroxysms  would  culminate  in  loss 
of  consciousness,  general  convulsions,  frothing  at  the  mouth  and  wound- 
ing of  the  tongue,  after  which  he  would  fall  into  a  profound  sleep  from 
which  he  could  not  be  awakened,  often  lasting  for  several  hours. 

Another  striking  instance  of  headache  associated  with  nervous  symp- 
toms was  a  tall,  thin,  pallid,  nervous  lad,  with  red  hair,  age  17.  He 
was  the  son  of  a  busy  attorney  who  was  overworked  and  in  feeble  health, 
and  a  nervous,  hysterical  mother  who  had  mixed  astigmatism.  The 
father  and  a  brother  had  high  myopic  astigmatism  and  chorioiditis. 
The  boy  was  brought  because  of  his  weak  eyes  and  severe  headaches, 
often  attended  with  nausea  and  vertigo.  The  day  before  his  visit  to 
me,  the  father  had  found  him  in  an  unconscious  condition  in  an  arm- 
chair in  the  library  before  breakfast,  with  his  legs  and  arms  extended 
and  rigid,  his  head  thrown  back,  the  eyes  wide  open,  and  the  morning 
newspaper  fallen  from  his  hands.  While  examining  the  eyes  I  had  an 
opportunity  to  witness  one  of  these  cataleptic  seizures,  which  came  on 
while  attempting  to  determine  the  range  of  accommodation.  He  had 
a  high  degree  of  mixed  astigmatism  and  insufficiency  of  the  interni. 
There  was  marked  chorioiditis  and  the  eyeballs  were  stretching;  that 
is  to  say,  the  refraction  was  passing  from  hypermetropia  into  myopia 
through  the  turnstile  of  astigmatism.  His  eyes  were  subjected  to  pro- 
longed treatment  and  finally  corrected  by  glasses  resulting  in  relief  so 
nearly  complete  that  he  finished  his  college  course  and  became  a  success- 
ful civil  engineer.  There  can  be  but  little  doubt  as  to  the  relation  of 
cause  and  effect  between  his  ocular  conditions  and  the  storm  in  his  ner- 
vous system.  Although  he  received  alteratives  internally,  and  his 
general  regimen  was  carefully  regulated,  all  this  had  been  done  before 
by  a  wise  and  experienced  family  physician  who  still  conducted  the 
general  treatment. 

The  group  of  patients  illustrated  by  the  foregoing  examples,  who 
had  all  been  lifelong  sufferers  from  headache  and  other  neuroses,  aggra- 
vated by  any  occupation  involving  the  use  of  the  eyes,  are  those  had  in 
mind  by  Gould  in  his  Biographic  Clinics,  and  render  highly  probable 
the  truth  of  his  contention,  at  least  in  the  examples  selected  by  him  from 
famous  literary  folk,  by  way  of  illustration  of  his  thesis.     They  all  may 


NEUROSES  OCCASIONED  BY  EYE-STRAIN         759 

without  difficulty,  so  far  as  the  symptomatology  is  concerned,  be  repro- 
duced again  and  again  from  the  case  books  of  every  ophthalmologist. 

A  class  of  patients,  some  of  whom  present  great  difficulty  in  diagno- 
sis, are  those  who  develop  headache  during  and  after  middle  life.  The 
pain  is  periorbital,  post-ocular,  or  occipital,  and  may  present  any  of  the 
characteristic  features  already  described  in  the  preceding  groups.  The 
pain  may  be  dull  and  constant,  more  or  less  aggravated  by  using  the 
eyes  or  by  exposure  to  light,  or  they  may  have  severe  periodical  exacerba- 
tions of  pain.  Any  existing  refraction  error  or  other  ocular  defects 
have  heretofore  given  no  evidence  of  their  presence,  or  the  eye  may  be 
approximately  emmetropic  and  the  vision  normal,  and  the  patient  never- 
theless associate  his  pain  with  the  use  of  his  eyes.  A  careful  ophthal- 
moscopic study  reveals  a  maroon-colored  fundus  with  pigment  clouds, 
and  fine  granular  changes  in  the  region  of  the  fovea  and  between  it 
and  the  optic  nerve.  The  nerve  itself,  while  possibly  clearly  outlined,  is 
too  vascular  and  appears  therefore  too  deeply  colored.  The  history  may 
be  entirely  negative  regarding  any  recognized  ocular  disturbance  in  the 
past.  There  is  a  widespread  feeling  in  the  community  that  glasses 
should  be  avoided  as  long  as  possible,  many  persons  taking  pride  in  the 
fact  that  they  are  forty-five  or  even  fifty  years  of  age  and  have  never 
worn  glasses.  The  fact  is  that,  given  an  emmetropic  or  model  eye,  the 
physiologic  range  of  accommodation  is  so  far  diminished  in  most  per- 
sons soon  after  forty  years  of  age,  that  a  weak  convex  glass  for  reading 
will  afford  both  additional  comfort  and  safety.  When  an  extra  innerva- 
tion strain  must  be  placed  upon  the  accommodation  to  secure  satisfac- 
tory vision  in  reading,  certain  anomalies  of  convergence  are  produced 
which  disturb  the  ocular  balance  and  all  of  the  chain  of  phenomena 
depending  upon  eye-strain  follow, — e.g.,  headache,  intra-ocular  hyper- 
aemia,  and  chronic  irritation  or  inflammatory  changes  in  the  fundus. 
As  representing  tliis  group  may  be  cited  the  case  of  an  eminent  clergy- 
man aged  fifty.  This  gentleman  had  resting  upon  him  not  only  the 
responsibilities  of  a  large  and  influential  parish,  but  often  occupied  the 
lecture  platform,  and  was  a  frequent  contributor  to  religious  and  lit- 
erary periodicals  and  the  author  of  several  books.  He  began  to  suffer 
from  insomnia  and  almost  immediately  became  the  victim  of  severe 
occipital  pains  and  occasional  attacks  of  vertigo,  for  which  he  sought 
the  advice  of  his  physician.  A  diagnosis  of  cerebral  hj^ersemia  from 
overwork  was  made  and  a  grave  prognosis  given.  He  was  advised  to 
abandon  his  work  for  a  year  and  seek  relief  in  foreign  travel.  Quite 
disheartened,  he  consulted  the  writer  and  told  him  of  his  plans.  His 
symptoms  suggested  the  possibility  of  eye-strain,  and  brought  out  the 


760  THE    EYE    AND    NERVOUS    SYSTEM 

fact  that  he  had  never  worn  glasses  notwithstanding  his  age,  and  the 
enormous  amount  of  literary  and  other  work  he  was  doing.  He  received 
a  pair  of  working  glasses.  His  insomnia  and  headache  disappeared 
promptly,  and  although  arrangements  had  been  made  for  a  long  vaca- 
tion tliey  were  cancelled  and  he  went  on  with  his  work.  He  was  to  all 
appearances  in  perfect  health  and  was  a  noteworthy  example  of  stalwart 
manhood.  There  was,  therefore,  no  suspicion  of  any  neurotic  or  other 
predisposing  element  entering  as  an  etiologic  factor,  his  symptoms 
unquestionably  being  the  result  of  his  neglect  to  neutralize  by  glasses 
the  physiologic  loss  in  his  range  of  accommodation. 

Another  simple  explanation  for  the  sudden  onset  of  pain  after  40 
years  of  age  is  the  existence  of  minor  errors  of  refraction  which  have 
given  no  recognized  trouble  until  the  diminishing  range  of  accommoda- 
tion has  arrived  at  a  point  where  the  accommodation  strain  sustains 
such  a  relation  to  its  total  value  that  it  can  no  longer  be  borne  with 
impunity  and  hence  the  onset  of  pain  and  its  attendant  phenomena. 
The  only  source  of  relief  for  such  patients  is  the  use  of  carefully  selected 
glasses.  These  two  groups  of  patients  fortunately  comprise  the  larger 
percentage  of  patients  between  forty  and  fifty  years  of  age  who  seek 
the  advice  of  the  ophthalmologist  for  the  somewhat  sudden  onset  of 
headache. 

It  must  be  borne  in  mind,  however,  that  the  loss  of  accommodation 
is  due  to  a  general  diminution  in  the  elasticity  of  the  crystalline  lens,  a 
change  probably  indicative  of  like  degeneration  processes  going  on  in 
the  other  tissues  of  the  body  and  which  culminate  in  old  age.  They 
have  at  least  reached  the  age  of  beginning  degeneration. 

In  many  persons  certainly,  the  wear  and  tear  of  life  begins  to  mani- 
fest itself  between  forty  and  fifty  years  of  age  in  tissue  changes,  often 
pathologic  in  character.  The  commencing  affections  of  the  general  vas- 
cular tree  are  visible  not  infrequently  to  the  magnified  image  of  the  de- 
tails of  the  eye-ground  furnished  by  the  ophthalmoscope  before  any  sus- 
picion of  their  presence  is  afforded  by  any  notable  general  symptoms; 
but  the  appearances  in  the  fundus,  while  they  awaken  the  suspicion  of 
the  ophthalmic  surgeon,  and  may  lead  to  a  study  of  general  conditions, 
are  not  always  characteristic  of  any  specific  dyscrasia  and  may  readily 
be  ascribed  to  the  irritation  and  other  local  changes  caused  by  pro- 
longed eye-strain,  as  described  in  the  preceding  paragraphs.  It  is  there- 
fore possible  to  err  in  diagnosis  at  this  point,  since  the  vascular  optic 
disk,  the  general  fundus  conditions,  and  the  headache  may  be  a  part  of 
the  symptom  complex  of  some  serious  dyscrasia  which  is  sapping  the 
vital  functions.     The  existence,  therefore,  of  some  refraction  defect,  or 


NEUROSES  OCCASIOXED  BY  EYE-STRAIX         TGI 

neglect  of  the  timely  use  of  suitable  glasses  for  near  work  should  not 
be  too  readily  accepted  as  the  sole  cause  of  headache  in  patients  who 
have  reached  middle  age. 

The  following  case  will  illustrate  the  need  for  great  care. 

^Ir.  X.,  aged  49  years,  an  artist  who  spent  many  hours  daily  in  his 
studio,  had  for  several  years  suffered  more  or  less  constant  occipito- 
frontal pain  with  frequent  paroxysms  of  nervous  headache  which  forced 
him  to  bed.  In  1902,  following  one  of  these  attacks,  he  noticed  a  central 
blurred  spot  before  the  left  eye.  This  proved  to  be  due  to  a  minute 
hemorrhage  at  the  fovea.  The  fundus  of  both  eyes  was  cinnabar  red, 
and  there  were  numerous  dark  pigment  clouds  in  the  periphery.  The 
veins  were  too  full  and  dark,  the  optic  nerves  dark  red  but  not  swollen, 
there  were  faint  gray  lines  along  the  larger  retinal  arteries,  and  fine 
granular  changes  in  the  macular  region  of  both  eyes.  He  was  using 
for  his  work  weak  convex  spherical  glasses,  whereas  he  had  in  both  eyes 
hypermetropic  astigmatism  requiring  the  axis  of  the  correcting  cylin- 
ders against  the  rule.  Here  was  sufficient  cause  for  his  headache,  and 
it  is  not  uncommon  to  find  similar  fundus  conditions  due  solely  to  the 
continued  eye-strain  to  which  his  eyes  had  been  subjected.  The  macular 
hemorrhage,  however,  and  the  gray  outline  of  the  arteries,  which  sug- 
gested endarteritis,  could  not  be  overlooked.  He  ascribed  his  trouble 
to  an  attempt  to  reproduce  upon  the  canvas  the  glint  from  a  bright 
sabre  which  was  placed  in  such  a  position  as  to  reflect  a  strong  light. 
The  effort  had  developed  a  severe  paroxysm  of  headache.  He  consid- 
ered himself  in  good  general  health.  He  had  never  eaten  meat,  having 
been  reared  as  a  vegetarian.  There  was  no  discoverable  arterial  athe- 
roma, but  he  had  an  accentuated  systole  and  any  vigorous  exertion 
caused  dyspnoea  of  a  mild  type.  His  habits  were  sedentary.  He  often 
passed  too  great  quantities  of  urine,  and  a  specimen  examined  was 
negative  but  had  a  low  specific  gravity  and  a  few  hyaline  casts.  His 
health  failed  rapidly,  an  intermittent  albuminuria  developed,  and  he 
died  in  ursemic  convulsions  in  a  year.  The  acute  symptoms  came  on 
while  he  was  on  a  sketching  tour  in  the  mountains,  and  was  ushered  in 
by  one  of  his  paroxysmal  headaches.  The  glasses  he  received  had  for 
some  months  modified  his  head  symptoms,  and  doubtless  the  eye-strain 
was  a  factor  in  their  etiology,  but  behind  the  local  conditions  lurked, 
unsuspected  by  himself  or  his  family  physician,  the  general  dyscrasia 
which  led  to  a  speedily  fatal  termination. 

Dr.  K.,  aged  56,  a  dentist,  became  the  victim  of  insomnia,  dyspeptic 
symptoms  and  occipital  headache  ,with  frequent  attacks  of  vertigo.  A 
diagnosis    of   "  concealed    gout"    had    been    made,    and    his    headache 


762  THE    EYE    AND    NERVOUS    SYSTEM 

ascribed  to  cerebral  hyperaemia.  The  general  regimen  had  been  care- 
fully and  wisely  regulated,  comprising  a  prescribed  diet,  horseback 
exercise,  bathing,  etc.,  together  with  lithia  waters  and  drugs.  In  spite 
of  an  intelligent  and  rigid  obedience  in  carrying  out  the  prescribed 
rules  laid  down  for  his  guidance,  the  symptoms  persisted  and  drove 
him  from  his  work  daily  with  severe  occipital  pain.  He  was  then 
advised  to  abandon  his  work  and  spend  six  months  in  Switzerland. 
He  experienced  marked  relief  and  returned  to  his  chair,  only  to  suffer 
a  speedy  relapse.  He  consulted  me  then  of  his  own  volition  about  his 
eyes.  He  was  unquestionably  the  subject  of  lithaemia,  but  that  this 
was. not  the  sole  cause  of  his  headache  was  demonstrated  by  a  steady  but 
rapid  subsidence  of  his  symptoms  after  the  correction  of  an  astigmatism 
which  had  been  neglected  in  the  glasses  he  had  been  wearing.  He  is 
now  nearing  seventy  years  of  age  and  has  steadily  pursued  tlie  arduous 
duties  of  his  profession  without  a  return  of  his  symptoms. 

It  is  not  unusual  for  a  patient  during  or  after  attacks  of  acute 
disease, — e.g.,  coryza,  influenza,  pneumonia,  etc. — to  become  the  victim 
of  headache  which  persists  and  is  aggravated  into  acute  paroxysms  by 
the  use  of  the  eyes.  Examination  reveals  the  fact  that  they  are  sub- 
jects of  some  congenital  anomaly  of  refraction,  which  has  for  the  first 
time  manifested  itself  in  the  symptoms  of  eye-strain,  under  the  low- 
ered physical  tone  produced  by  disease,  and  they  are  never  again  able 
to  avoid  the  use  of  correcting  glasses. 

There  are  numerous  patients  who  never  suffer  severe  pain  as  the 
result  of  their  eye-strain,  but  are  nevertheless  victims  of  a  group  of 
symptoms  which  must  be  classed  as  ocular  neuroses.  Insomnia  has 
been  mentioned  as  a  frequent  symptom.  The  reverse  of  this  is  a  com- 
mon complaint.  Many  persons  become  drowsy,  especially  in  the  even- 
ing, and  find  it  quite  impossible  to  read  or  do  any  eye  work  without 
falling  asleep.  I  have  many  times  seen  this  relieved  by  a  pair  of 
glasses.  A  constant  sense  of  general  fatigue  or  malaise  is  often  caused 
by  eye-strain.  A  busy  physician  came  for  relief  for  some  illy  defined 
ocular  disturbance.  He  was  49  years  of  age  and  used  -|-2D.  for 
reading.  He  received  for  constant  wear  the  following  formula  for 
glasses : 

O.D.  +    .62  C  +  1."  ax.    30°  V.  =  ;^ 

O.S.   +  1.00  C  +  l.«  ax.  150°  V.  =  ;— 

with  a  reading  segment  added  of  -}-  1.75D. 

He  returned  in  a  week  to  express  his  satisfaction  and  remarked  that 


NEUROSES  OCCASIONED  BY  EYE-STEAIN         763 

he  had  been  chronically  tired  for  a  long  time ;  had  had  his  heart  exam- 
ined and  an  analysis  made  of  his  urine,  fearing  the  incipiency  of  some 
organic  disease,  but  that  all  these  symptoms  had  promptly  disappeared 
under  the  relief  from  his  eye-strain. 

The  facial  twitchings  of  school  children,  with  brow  pain,  irritabil- 
ity of  temper,  uncontrollable  restlessness,  disturbed  sleep,  precarious 
appetite,  and  poor  progress  at  school,  all  of  which  make  them  the  despair 
of  their  mothers  and  teachers,  have  many  times  been  relieved  by  a  pair 
of  glasses.  Whether  these  cases  can  be  properly  classed  as  petit  chorea 
or  not,  it  is  nevertheless  certain  that  the  eye-strain  is  for  them  "  the 
thorn  in  the  flesh"  which  produces  a  constant  irritation  of  the  nervous 
system.  In  another  group,  although  the  defective  eyes  are  evidently 
an  important  factor  in  the  causation  of  symptoms,  they  are  obviously 
not  the  sole  cause.  The  above  symptom  complex  may  be  present,  but 
there  is  in  addition  many  evidences  of  a  vicious  metabolism.  The 
eye  ground  is  fluffy  and  turgid,  with  here  and  there  areas  of  pigment 
absorption ;  the  veins  are  large  and  dark,  the  lymph  channels  are 
engorged,  the  optic  disk  is  too  red  and  its  margins  obscured ;  the  retro- 
tarsal  folds  of  the  conjunctiva  are  thickened  and  opaque,  the  caruncles 
are  swollen  and  red,  they  have  blepharitis  and  frequently  recurring 
styes.  A  careful  and  painstaking  study  will  in  many  cases  reveal  an 
irregular  astigmatism  as  shown  by  the  ophthalmometer  or  by  skioscopy. 
They  suffer  severely  from  frontal  headache  and  undue  sensitiveness  to 
light;  the  anterior  perforating  blood-vessels  are  enlarged.  No  glass 
will  afford  a  normal  acuity  of  vision.  The  pain  in  the  head,  the  restless 
sleep,  the  precarious  appetite,  the  muddy  skin,  the  many  evidences  of 
deranged  alimentation  and  impaired  metabolism,  make  it  difficult  to 
draw  the  line  sharply  as  to  the  relative  responsibility  of  local  and  gen- 
eral conditions.  The  eyes  are  unquestionably  suffering  from  the  strain 
at  school  in  overcoming  a  congenital  anomaly.  The  anterior  segment 
of  the  eyeballs  is  stretching  under  the  increased  pressure  from  within, 
due  to  the  hyperaemia  and  turgescence  of  the  intra-ocular  tunics,  and 
this  increased  tension  is  doubtless  in  some  measure  the  origin  of  the 
brow  pain  and  other  reflex  phenomena ;  but  it  is  doubtless  true  also 
that  these  local  ocular  conditions  might  not,  and  probably  would  not, 
have  occurred,  in  many  cases  certainly,  but  for  the  predisposing  influ- 
ence of  the  impairment  of  the  general  nutrition.  While,  therefore,  it 
would  be  a  fallacy  to  ascribe  all  of  this  group  of  conditions  to  eye-strain 
alone,  nevertheless  the  correcting  glasses  and  prolonged  rest  from  near- 
work  often  prove  of  great  relief  to  both  eyes  and  head.  The  sleep 
improves,  the  nervous  symptoms  disappear,  and  in  many  instances  the 


764  THE    EYE    AND    NERVOUS    SYSTEM 

improvement  in  general  health  is  so  speedy,  following  the  treatment 
of  the  eyes,  that  the  inquiry  is  suggested  whether  the  impaired  metab- 
olism was  not  in  large  measure  due  to  the  restless  sleep,  and  constant 
headache  due  to  ocular  conditions.  Analogous  states  have  been 
observed  as  due  to  pharyngeal  adenoids  and  to  phymosis,  so  that  it  is 
not  unreasonable  to  suppose  that  similar  general  conditions  may  be 
produced  by  the  pain  and  irritation  of  prolonged  eye-strain,  the  conse- 
quent fatigue  of  the  nerve-centres  involved,  and  the  turgid  states  of  the 
intra-ocular  tunics. 

Tiie  headache  and  insomnia  of  aged  people  is  not  infrequently  pro- 
duced by  similar  conditions  of  the  chorioid  and  retina,  and  rapidly 
disappear  under  suitable  local  treatment  addressed  to  the  ocular  condi- 
tions alone.  I  have  seen  many  examples  of  this,  some  of  which  excite 
commiseration.  The  following  history  is  illustrative :  Miss  McA.,  aged 
68  years,  came  for  advice  regarding  her  failing  vision,  inflamed  eye- 
lids and  a  troublesome  nictitation.  Vision  was  much  reduced  in  both 
eyes,  but  was  improved  by  convex  glasses.  She  had  hypermetropic 
astigmatism  in  both  but  much  higher  in  the  left  eye  than  in  the  right. 
There  was  no  complaint  of  pain.  The  caruncles  were  red  and  swollen, 
there  was  long-standing  blepharitis,  incipient  cataract,  floating  vitre- 
ous web,  and  chorioiditis  with  macular  retinitis  were  present  in  both 
eyes.  Inquiry  about  her  general  health  educed  the  reply  that  all  her 
life  she  had  been  a  victim  of  sick  headaches.  She  recalled  that  as  a 
small  child  she  returned  from  school  with  red  eyes  and  headache.  Bury- 
ing her  head  in  her  mother's  lap  she  would  burst  into  tears  from  the 
violent  pain.  These  attacks  had  continued  until  fifty  years  of  age, 
but  since  then,  although  she  suffered  more  or  less  constantly  from 
"  neuralgia,"  the  pain  did  not  cause  nausea  or  emesis  as  in  former 
years.  The  neuralgia  had  been  "  better  of  late  because  of  medicine 
from  her  physician,"  which  proved  to  be  Kenyon's  neuralgia  pills.  So 
utterly  w^as  this  lifetime  of  pain  divorced  in  her  mind  from  ocular  par- 
ticipation that  she  did  not  think  it  necessary  to  mention  it  until  the 
history  was  drawn  out  by  inquiry.  Yet  the  ophthalmoscope  revealed  the 
scars  produced  by  years  of  eye-strain,  which  was  finally  culminating  in 
impaired  nutrition  of  the  vitreous  body  and  the  crystalline  lens.  The 
neuralgia  pill  was  omitted,  homatropine  instilled  four  times  daily,  and 
smoked  glasses  with  complete  rest  of  the  eyes  advised.  In  a  few  days 
the  external  redness  and  nictitation  disappeared,  she  slept  soundly,  the 
appetite  improved,  and  a  sense  of  discomfort  and  tension  about  the 
head  vanished. 

It  is  therefore  important  in  both  children  and  adults  who  are  ob- 


NEUROSES  OCCASIONED  BY  EYE-STRAIN         765 

• 
vioiisly  the   subjects   of  impaired  general   nutrition   and  suffer   from 
headache  that  the  ill  health  should  not  be  too  rigorously  regarded  as 
the  sole  cause  of  all  their  symptoms. 

In  a  large  percentage  of  persons  whose  headaches  are  without 
question  due  solely  to  eye-strain  and  its  associated  local  conditions,  the 
symptoms  disappear  promptly  and  permanently  under  correcting 
glasses,  but  in  many  others  the  result  is  disappointing.  It  has  already 
been  j^ointed  out  that  as  a  result  of  protracted  eye-strain  pathologic 
states  of  the  fundus  oculi  are  produced.  The  turgid,  fluffy  chorioid, 
absorption  chorioidal  crescents  at  the  temporal  margin  of  the  optic 
nerves,  granular  changes  at  the  fovea  with  impaired  acuity  of  central 
vision  are  conditions  witnessed  daily  in  the  consulting  room  as  the  out- 
come of  constant  strain  required  to  overcome  the  existing  congenital 
defect.  The  congestion  of  the  vascular  chorioid  produces  increased 
intra-ocular  tension  and  pressure  upon  the  terminals  of  the  ciliary 
nerves,  resulting  in  headache  and  the  other  reflex  symptoms  under  dis- 
cussion. The  tender,  readily-yielding  sclerotic  coat  of  childhood  often 
yields  to  this  increased  pressure  from  within ;  the  eyeballs  stretch  and 
pass  from  hypermetropic  refraction  into  myopia.  It  is  to  these  condi- 
tions that  the  increasing  percentage  of  near-sight  among  school  children 
is  due,  and  statistics  have  shown  that  in  this  group  is  found  the  sufferers 
from  headache,  restlessness  in  school,  blepharitis,  weak  eyes,  and  im- 
paired acuity  of  vision.  The  distention  of  the  globe  is  not  always  uni- 
form, sometimes  falling  upon  the  posterior  pole  as  in  many  cases  of 
high  myopia,  but  often  affecting  the  anterior  segment,  especially  in  the 
ciliary  region,  which  becomes  thin,  appears  bluish  in  color,  and  is  trav- 
ersed by  the  dilated  anterior  ciliary  blood-vessels  which  perforate  the 
globe  in  this  locality  to  anastomose  with  the  long  posterior  ciliary 
arteries  in  the  ciliary  region  of  the  uveal  tract.  It  is  in  these  cases  of 
change  in  the  curvature  of  the  anterior  portion  of  the  ball  that  varia- 
tions in  the  corneal  astigmatism  are  observed.  Now  it  is  not  to  be 
expected  that  these  conditions,  which  gravely  involve  the  nutrition  of  the 
entire  globe  of  the  eye,  will  vanish  at  once  under  the  use  of  correcting 
glasses.  More  or  less  protracted  rest  from  use  at  a  near-point,  avoid- 
ance of  exposure  to  strong  light,  careful  attention  to  the  general  regi- 
men, and,  more  than  all,  the  passage  of  sufficient  time  for  recovery,  are 
all  required  to  effect  relief  from  symptoms  and  a  cure  of  the  pathologic 
states  primarily  set  up  by  the  eye-strain. 

Taken  at  the  onset  of  symptoms  the  results  might  have  been  pre- 
vented. These  are  the  conditions  which  lie  at  the  foundation  of  the 
modern  requirement  that  no  child  shall  be  permitted  to  enter  upon 


766  THE    EYE    AND    NERVOUS    SYSTEM 

• 
school   life   until   examination   of   the   eyes   has   either   excluded   the 
existence  of  these  congenital   defects  or  secured  their  correction  by 
glasses. 

The  same  chain  of  results  may  occur  in  adults  without  the  existence 
of  anomalies  of  refraction,  through  the  neglect  of  suitable  glasses  after 
the  onset  of  presbyopia;  macular  disease,  hyperaemia  of  the  chorioid 
with  occipital  or  frontal  pain,  often  occur  after  forty  years  of  age. 
The  tougher  sclerotic  coat  of  adult  life  is  not  so  liable  to  distention  as 
in  childhood  and  youth,  but  the  reflex  pain,  hypera?mia  of  the  con- 
junctiva and  impaired  nutrition  of  the  globe  are  equally  imminent, 
and  relief  comes  only  after  sufficient  time  has  elapsed,  under  suitable 
hygienic  conditions,  to  permit  a  subsidence  of  the  pathologic  condi- 
tions of  the  tissues.  In  many  patients  the  use  of  glasses  is  not  suffi- 
cient, but  careful  and  persistent  therapeutic  measures  are  required,  in 
addition  to  the  rest  from  work.  Time  for  recovery  from  the  local, 
secondary  results  of  eye-strain  must  therefore  be  allowed  in  many 
patients  before  it  can  be  said  positively  that  the  eyes  are  not  responsible 
for  the  headache. 

The  subsidence  of  pain  is  not  infrequently  gradual,  the  paroxysms 
being  less  frequent,  not  so  severe,  and  of  shorter  duration,  until  finally 
they  fail  to  return,  or  only  at  long  intervals,  and  then  under  special 
stress  of  circumstances.  The  etiologic  relation  of  these  ocular  condi- 
tions to  disease  of  the  lining  membranes  of  the  bony  sinuses  of  the 
skull  which  lie  contiguous  to  the  orbit  is  too  frequently  overlooked. 
That  sinusitis  is  a  frequent  cause  of  certain  forms  of  headache  is  well 
known,  but  that  the  eyes  participate  in  the  congestion  due  to  blocking 
up  of  the  exits  from  these  sinuses  into  the  nose  has  not  met  with  the 
wide  recognition  its  importance  suggests. 

It  will  be  observed  in  the  foregoing  paragraphs,  therefore,  that  in 
the  study  and  treatment  of  ocular  neuroses  many  complex  conditions 
are  encountered  which  require  a  conservative  application  of  knowledge, 
skill,  and  wide  experience  for  their  successful  management.  It  is  pos- 
sible that  the  ophthalmologist  may  be  prone  to  exaggerate  the  im- 
portance of  ocular  affections  as  a  factor  in  producing  headache  and 
general  nervous  symptoms,  but  this  should  not  cause  surprise  when  an 
analysis  of  one  thousand  private  patients,  applying  consecutively  for 
treatment,  revealed  the  fact  that  approximately  50  per  cent,  of  these 
complained  of  this  symptom,  and  a  considerable  percentage  of  these 
headache  patients  came  by  the  advice  of  their  physicians  and  were  un- 
conscious of  any  ocular  defect.  While  his  daily  experience  might  there- 
fore lead  him  to  overestimate  the  significance  of  eye-strain,  it  has  taught 


NEUROSES  OCCASIONED  BY  EYE-STRAIN         767 

him  that  there  is  great  danger  of  its  importance  being  overlooked  or 
ignored  by  those  laboring  in  other  fields  of  practice. 

There  can  be  no  doubt  as  to  the  fact  that  exaggerated  importance 
has  often  been  given  to  minor  errors  of  refraction  in  patients  whose  eye 
symptoms, — e.g.,  hypersemia  of  the  fundus,  impaired  function,  head- 
ache, undue  sensitiveness  to  light,  etc., — were  due  to  other  causes  than 
eye-strain.  That  these  symptoms  of  eye  disease  are  due  to  some  general 
dyscrasia, — e.g.,  lithaemia,  diabetes,  and  general  blood-vessel  disease, — 
is  well  known  to  all  careful  observers. 

That  very  small  errors  of  refraction  are  frequently  the  cause  of 
headache  and  other  symptoms  is  true,  but  other  sources  of  disturbances 
should  be  carefully  excluded  before  ascribing  the  symptoms  to  eye- 
strain alone.  Many  persons  with  delicately  poised  nervous  systems  are 
nevertheless  greatly  benefited  by  the  correction  of  very  low  degrees  of 
astigmatism,  particularly  if  the  slight  error  of  refraction  is  associated 
wath  ocular  imbalance,  and  they  are  unable  to  abandon  their  glasses 
since  any  attempt  to  do  so  leads  promptly  to  a  return  of  their  headache. 

In  others,  and  they  constitute  a  large  group  of  persons  who  wander 
from  one  consulting  room  to  another,  the  writer  is  convinced  that  the 
glasses  afford  the  temporary  relief  which  they  claim  to  experience,  by  a 
species  of  suggestion,  precisely  as  they  would  be  relieved  for  a  short 
time  by  the  mummeries  of  a  Christian  scientist  or  the  manipulations  of 
an  osteopath.  Many  women  who  have  been  despoiled  of  their  ovaries 
belong  to  this  group,  and  while  they  are  often  unable  to  go  without 
their  correcting  glasses,  never  secure  entire  relief  from  their  headaches, 
which  they  associate  with  any  continued  use  of  their  eyes. 

The  fact  that  many  persons,  even  children,  often  abandon  their 
glasses  after  wearing  them  for  a  few  months,  and  that  without  return 
of  their  headache,  is  not  to  be  accepted  as  proof  that  their  symptoms 
were  in  the  first  place  wrongly  ascribed  to  eye-strain.  Improved 
health,  a  long  summer  vacation,  or  change  of  environment,  as  for 
example,  a  better-lighted  room  or  less  pressing  work,  may  enable  them 
to  bear  the  eye-strain  with  impunity,  but  the  glasses  had  probably  car- 
ried them  over  an  important  interval  and  prevented  permanent  injury. 

In  treating  headache  demonstrably  due  to  eye-strain,  and  for  the 
present  contention  excluding  from  consideration  all  other  possible 
causes  and  associated  conditions,  the  writer  will  now  pass  to  the  discus- 
sion of  the  methods  which  experience  has  taught  to  be  of  greatest 
efficiency. 

In  patients  who  suffer  but  little  pain  but  complain  only  of  blurring 
page  after  protracted  effort  at  near-work,  and  in  whom  the  ophthalmo- 


768  THE    EYE    AND    NERVOUS    SYSTEM 

scope  shows  a  healthy  eye  ground,  it  is  often  sufficient  to  supply  these 
with  so-called  manifest  corrections;  but  in  the  class  of  persons  under 
discussion  in  this  chapter  who,  by  reason  of  eye-strain  are  victims  of 
headache  and  present  the  local  inflammatory  or  irritative  changes  which 
have  been  described,  it  is  necessary  to  use  one  of  the  mydriatic  alkaloids 
to  temporarily  suspend  the  action  of  the  ciliary  muscle,  in  order  to 
determine  with  accuracy  the  static  refraction  of  the  eye. 

The  drugs  usually  selected  for  this  purpose  are  the  sulphates  of 
atropia,  hyoscyamine,  and  duboisine,  and  the  hydrobromate  of  homa- 
tropine.  Others  are  employed,  but  those  named  are  more  commonly 
used.  These  drugs  possess  not  only  the  power  of  dilating  the  pupil  and 
paralyzing  the  ciliary  muscle,  but  are  also  local  anaesthetics  or  analge- 
sics. Their  value  is  twofold.  In  the  first  place  they  secure  to  the 
eyes  a  rest  from  accommodative  efforts,  and  so  relieve  the  eye-strain ; 
at  the  same  time  through  their  soothing  influence  they  aid  materially  in 
relieving  the  nervous  irritability  of  the  eye.  It  has  been  claimed  in  the 
foregoing  pages  that  the  immediate  result  of  the  accommodative  strain 
constantly  required  to  overcome  some  anomaly  of  refraction  is  to  pro- 
duce hyperaemia  and  turgescence  of  the  ocular  tunics,  and  that  this, 
together  with  the  exhaustion  of  the  nerve-centres  involved,  is  in  great 
measure  the  cause  of  pain  in  the  head  and  its  associate  reflex  phenomena. 
The  advantages  therefore  accruing  to  the  patient  from  the  use  of  the 
salts  of  mydriatic  alkaloids  are  obvious.  The  pain  in  the  eyes  and 
head,  the  insomnia,  the  external  redness  and  the  turgescence  of  the 
uveal  tract  rapidly  subside.  These  drugs  should  not,  therefore,  be 
regarded  merely  as  cyclophlegics  or  paralyzers  of  the  accommodation 
only,  but  as  important  and  efficient  therapeutic  agents  in  the  treatment 
of  the  ocular  conditions  associated  with  headache.  To  the  class  of 
patients  under  study  this  contention  is  of  signal  importance,  since  for 
their  relief  approximate  corrections  of  the  errors  of  refraction  are  not 
sufficient.  The  exact  nature  and  degree  of  the  defect  must  be  accurately 
known,  and  this  cannot  be  determined  in  a  pair  of  congested,  irritable 
eyes,  with  a  fluffy  chorioid,  and  exhibiting  undue  sensibility  to  light. 
Before  any  trustworthy  data  as  to  the  static  refraction  can  be  secured, 
the  eye  must  have  time  to  recover  from  these  conditions,  and  recovery  is 
not  only  more  rapid  under  the  use  of  mydriatics  than  any  other  kno^vIl 
means,  but  in  most  cases  they  are  the  sole  means  by  which  satisfactory 
results  may  be  secured.  This  is  particularly  important  where  diseased 
changes  at  the  macula  are  present. 

Many  failures  to  demonstrate  ocular  responsibility  for  headache  are 
due  to  neglect  at  this  point.     So-called  manifest  corrections  of  refrac- 


XEUROSES  OCOASIOIN^ED  BY  EYE-STRAIX         769 

tion  errors  in  this  class  of  patients  are  valueless  for  diagnostic  purposes. 
All  hurrjMip  methods  are  almost  equally  so.  There  is  much  variety 
in  the  opinion  and  practice  of  different  ophthalmic  surgeons  as  to  the 
best  methods  of  employing  the  cyclophlegics.  Many  prefer  to  use  the 
stronger  and  more  persistent  members  of  the  group;  for  example,  solu- 
tions of  the  sulphates  of  atropine  or  hyoscyamine,  while  others  use 
almost  habitually  the  hydrobromate  of  homatropine  and  consider  it 
sufficient.  Homatropine  possesses  the  sole  advantage  of  being  evanes- 
cent in  the  duration  of  its  mydriatic  and  cycloplegic  properties.  The 
writer  demonstrated  its  efficiency,  in  1881,  for  the  determination  of 
errors  of  refraction  in  healthy  eyes,  but  in  headache  patients  the  eyes 
do  not  as  a  rule  belong  to  this  class.  For  these  patients  homatropine 
is  not  efficient  unless,  in  strong  solution  frequently  instilled,  it  is  con- 
tinued day  after  day  until  the  pathologic  conditions  of  the  fundus  oculi 
have  subsided,  in  which  case  it  is  better  to  adopt  at  the  outset  one  of  the 
stronger,  more  efficient,  and  enduring  drugs. 

Then,  too,  after  the  measurements  have  been  made,  the  correcting 
glasses  ordered  and  the  mydriatic  omitted,  another  practical  advantage 
is  secured  by  the  long-continued  influence  of  the  cycloplegic.  The 
glasses  should  be  worn  by  the  patient  as  soon  as  possible  so  that  the 
power  of  accommodation  shall  be  recovered  under  their  use. 

An  important  factor  in  the  disturbance  of  function  in  all  cases  of 
refraction  error  is  the  derangement  of  the  binocular  balance.  That  is 
to  say,  since  the  convergence  and  accommodation  are  under  the  same 
innervation  impulse,  the  existence  of  any  refraction  error  which  in- 
creases or  diminishes  the  amount  of  accommodation  required  to  secure 
a  clear  image  upon  the  fovea  of  each  eye,  must  of  necessity  disturb  the 
relation  of  the  accommodation  impulse  to  that  required  for  convergence 
of  the  two  eyes  to  the  same  point. 

Theoretically,  therefore,  every  hypermetropic  or  myopic  eye  would 
have  as  an  alternative  a  choice  between  a  dim  or  imperfectly  focussed 
image  upon  the  fovea,  or  double  images,  for  the  reason  that  the  eyes 
with  a  convergence  impulse  corresponding  to  the  power  of  accommoda- 
tion required  to  be  exerted  for  the  clear  image,  would  not  be  converged 
to  the  required  point,  but  in  hypermetropia  to  a  too  near  point  and  in 
myopia  to  one  too  remote.  The  fusion  sense  has,  however,  taught  most 
persons  the  trick,  in  hypermetropia,  of  accommodating  within  narrow 
limits  without  convergence,  and  in  myopia  to  converge  to  the  required 
point  without  accommodation,  a  fact  pointed  out  by  the  investigations 
of  Donders  and  designated  by  him  as  "  the  relative  range  and  region  of 

accommodation  and  convergence."     By  this  means  the  alternative  of 
49 


770  THE    EYE    AND    NERVOUS    SYSTEM 

dim  sight  or  double  sight,  in  most  individuals,  is  in  large  measure,  or 
entirely,  averted.  This  result  of  lifelong  experience,  however,  becomes 
a  disturbing  factor  when  correcting  glasses  are  attempted.  As  soon  as 
the  necessity  for  the  accustomed  undue  strain  upon  the  accommodation 
is  removed  by  a  convex  glass,  as  in  the  correction  of  hypermetropia,  it 
becomes  necessary  for  satisfactory  binocular  vision  that  they  should 
unlearn  the  habit  of  a  lifetime  and  exert  the  same  innervation  impulse 
for  both  the  convergence  and  accommodation,  as  in  emmetropia.  The 
great  difficulty  many  persons  have  in  learning  to  wear  a  pair  of  correct- 
ing glasses  grows  out  of  the  fact  that  they  cannot  adjust  themselves 
to  this  new  order  of  things  immediately.  They  do  so,  however,  witli 
much  less  difficulty  if  the  glasses  are  worn  during  the  days  required  for 
the  cycloplegic  to  disajipear.  The  gradually  returning  accommodation 
affords  an  opportunity  for  a  readjustment  of  the  new  relations  between 
convergence  and  accommodation.  Having  these  considerations  in  view, 
the  glasses  for  headache  patients  certainly  should  be  prescribed  at  once 
and  worn  steadily. 

The  careful  study  of  the  binocular  balance  is  a  most  important 
phase  of  the  treatment  of  eye-strain.  It  will  be  found,  if  appropriate 
means  are  employed,  that  a  large  percentage  of  patients  applying  for 
relief  from  eye-strain  s\Tnptoms  present  some  form  of  ocular  imbalance. 
Esophoria  and  exophoria,  or  low  degrees  of  vertical  deviation  are  the 
rule.  Fortunately,  under  the  sufficiently  prolonged  use  of  a  mydriatic, 
many  of  these  deviations  wall  be  found  to  have  disappeared  when  sought 
for  through  accurately  adjusted  glasses  which  correct  the  existing  defect 
of  refraction.  There  is  nevertheless  a  large  percentage  of  them  which 
remain  as  absolute  defects  of  balance  between  the  two  eyes.  Those  which 
disappear  are  related  to  the  muscular  strain  and  anomalous  innervation 
impulses  brought  about  by  the  attempt  to  overcome  the  defects  of  refrac- 
tion in  the  interests  of  clear  vision.  I  have  elsewhere  designated  them 
as  relative  heterophorias.  When  the  strain  is  removed  by  glasses  and 
clear  vision  secured  without  undue  accommodation  effort  the  tendency 
of  the  imbalance  is  to  disappear,  either  spontaneously,  or  by  suitable 
muscular  exercise  with  prisms. 

The  permanent  defects  of  balance  have,  however,  an  anatomical 
basis,  as  already  pointed  out  (see  page  0)  and  require  careful  analysis 
and  separate  treatment. 

The  following  clinical  history  will  serve  to  illustrate  a  somewhat 
numerous  class  of  patients  with  defective  vision  who  apply  for  relief 
from  their  painful  eyes,  headache,  and  a  complex  of  symptoms  which 
can  be  regarded  only  as  ocular  neuroses. 


XEUROSES  OCCASIONED  BY  EYE-STRAIN         771 

Mr.  K.,  aged  26,  applied  for  treatment  in  December,  1899.    He  was 
wearing  the  following  glasses  ordered  by  Saemisch  of  Bonn,  in  1894: 

O.D.  -2.75"  ax.  105° 
O.S.  -1.75«ax.    90° 

with  these  V.^O.D.  6/60,  O.S.  6/9.  He  suffered  from  constant  fronto- 
occipital  headache  which  frequently  culminated  in  violent  attacks  of 
pain  and  general  "  nervousness"  which  drove  him  from  business  and  to 
bed.  He  carried  his  head  tilted  to  the  ri^ht ;  the  eyes  were  red,  unduly 
sensitive  to  light,  and  he  suffered  from  blepharitis  and  recurring  styes. 
The  ophthalmoscope  showed  a  fluffy,  turgid  fundus  in  both  eyes,  and 
in  the  right  a  distorted  disc,  and  a  large  conus  below,  with  concentric, 
crescentic  markings  indicating  successive  fresh  encroachments  upon 
the  chorioid.  The  axis  of  the  conus  was  at  120°.  In  the  left  eye  the 
conus  was  at  the  temporal  border  of  the  nerve  and  beyond  it ;  approach- 
ing the  fovea  there  were  granular  changes  in  the  chorioid.  The  right 
eye  was  on  a  plane  in  front  of  the  left,  the  right  side  of  the  face  was 
larger  than  the  left,  the  zygomatic  arch  being  prominent,  while  on  the 
left  it  was  flat  or  slightly  concave.  The  left  eye  was  nearer  the  bridge 
of  the  nose  than  the  right.  The  ophthalmometer  indicated  in  the  right 
eye  a  corneal  astigmatism  of  4.D.,  the  principal  meridians  being  at  30° 
and  120°  respectively.  In  the  left  eye  an  astigmatism  of  3.D.  the 
meridians  lying  at  170°  and  80°.  After  prolonged  mydriasis  and  rest 
a  careful  measurement  by  objective  and  subjective  methods  resulted 
as  follows : 

O.D.  -    .25'     O  -  4.00  D.  cyl.  ax.  30°  V.  -  ^r^rr 

\  ilj. 

O.S.  -  1.25D.  C  +  2.50  cyl.  ax.        80°  V.  =  ^ 
-^  VI. 

Through  these  correcting  glasses,  carefully  centred,  the  ocular  imbalance 
was  measured  at  6™.  and  revealed  a  left  hyperphoria  of  8°  and  an 
esophoria  of  6°.  These  deviations  would  vary,  the  hyperphoria  be- 
coming greater,  while  the  esophoria  diminished,  or  the  reverse  would 
occur,  in  the  meantime  the  upper  end  of  the  vertical  meridian  of  each 
cornea  rotating  outward  (cyclophoria).  The  sketch  on  page  747,  Fig. 
la;  taken  by  his  hatter,  gives  the  skull  outline.  The  septum,  in  the 
nose,  was  deflected  toward  the  left  side,  and  the  left  middle  turbinate 
was  firmly  wedged  between  it  and  the  bony  wall  of  the  nostril.  The 
roof  of  the  mouth  was  a  deep  cathedral  arch.  This  case  presents  a 
somewhat  exaggerated  picture  of  the  anatomical  basis  for  many  cases 
of  anomalv  of  refraction  associated  with  ocular  imbalance.     His  father 


772  THE    EYE    AND    NERVOUS    SYSTEM 

and  mother  and  a  younger  sister  were  subjects  of  hypermetropic  astig- 
matism, and  headache  was  a  family  trait.  The  eyes  of  this  young  man 
were  evidently  distending,  the  right  having  passed  into  myopic  refrac- 
tion, and  the  left  was  myopic  in  the  vertical  meridian,  the  horizontal 
being  still  hypermetropic, — i.e.,  mixed  astigmatism.  The  measurement 
and  correction  by  glasses  of  the  refraction  error  for  each  eye  was  not 
difficult,  but  the  obvious  difficulties  presented  in  any  effort  to  secure 
comfortable  binocular  vision  illustrates  forcibly  the  complex  problem 
often  presented  in  these  cases  of  ocular  imbalance. 

It  is  by  no  means  always  an  easy  task  to  discern  the  precise  nature 
of  the  muscular  defect,  or  which  of  the  extra-ocular  muscles  is  at  fault, 
or  whether  the  muscle  or  muscles  at  fault  are  so  by  virtue  of  impaired 
innervation,  faulty  development,  or  as  the  result  of  abnormal  attach- 
ment to  the  globe.  The  analysis  of  the  ocular  movements,  however,  is 
often  greatly  facilitated  by  making  the  study  at  6™.  while  the  accom- 
modation is  still  paralyzed.  For  this  purpose  it  is  important  to  employ 
some  fixed  form  of  apparatus  in  which  tlie  lenses  required  to  correct 
the  existing  refraction  error  for  each  eye  can  be  placed  and  carefully 
centred.  The  examination  should  be  made  with  the  eyes  in  the  primary 
position,  using  a  small  point  of  light  placed  at  six  metres  from  the 
patient,  preferably  before  a  black  background,  from  which  all  objects 
which  may  distract  the  attention  are  removed. 

Much  skill,  patience,  and  experience  are  required  in  the  diagnosis 
and  treatment  of  these  cases  of  imbalance  due  to  absolute  muscular 
defects.  In  some  instances,  when  there  is  much  distortion  of  the  walls 
of  the  orbit  and  of  the  peri-orbital  region  of  the  skull,  it  is  probable 
that  no  amount  of  skill  either  by  surgical  interference  or  by  other 
means  can  bring  about  comfortable  binocular  vision. 

One  of  these  patients  after  many  operations  at  the  hands  of  several 
men  of  wide  reputation  came  to  the  writer  with  the  request  that  he 
should  remove  one  of  her  eyes  as  the  only  means  of  promising  any  hope 
of  relief  from  her  distressing  headache  and  nervous  symptoms.  The 
request  was  refused,  but  was  subsequently  granted  by  an  eminent  sur- 
geon in  another  city.  It  is  probable  that  repeated  tenotomies,  advance- 
ment of  tendons,  etc.,  to  which  her  eyes  had  been  subjected  had  done 
harm,  certainly  had  failed  to  afford  relief.  It  is  true,  nevertheless,  that 
there  is  a  large  number  of  patients  who  do  not  secure  relief  from  accu- 
rate correcting  glasses,  whose  continued  trouble  is  unquestionably  occa- 
sioned by  a  defective  balance  between  tbe  eyes  which  is  susceptible  of 
correction  by  surgical  means.  The  power  of  convergence  is  insufficient 
or  it  is  relatively  too  great  for  the  power  of  abduction,  or  in  the  position 


XEUROSES  OCCASIONED  BY  EYE-STRAI:N^         773 

of  rest  one  eye  is  above  the  horizontal  plane,  or  the  other  below  it,  and 
so  binocular  single  vision  is  either  intermittent  or  impossible,  or  is 
maintained  by  virtue  of  constant  muscular  tension  which  causes  head- 
ache, etc.  Experience  has  shown  that  in  the  minor  degrees  of  these 
faulty  muscular  conditions,  the  suitable  use  of  prisms,  combined  with 
the  glasses  required  for  the  correction  of  any  existing  error  of  refrac- 
tion, often  give  practically  complete  relief.  In  the  higher  degrees, 
however,  surgical  interference,  designed  to  restore  ocular  balance,  is 
theoretically  a  correct  procedure  and  its  value  has  been  abundantly  jus- 
tified by  results.  Where  failure  is  met  it  may  often  be  ascribed  to  mis- 
taken diagnosis,  usually  as  to  the  nature  of  the  fault  the  surgeon  has 
sought  to  correct,  or  possibly  to  a  faulty  technique,  or  to  some  unavoid- 
able accident, — e.g.,  excessive  hemorrhage  into  the  capsule  of  Tenon, 
or  to  excessive  inflammatory  reaction  during  repair.  In  two  cases  seen 
in  consultation,  advancement  operations  had  led  to  overcorrection  of  the 
defect  through  the  contraction  of  the  resulting  cicatricial  tissue.  Mis- 
takes may  occur  in  the  selection  of  the  wrong  muscle  for  surgical  pro- 
cedure. For  example,  tenotomy  of  an  apparently  too  strong  muscle 
may  be  performed  wh^n  advancement  of  the  weak  one  should  have  been 
the  operation  of  selection,  or,  the  so-called  secondary  deviations  may 
mislead  the  incautious  operator.  Then,  too,  it  is  not  always  easy  to 
differentiate  between  deviations  due  to  faulty  attachments  of  a  muscle 
tendon  or  a  partial  palsy  of  a  muscle. 

It  is  not  the  intention  in  this  chapter  to  present  a  treatise  on  either 
errors  of  refraction  or  the  heterophorias,  but  to  show  the  responsibility 
oi  these  ocular  abnormalities  in  causing  ocular  neuroses.  In  the  pres- 
ence of  these  defects  of  vision  the  associated  headaches  should  be 
regarded  as  possibh^  due  to  them  until  they  have  been  corrected,  and 
the  resulting  local  pathologic  states  have  been  cured. 

Unfortunately,  but  little  is  actually  known  of  the  real  mechanism 
of  headache.  For  our  present  purpose,  however,  it  is  fortunate  that 
the  mechanism  of  eye-strain,  if  not  of  the  resulting  pain,  is  well  under- 
stood. It  is  probable  that  no  analogous  conditions  are  presented  else- 
where in  the  body.  An  illustration,  however,  of  the  possible  results  of 
eye-strain  is  afforded  by  writer's  cramp.  In  the  hypermetropic  eye, 
for  example,  clear  vision  is  maintained  only  by  the  constant  contraction 
of  the  ciliary  muscle.  When  astigmatism  is  also  present,  or  if  the 
defect  of  refraction  is  heteronymous,  or  of  differing  degrees  between 
the  eyes,  or  if  the  tormenting  effect  of  ocular  imbalance  is  added  to  the 
problem  of  maintaining  clear  vision,  the  difficulties  of  the  struggle  are 
greatly  enhanced  and  the  resulting  exhaustion  or  derangement  of  the 


774  THE    EYE    AXD    KERVOUS    SYSTEM 

nerve-centres  is  more  rapid  and  enduring.  No  muscle  or  group  of 
muscles  in  the  bodj  could  endure  with  impunity  the  exhausting  fatigue 
to  which  the  ciliarv  muscles  and  their  associated  group  of  extra-ocular 
muscles  are  subjected,  by  the  above  described  conditions,  for  the  hours 
ordinarily  required  for  the  purpose  of  vision.  To  this  muscular  fatigue 
must  be  added  the  exhaustion  of  the  nerve-centres  involved  in  the 
innervation  of  the  ocular  muscular  apparatus,  the  resulting  congestion 
of  the  intra-ocular  tunics  and  the  consequent  pressure  upon  the  terminal 
distribution  of  the  ciliary  nerves,  and  possibly  some  subtle  derange- 
ment of  their  nutrition  occasioned  by  the  impaired  circulation  produced 
by  the  increased  intra-ocular  tension.  It  is  plain  that  in  neurasthenics 
or  feeble  individuals,  or  in  industrious  people  who  use  their  eyes  per- 
sistently under  the  added  strain  of  near-work,  the  exhaustion  would  be 
relatively  more  complete  and  enduring  than  in  vigorous  and  relatively 
indolent  individuals.  While  the  latter  might  endure  with  comparative 
impunity  the  imposed  strain,  the  former  groups  of  individuals  would 
be  forced  into  semi-invalidism. 


Pakt  II. 
By  HOWARD  F.  HANSELL,  A.M.,  M.  D. 

GE^EEAL  COIfSIDEEATIOKS :  EPILEPSY;  CHOEEA ;  VEE- 
TIGO;  GASTEIC  DISTUEBANCES ;  SPASMODIC  MOYE- 
MEN^TS  OF  THE  HEAD  OTHEE  THAN  CHOEEIFOEM ; 
GEXEEAL   ASTHENOPIA. 

GENERAL   CONSIDERATIONS. 

The  tendency  of  modern  medicine  is  encouragingly  strong  in  its 
insistence  that  the  diagnosis  and  treatment  of  disease  shall  be  reduced 
to  a  demonstrable  basis.  Dogmatism  and  empiricism  have  been  pushed 
farther  and  farther  into  the  background  of  recent  thought  and  litera- 
ture by  the  revelations  of  the  microscope,  the  exactness  of  physiologic 
research,  and  by  the  accuracy  of  trained  work  and  observation  in  the 
laboratory.  Disease  is  not  general  to  the  system,  but  special  to  some 
part  of  it.  It  has  a  local,  if  not  a  discoverable,  habitation  in  the  human 
economy,  and  a  cause  which  it  is  the  mission  of  science  to  discover  and 
remove.  The  more  general  recognition  of  this  fact  is  bearing  fruit  in 
the  minds  and  labors  of  medical  men.  Each  year  fewer  diseases  are 
classified  by  their  symptoms,  and  more  are  referred  to  the  category 
indicated  by  their  etiology.  As  the  causes  of  disease  are  laid  bare, 
prophylaxis  and  treatment  are  simplified  and  become  more  efficacious. 
Surgery  has  broadened  to  the  inclusion  of  affections  that  were  con- 
sidered, not  many  years  ago,  to  belong  strictly  to  medicine,  while  the 
specialists  are  invading  the  old  fields  of  both  medicine  and  surgery. 
They  are  rightly  accorded  a  more  and  more  extended  domain  as  their 
importance  grows  in  recognition.  This  is  particularly  true  of  ophthal- 
mology. The  well-directed  and  resourceful  labors  of  earlier  workers  in 
this  specialty,  notably  Thomson,  Weir  ]\Iitchell,  Dyer,  Xorris,  Harlan, 
and  younger  men,  trained  in  their  clinics  and  influenced  by  their 
teachings,  have  forced  the  acknowledgment  of  ophthalmology  as  an 
indispensable  adjunct  to  the  practice  of  medicine  and  surgery.  Its 
services  to  the  medical  sciences  is  especially  great  in  the  diagnosis  and 
treatment  of  the  so-called  functional  diseases  oi  the  nervous  system, 
and  organic  affections  of  the  brain  and  abdominal  organs.  This 
specialty  is  also  a  brilliant  contributor  to  the  diagnosis  of  obscure 

775 


776  THE    EYE    AND    NERVOUS    SYSTEM 

organic  lesions  of  the  nervous  system,  circulatory  apparatus  and  di- 
gestive organs.  It  reveals  by  the  ophthalmoscope  pathologic  changes  in 
the  ocular  tissues  themselves,  and  in  many  obscure  conditions  discovers 
the  active  cause  of  a  disease  in  an  error  of  refraction  or  anomaly  of  the 
ocular  muscles.  Any  theory  of  the  origin  of  disordered  function  which 
does  not  embrace  a  consideration  of  the  ocular  apparatus  is  unscientific 
and  open  to  criticism.  There  are  incessant  demands  on  the  peripheral 
organs  of  vision  which  are  in  direct  connection  with  the  central  nervous 
system,  and  the  many  ways  in  which  this  connection  is  provocative  of 
disordered  function  is  a  fact  of  deep  significance  in  the  causation  of 
disease.  The  cause  or  association  is,  moreover,  continually  active  during 
the  life  of  the  individual. 

The  etiological  connection  between  eye-strain  and  reflex  nervous 
disorders  is  well  established  and  rests  upon  the  foundation  of  extensive 
clinical  experience.  In  no  other  branch  of  medicine  is  knowledge  so 
demonstrably  true.  Given  an  optical  defect,  with  a  responsive  or  im- 
pressionable nervous  system,  reflex  phenomena  (the  intensity  and  kind 
of  which  depend  upon  the  character  of  the  optical  defect  and  the  pecu- 
liarities of  the  individual)  will  be  the  invariable  consequence.  The 
degree  of  impressionability  of  the  nervous  system  is  of  vital  importance 
in  the  estimation  of  the  relation  of  ocular  cause  to  systemic  effect.  Not 
every  error  of  refraction  or  anomaly  of  muscular  balance,  however 
severe,  will  give  rise  to  reflex  symptoms.  The  same  degree  of  defect  in 
a  person  of  sensitive  and  nervous  disposition  that  will  originate  dis- 
turbances in  the  functions  of  the  body  of  no  small  import,  will,  in  a 
phlegmatic,  dull,  unresponsive  individual,  give  rise  to  no  reflexes  what- 
ever. It  must  not  be  forgotten,  however,  in  determining  the  nature 
and  severity  of  symptoms,  that  the  power  of  observation  and  expression 
differ  greatly  in  different  persons,  and  one  should  not  be  misled  by  the 
failure  of  a  patient  to  adequately  express  the  feelings  which  demand 
the  physician's  attention.  It  may  be  necessary  for  the  medical  attendant 
to  elaborate  the  facts  without  much  reference  to  the  ill-defined  and 
misleading  statements  of  the  patient. 

The  exhaustion  or  irritability  of  the  nervous  system  consequent  upon 
eye-strain  is  indisputable  and  manifold  in  its  manifestations.  In 
order  thoroughly  to  comprehend  ocular  participation  in  the  etiology  of 
the  disturbances  of  the  functions  of  the  body  indicative  of  exhausted  or 
perverted  nerve  action,  the  visual  apparatus  should  be  considered,  (1) 
in  relation  to  the  function  of  accommodation  in  hyperopia,  myopia,  and 
astigmatism  ;  (2)  in  the  gradual  failure  of  accommodation  in  advancing 
age;  (3)  in  the  associated  action  of  the  accommodative  and  extrinsic 


NEUROSES  OCCASIOXED  BY  EYE-STRAIN         777 

ocular  muscles;  and  in  (4)  the  connection  of  the  peripheral  organs 
of  vision  with  the  central  and  sympathetic  nervous  systems. 

1.  Emmetropia  and  equilibrium  of  the  ocular  muscles  present  ideal 
conditions.  The  emmetrope  receives  on  his  retina  clear,  defined  images 
of  distant  objects  (6  m.  or  more),  with  minimum  effort  of  accommoda- 
tion and  contraction  of  the  extraocular  muscles  (equilibrium  and 
binocular  fixation).  For  objects  nearer  than  infinity  and  to  within  33 
cm.  (the  reading  distance)  of  the  eyes,  the  refracting  power  of  each  eye 
is  increased  by  the  uniform,  gradual  or  sudden  contraction  of  the  ciliary 
muscles  and  the  simultaneous  contraction  of  the  muscles  of  convergence, 
the  equivalent  relaxation  of  the  muscles  of  divergence  and  the  mainten- 
ance of  the  same  visual  horizontal  plane  for  both  eyes  by  equal  tension 
of  the  muscles  of  supraduction  and  infraduction. 

That  equilibrium  and  physiologic  relation  between  the  action  of 
intraocular  and  extraocular  muscles  shall  be  originated  and  sustained, 
nerve  stimulation  and  muscular  response  must  be  normal.  Such  a  com- 
bination implies  a  healthy  condition  of  the  centres  of  vision,  an  unin- 
terrupted and  unimpaired  function  of  the  efferent  and  afferent  nerves 
and  that  their  peripheral  distribution  is  normal.  The  separate  muscles 
of  both  eyes  do  not  co-ordinate  perfectly  unless  they  have  normal  rela- 
tiens  with  each  other  and  with  the  eyeballs.  The  development  of  ocular 
reflex  neuroses  in  emmetropia  and  extraocular  muscle  balance  must  be 
referred  to  an  extraocular  cause,  such  as  the  weakened  or  deficient  action 
of  the  central  nervous  system,  or  of  the  circulatory  apparatus,  as  in  con- 
valescence from  exhausting  disease  or  confinement,  affections  of  the 
organs  of  the  chest  or  abdominal  cavity,  prolonged  near  use  of  the 
eyes  among  unfavorable  surroundings,  the  gouty  or  rheumatic  diathesis, 
venereal  excess,  overindulgence  in  stimulants  or  narcotics,  or  other 
source  by  which  the  visual  apparatus  may  be  deranged.  The  remedy  is 
not  far  to  seek.  The  restoration  to  health  of  the  diseased  organs,  the 
avoidance  of  all  excesses,  and  the  use  of  the  eyes  under  favorable  con- 
ditions and  for  a  reasonable  length  of  time,  will  be  followed  by  a  gradual 
return  to  normal  condition. 

In  the  presence  of  an  error  of  refraction,  the  physiologic  relations 
between  the  peripheral  organ  of  vision,  including  its  extrinsic  muscles 
and  the  cerebral  centres,  are  disturbed.  For  example,  in  hyperopia 
abnormal  contraction  of  the  ciliary  muscle  is  required  for  clear  vision. 
Thus  the  muscles  of  convergence  receive  an  excess  of  stimulation,  those 
of  divergence  a  stimulation  so  modified  that  they  may  yield  to  the 
demand  of  convergence,  and  those  of  elevation  and  depression  a  degree 
of  innervation  corresponding  to  their  anatomical   relations  with  the 


778  THE    EYE    AND    NERVOUS    SYSTEM 

muscles  of  convergence  and  divergence.  The  primary  cause — hyperopia 
— tlius  initiates  a  series  of  morbid  phenomena,  abnormal  muscular  action 
and  centrifugal  impulse  that  eventuates  in  a  deranged  ocular  function 
which,  if  not  corrected,  involves  the  general  health  and  nervous  organiza- 
tion, exhibited  by  definite,  pronounced,  vague,  or  bizarre  reflexes.  The 
eye-strain  in  myopia,  is,  conversely,  due  to  the  unassociated  action  of 
convergence  and  accommodation,  the  tendency  to  monocular  vision  and 
the  intense  effort  to  converge  and  maintain  binocular  singular  vision 
for  all  distances.  The  eye-strain  in  astigmatism  is  that  of  hyperopia  or 
myopia  in  conjunction  with  the  unequal  contraction,  hence  innervation 
of  the  ciliary  muscle  in  the  constant  effort  to  obtain  clearly  defined 
retinal  images.     (See  Chapter  II.) 

The  ill  effects  of  the  use  of  the  eyes  in  all  forms  of  ametropia  are 
referable  to  the  eyes  as  well  as  to  the  muscles  which  control  their  move- 
ments. In  most  cases  an  error  of  refraction  is  the  primary  cause,  and 
muscular  anomalies  are  secondary  and  dependent  affections.  In  a  small 
minority  of  cases,  however,  as  in  anatomical  peculiarities  of  muscular 
insertion,  malformations  of  the  bones  of  the  orbit  or  skull,  or  in  muscu- 
lar weakness  or  paralysis,  eye-strain  may  exist  as  the  result  primarily 
of  muscular  incoordination.  These  conditions,  irrespective  of  the  re- 
fraction of  the  eyes,  react  upon  the  central  nervous  system  and  occasion 
manifestations  of  disordered  function  exactly  as  though  the  muscular 
anomaly  were  secondary  to  ametropia. 

2.  The  symptoms  of  uncorrected  presbyopia,  or  the  gradual  failure 
of  accommodation  in  advancing  years,  are  indistinct  near  vision,  reces- 
sion of  the  reading  distance,  the  need  of  stronger  light  and  larger  type, 
headache,  and  other  forms  of  asthenopia.  To  these  increasing  disabili- 
ties may  be  added,  in  exceptional  cases,  any  or  all  forms  of  ocular  re- 
flexes common  to  ametropia  and  muscular  imbalance.  The  direct  and 
indirect  symptoms  are  referable  to  the  strain  of  an  accommodative 
power  weakened  by  lowered  contractility  of  the  ciliary  muscle  as  a 
part  of  the  general  muscular  loss  of  tone  coincident  with  age,  and  to  a 
gradual  stiffening  and  loss  of  elasticity  of  the  crystalline  lens.  Such  an 
eye  is  no  longer  able  to  adapt  itself  quickly,  if  at  all,  to  the  demands  of 
vision  at  close  range,  and  the  nerve  stimulus  to  the  ciliary  muscle,  prob- 
ably not  lessened  by  age,  meets  with  insufficient  response.  Hence,  vision 
is  blurred  and  cannot  be  cleared  except  by  the  emplo^^nent  of  convex 
lenses. 

3.  There  is  an  interdependence  of  the  functions  of  the  intraocular 
and  extraocular  muscles.  Every  accomplished  act  of  accommodation, 
and  every  rotation  of  the  eyes  in  any  direction,  is  consummated  by  the 


NEUROSES  OCCASIONED  BY  EYE-STRAIN         779 

co-ordiuated  contraction  or  relaxation  of  twelve  extraocular  muscles. 
Balance  or  equilibrium  presupposes  normal  action  of  each  of  the  muscles 
individually  and  all  of  them  in  combination,  and  equivalent  and  asso- 
ciated response  of  the  ciliary  muscles  and  irides.  An  abnormal  demand 
on  the  accommodation,  too  great  or  too  small,  is  responsible  for  hetero- 
phoria  or  heterotropia,  both  lateral  and  vertical.  The  tendency  of  the 
eyes  to  rotate  inwardly  (insufficiency  of  the  externi),  or  convergence  of 
the  visual  axes,  is  associated  with  excessive  accommodative  effort,  while 
the  tendency  to  outward  rotation  (insufficiency  of  the  interni)  in- 
volves little  or  no  accommodative  effort.  Both  inward  and  outward 
tendencies  to  deviation  are  combined  with  vertical  insufficiencies  in 
every  instance,  as  all  muscles  governed  by  the  same  nerve  respond  to 
the  stimulation,  and  individual  muscles,  or  sets  of  muscles,  must  act 
jointly  with  all  the  extraocular  muscles  in  contraction  and  relaxation. 
This  close  relationship  of  the  ciliary  and  the  extraocular  muscles,  and 
of  the  extraocular  muscles  to  the  nerves  that  govern  them,  is  invariably 
deranged  by  errors  of  refraction.  Ametropia,  therefore,  is  responsible 
for  accommodative  strain  and  muscular  asthenopia,  the  effects  of  which 
are  by  no  means  limited  to  the  eyes,  or  to  the  head,  but  are  reflected  to 
the  various  organs  of  the  body  in  such  way  as  to  interfere  with  their 
nutrition  and  function.  The  use  of  the  eyes  under  these  conditions  of 
faulty  focussing,  or  faulty  rotation,  in  neurotic  individuals,  gives  rise 
to  local  discomfort  and  reflex  phenomena  of  various  kinds. 

4.  We  have  seen  how  errors  of  refraction  and  muscular  anomalies 
may  be  the  cause  of  asthenopia  and  reflex  neuroses.  Morbid  states  of 
the  central  and  sympathetic  nervous  systems,  on  the  other  hand,  are  so 
well  known  that  it  is  only  necessary  to  allude  to  them  as  one  of  the 
factors  in  the  development  of  ocular  symptoms.  Intracranial  affections, 
for  example,  by  pressure  upon  a  nerve  supplying  an  eye  muscle,  or 
involvement  of  the  optic  nerve  directly  or  indirectly,  spinal  disease, 
affections  of  the  circulatory  apparatus  or  of  the  abdominal  organs,  will 
impair  or  destroy  binocular  vision  or  sight  itself.  The  so-called  con- 
stitutional dyscrasias,  syphilis,  tuberculosis,  rheumatism,  scrofula,  dia- 
betes, anemia,  etc.,  directly  and  markedly  affect  the  organs  of  vision  by 
inflammatory  outbreaks  and  indirectly  by  vitiating  the  tone  and  health 
of  the  component  tissues  of  the  eye.  The  demands  of  modern  business 
and  active  competition  in  all  lines  of  work,  the  energy  and  unremitting 
industry  essential  to  success, — in  a  word,  the  struggle  for  existence, — 
make  too  many  and  great  and  uninterrupted  calls  upon  the  vital  forces  of 
the  individual.  A  little  more  time  for  relaxation,  a  little  more  freedom 
from  care  and  responsibility  in  the  great,  and  apparently  necessary. 


780  THE    EYE    AND    NERVOUS    SYSTEM 

light  for  individual  supremacy  in  the  daily  affairs  of  business  and  pro- 
fessional life,  would  give  much-abused  nature  the  opportunity  to  assert 
her  rehabilitating  influences  and  thus  enable  the  individual  to  preserve, 
unimpaired,  his  faculties  and  powers  of  endurance  almost  to  the  end 
of  extreme  old  age.  It  is  a  noteworthy  fact  that  in  our  strenuous  Ameri- 
can life  the  eyes  suffer  first  and  most  in  the  general  break-down  of  the 
system. 

Low  defects  of  refraction  and  comparatively  insignificant  muscular 
insufficiencies  which,  under  healthier  and  more  sanitary  conditions 
would  have  remained  in  harmless  abeyance,  are  provocative  of  ocular 
distress,  headache,  and  other  symptoms  of  a  serious  and  more  widely 
disseminated  nature. 

An  editorial  writer  in  American  Medicine,  February  20,  1904, 
states  the  same  thought  in  different  language :  "  One  Overlooked  Factor 
in  the  Increase  of  Insanity.  The  new  element  in  modern  civilization 
which  is  not  recognized  and  which  is  known  to  be  a  profound  source 
of  many  derangements  is  school  life  and  reading.  The  compulsory  or 
chosen  occupation  of  reading  and  near  use  of  the  eyes  in  a  thousand  occu- 
pations has  been  the  dominant  element  in  the  life  of  the  city  dweller. 
Printing,  books,  schools,  libraries,  cheap  books,  newspapers,  and  handi- 
craft occupations  have  appeared  and  compel  a  physiologic  or  unphysio- 
logic  activity  which,  whether  harmful  or  not,  is  a  new  function,  and 
one  to  which  in  evolution  neither  the  mind  nor  the  body  has  been 
habited,  and  especially  to  which  the  highest,  most  delicate  and  most 
used  sense  organ  has  never  been  bred.  ^loreover,  this  sense  organ  ia 
demonstrably  imperfect  in  a  large  proportion  of  all  persons,  and  the 
action  of  the  malformed  eye,  as  has  been  demonstrated  by  science,  and 
by  thousands  of  clinical  cases,  begets  disturbances  of  eye,  of  cerebral 
action,  of  feelings,  and  of  nutrition.  Malfunction  is  the  beginning  of 
all  disease,  and  of  many  it  is  the  middle  and  end.  Suicide,  it  has  been 
found,  is  exactly  in  proportion  to  the  number  of  hours  of  school  life 
and  study  demanded  in  the  country  concerned.  To  this  suggestive  fact 
is  now  added  the  demonstration  that  insanity  is  almost  precisely  in  the 
same  proportion.  When,  lastly,  it  is  observed  that  the  use  of  the  eye 
demanded  by  the  civilization  of  the  last  century  or  two  is  one  that  its 
evolution  has  never  j)rovided  for,  either  in  structure  or  in  physiology, 
one  gets  a  glirajise  of  morbid  mental  action  in  the  making.  When  it 
is  found  that  the  insane  and  criminal  have  an  astonishingly  high  pro- 
portion of  high  optical  defects,  the  suggestion  grows  to  conviction  that 
the  morbid  action  of  the  eye  and  its  resultant  cerebral  functions  are 
considerable  causes  of  the  increase  of  insanity." 


NEUROSES  OCCASIONED  BY  EYE-STRAIN         781 

But  the  eyes  are  not  peculiar  as  disturbing  agents  of  the  human 
body.  Physical  weakness  in  one  part  of  the  body  interferes  with  the 
functions  of  the  entire  organization.  Moreover,  asthenopia,  whether 
refractive  or  muscular,  reacts  upon  the  weakened  body,  and  aggravates 
the  symptoms  of  nervous  exhaustion  by  still  further  -reducing  assimila- 
tion of  food  and  constructive  metamorphosis.  In  this  sense,  eye-strain 
is  a  cause  as  well  as  an  effect.  In  health,  on  the  other  hand,  ocular 
defects  of  considerable  degree  are  suppressed,  have  no  deleterious  effects 
upon  the  nervous  system,  and  are  recognized  only  by  a  lowering  of  the 
visual  acuity  from  failure  of  accommodation  or  from  appreciable  loss 
of  muscular  co-ordination. 

Eye-strain,  productive  of  local  symptoms  and  reflex  neuroses,  is  thus 
accommodative  and  muscular,  the  former  referring  to  overaction,  and 
the  latter  to  imbalance,  or  unequal  tension  of  the  muscles  which  govern 
the  eyes  in  their  rotations  in  the  orbital  cavities.  Manifestly,  one-eyed 
individuals  are  excluded  from  the  second  class.  This  fact  applies  to 
those  who  literally  have  but  one  eye,  but  also  to  persons  who  do  not, 
under  any  circumstances,  have  binocular  single  vision.  Muscular  as- 
thenopia is  also  absent  in  paralytic  or  functional  squint.  This  is  ob- 
viously true,  as  the  essential  factor  in  the  causation  of  ocular  pain  or 
distress  is  the  maintenance  of  binocular  fixation  under  the  stress  of 
muscular  imbalance  of  a  degree  not  incompatible  with  the  simultaneous 
use  of  both  eyes.  The  indirect  cause  of  asthenopia  is  the  tendency  to 
turn  the  visual  axes  away  from  parallelism  ;  the  direct  cause,  the  tension 
of  the  opposing  set  of  muscles,  wdiose  function  it  is  to  resist  the  ten- 
dency. For  instance,  esophoria,  the  tendency  to  cross  the  visual  axes, 
creates  an  abnormal  effort  on  the  part  of  the  abducting  muscles  in  order 
to  preserve  binocular  single  vision,  and  it  is  this  extra  innervation,  and 
extra  muscular  response,  which  is  the  source  of  the  distress.  And  this 
is  true  whether  the  muscles  of  convergence  are  relatively  strong  or  those 
of  divergence  relatively  weak. 

As  already  stated,  errors  of  refraction  and  muscular  anomalies  are 
the  ocular  causes  of  accommodative  asthenopia ;  of  the  former,  hyper- 
opia and  hyperopic  astigmatism  are  of  the  most  frequent  occurrence; 
of  the  latter,  hyperphoria  is  most  common.  Among  the  general  or  con- 
stitutional causes  may  be  mentioned  neurasthenia,  heredity,  nervous 
affections,  debility  of  any  nature,  sexual  excess,  gout,  rheumatism,  or 
any  condition  of  the  system  in  w^hich  the  power  of  endurance  is  reduced 
below  the  healthy  standard.  Frequent  contributory  causes  are  constant 
application  of  the  eyes  in  close  work,  prolonged  reading  of  fine  type, 
too  much  or  too  little  light,  fine  sewing  and  embroidery,  and  reading  in 


782  THE    EYE    AND    NERVOUS    SYSTEM 

moving  vehicles,  or  with  the  head  in  a  position  either  on  a  line  with  or 
below  the  level  of  the  trunk. 

Inasmuch  as  a  pure  horizontal  or  vertical  tendency  of  the  visual  axes 
may  not  exist  [excepting  in  rare  abnormal  tensions  and  relaxations 
originating  in  anatomical  abnormalities,  as  a  separate  and  distinct  con- 
dition, uncomplicated  with  an  upward  (in  predominating  lateral  ten- 
dency) or  a  horizontal  (in  vertical)  tendency],  we  are  forced  to  deal 
with  an  obliquely  directed  tendency  to  deviation  of  the  visual  lines.  A 
consideration  of  the  heterophorias  as  being  the  dominant  causes  of 
asthenopia  and  reflex  neuroses,  necessarily  involves  a  study  of  the 
relative  strengths  of  adduction,  abduction,  and  sursumduction  for  ob- 
jects at  all  distances.  If  in  emmetropia  adduction  equals,  approxi- 
mately, 24°  and  abduction  2°,  esophoria  would  be  due  to  insufficiency  of 
the  muscles  of  divergence.  If  right  (supraduction)  =5°  (left  infraduc- 
tion)  and  left  supraduction  =  3°  (right  infraduction)  the  elevators  of 
the  right  eye  or  the  depressors  of  the  left  eye  would  be  at  fault.  If 
right  supraduction  (left  infraduction)  =3°  and  left  supraduction 
(right  infraduction)  =^1°  the  fault  would  lie  with  the  elevators  of  the 
left  eye,  or  depressors  of  the  right.  For  measuring  the  limits  of  rota- 
tion of  the  cornea  in  the  horizontal  and  vertical  meridians,  the  clino- 
scope  of  Stevens  is  valuable.  By  its  reading,  in  degrees  of  the  circle,  the 
excursions  of  the  eye  may  be  readily  compared.  In  addition  to  the  alter- 
nate vertical  tendency  already  mentioned,  cases  are  met  with  in  which  the 
vertical  inclinations  are  present  and  of  the  same  direction  in  both  eyes. 
Anaphoria  is  the  name  given  to  designate  upward,  and  kataphoria 
downward,  abnormal  tensions  of  both  eyes.  Such  conditions  are  the 
result  of  pathologic  states  of  the  muscles  or  nerves  supplying  them, 
or  of  some  obscure  unknown  disease.  They  are  not  satisfactorily  ex- 
j)lained  by  errors  of  refractioji  or  by  the  muscular  contractions  induced 
by  the  effort  to  see  clearly.  It  is  of  vital  importance  to  know  that  such 
conditions  exist,  and  that  they  may  cause  eye-strain  and  reflex 
anomalies. 

EPILEPSY. 

There  is  no  uniformity  among  authors  in  their  definition  of  epilepsy. 
It  is  hardly  possible  to  define  it  exactly.  Epilepsy  is  a  symptom  ex- 
pressive of  an  organic  or  functional  derangement  of  the  nervous  system. 
It  is  not  a  disease  per  se,  etiologically  or  pathologically,  but  a  symptom 
complex.  One  of  the  tersest,  most  accurate,  and  comprehensive  de- 
scriptions of  the  commonly  accepted  meanings  of  the  word  is  given  nega- 
tively in  Gower's  "  Diseases  of  the  Nervous  System,"  published  in  1888. 
"  The  term  epilepsy,"  he  writes  (page  1078),  "  is  applied  to  a  disease 


LEUKOSES  OCCASIOXED  BY  EYE-STRAIN         783 

in  which  there  are  convulsions  of  a  certain  type,  or  sudden  loss  or  im- 
pairment of  consciousness,  but  in  which  the  convulsions  are  not  directly 
due  to  active  organic  brain  disease,  to  reflex  irritation,  or  to  abnormal 
blood  states,  and  in  which  the  loss  of  consciousness  is  not  due  to  primary 
failure  of  the  heart's  action."  Subsequent  writers  have  not  improved 
this  definition.  H.  C.  Wood  defines  epilepsy  ^  as  "  A  disease  of  un- 
known pathology  in  which,  at  irregular  intervals  and  without  obvious 
existing  causes,  an  abnormal  disturbance  of  nerve  force  occurs,  in  most 
cases  accompanied  with  loss  of  consciousness,  and  very  frequently  with 
convulsive  disturbance."  Landon  Carter  Gray  ^  admits  that  "  much 
confusion  has  been  injected  into  the  subject  by  the  failure  to  recognize 
that  epilepsy  is  only  a  symptom,  just  as  is  fever  or  cough,  and  that  in 
every  case,  the  question  of  diagnosis  is  resolved  into  the  further  one  of 
what  the  epilepsy  is  a  symptom  of."  Other  authors  might  be  quoted 
without  adding  useful  evidence  to  the  universally  admitted  opinion  that 
the  so-called  disease,  epilepsy,  is  the  accompaniment  of  not  one  but  many 
affections,  in  some  of  which  organic  changes  in  the  nervous  system  are 
found  after  death,  while  in  others  failure  to  recognize  that  any  of  the 
tissue  is  diseased  is  the  only  result  of  microscopic  examination. 

Epilepsy,  as  a  symptom  of  disease,  has  no  pathology.  Authorities 
are  conflicting  and  inconsistent  as  to  this  question.  It  is,  for  instance, 
classified  as  a  functional  neurosis,  and  yet  pages  are  devoted  to  the  dis- 
cussion of  its  pathology.  Local  and  general  convulsions  are  due,  in 
many  cases,  to  organic  changes  in  the  brain  or  spinal  cord,  and  are  un- 
scientifically classified  as  functional  affections,  when  they  should  be 
designated,  for  example,  as  "  epileptic  convulsions  in  hemiplegia." 
Exactly  identical  convulsions  may  appear  as  reflex  symptoms  of  some 
peripheral  lesion.  A  wider  recognition  of  these  facts  would  lead  to  a 
more  tenable  classification  of  epilepsy  than  that  usually  given,  and 
would  tend  eventually  to  eliminate  such  objectionable  adjectives  as 
idiopathic.  The  subdivision  of  the  subject  would  then  be  "  Epilepsy 
from  organic  disease  of  the  nervous  system"  and  "  Epilepsy  from 
peripheral  irritation,"  although  the  exciting  cause  might  remain  un- 
known in  many  cases. 

The  testimony  of  too  many  trustworthy  clinicians  has  been  pub- 
lished in  confirmation  of  the  statement  that  epileptic  convulsions  may 
be  due  to  affections  of  other  organs  than  the  brain  and  spinal  cord,  to 
permit  the  causative  relation  of  distant  lesions  to  the  development  of 
the  affection  to  be  ignored.     It  must  be  admitted,  however,  that  some 

'  Pepper's  System,  Theory,  and  Pfactice  of  Medicine,  1893. 

^  Nervous  Diseases,  by  American  Authors,  edited  by  F.  X.  Dercum,  1895. 


784  THE    EYE    AND    NERVOUS    SYSTEM 

other  factor,  aside  from  the  peripheral  lesion,  must  be  active.  In 
considering  the  nature  of  the  factor,  we  must  assume  that  some  state  or 
condition  of  the  nervous  system  makes  it  peculiarly  receptive,  impres- 
sionable, and  responsive.  And  the  question  arises,  What  are  the  pre- 
disposing and  underlying  causes  of  epilepsy  ?  We  confess  our  ignor- 
ance here,  as  we  are  forced  to  confess  it  in  many  other  affections,  and 
expose  our  lack  of  exact  knowledge  by  the  use  and  abuse  of  such 
names  as  heredity,  susceptibility,  idiosyncrasy.  We  cannot  explain  by 
the  results  of  pathologic  examination  or  post-mortem  studies  why  an 
old  cicatrix  should  cause  epileptic  attacks  in  one  individual  and  not 
another,  or  why  intestinal  worms  will  cause  corneal  ulcer  in  one  child 
and  not  in  all  children,  without  having  recourse  to  the  intangible  but 
none  the  less  cogent  influence  of  personal  susceptibility,  an  indefinable 
quality,  inherent  and  peculiar  to  the  individual. 

Etiology. — In  some  respects  the  theories  of  the  diagnosis  and  treat- 
ment of  functional  neurosis  need  reconstruction.  It  can  certainly  not 
be  claimed  by  any  enlightened  medical  man,  how^ever  earnest  and 
enthusiastic  he  may  be,  that  all  or  even  a  large  proportion  of  "  idio- 
pathic" cases  are  the  reflex  result  of  perverted  function  of  some  distant 
organ.  On  the  other  hand,  in  view  of  the  authoritative  statements  and 
experience  of  the  general  practitioner,  it  cannot  be  doubted  that  at 
least  a  minority  of  such  affections  are  reflex  in  origin.  H.  C.  Wood  * 
divides  epilepsy  into  organic,  toxemic,  Jacksonian,  idiopathic,  and 
reflex,  and  gives  as  causes,  wounds  of  the  head  or  other  portion  of  the 
body,  astigmatism  or  other  imperfection  of  the  eyes,  diseases  and  mal- 
formations of  the  nasal  cavity,  carious  teeth,  retained  milk  teeth,  aural 
diseases,  adherent  prepuce  or  other  irritation  of  the  genital  organs,  and 
intestinal  worms."  W.  B.  Pritchard  ^  gives  as  causes  of  reflex  epilepsy, 
the  existence  of  which,  he  says,  is  somewhat  widely  questioned,  phimo- 
sis, adherent  prepuce  or  clitoris,  stenosis  of  uterine  os,  nerve  cicatrices, 
or  surgical  disease  of  the  limbs  or  joints  implicating  nerve  trunks, 
carious  teeth,  eye-strain  (esophoria,  exophoria),  obstructive  diseases  of 
the  nasal  passages,  aural  disease,  etc.,  etc.  Herter  and  Smith,^  in 
considering  the  etiology  of  epilepsy,  mention  as  one  of  the  most  im- 
portant causes  "  putrefactive  changes  in  the  intestinal  contents,  toxic 
substances  produced  in  the  intestines"  (reflex  epilepsy).     Stoewer  ®  says 

'  Pepper's  Text-Book. 
*  Sajous's  Annual,  vol.  iii.,  1899. 

'New  York  Medical  Journal,  August  and  September,  1892. 

'  Augenkrankheiten  als  Ursache  der  Epilepsia,  Klin.  Monattib.  f.  Augenh.,  30, 
1896. 


IsEUKOSES  OGCASIOXED  BY  EYE-STRAIX         T85 

"  most  cases  are  laid  to  the  cerebrum,  while  for  other  cases,  certain  irri- 
tations of  peripheral  nerves  are  necessary  for  outbreaks  of  an  attack," 
and  "  many  of  the  last  reflex  class  are  cured  by  the  removal  of  painful 
scars,  irritation  from  nose  and  ear."  P.  F.  Barbour  "  reports  the  case  of 
a  male,  married,  who  had  epileptic  seizures  since  1892.  He  found  a 
marked  increase  of  indican  in  the  urine  and  dilatation  of  the  stomach. 
Hence  he  inferred  that  the  trouble  arose  from  intestinal  indigestion  and 
constipation.     Treatment  for  these  conditions  relieved  the  epilepsy. 

These  quotations  and  abstracts,  and  more  might  readily  be  added, 
arc  sufficient  to  prove  that  epileptic  attacks  may  be  reflex  in  origin,  and 
that  removal  of  the  source  of  irritation  will  re-establish  normal  function 
of  the  nervous  system.  It  is,  therefore,  logical  to  assume,  and  the 
assumption  has  been  abundantly  verified,  that  reflex  epilepsy  may  be 
the  result  of  eye-strain,  accommodative  and  muscular.  The  relation 
of  the  function  of  the  ciliary  muscles  to  the  extraocular  muscles,  has 
already  been  alluded  to.  Most  cases  of  accommodative  asthenopia  are 
also  cases  of  muscular  asthenopia.  Therefore,  both  causes  may  be 
active  in  the  same  individual,  and  should  be  taken  into  account  as 
factors.  The  relative  importance,  in  the  etiology  of  nervous  affections, 
of  refraction  and  muscular  anomalies,  is  difficult  to  determine.  Some 
authors  ascribe  the  whole  responsibility  to  refractive  errors,  others  to 
want  of  equilibrium  of  the  extraocular  muscles,  and  not  a  few  writers 
utterly  ignore  the  eye  and  its  muscles.  Strohmayer,*  for  instance,  dips 
deeply  into  the  consideration  of  etiology  and  treatment  by  medical  and 
hygienic  measures.  The  omission  in  a  systematic  monograph  of  such 
recent  date  is  surprising,  and  the  only  inference,  of  course,  is  that 
the  writer  does  not  believe  in  eye-strain  as  a  cause  of  epilepsy.  Landon 
Carter  Gray  ^  makes  a  distinction  between  the  two  sources  of  eye- 
strain. "  The  removal  of  insufficiencies  of  the  ocular  muscles  is,  in 
my  opinion,  quite  useless,  but  marked  errors  of  refraction  should  be 
remedied."  The  reasonableness  of  this  decision,  half-hearted  though  it 
is,  may  well  be  questioned.  The  dual  cause  is  so  intimately  and  so 
inseparably  bound  together,  that  a  separation  or  elimination  of  either 
is  not  only  unscientific  but  inconsistent  with  intelligent  criticism. 

M.  Allen  Starr  ^  ^  believes  that  epilepsy  is  an  organic  disease.  He 
bases  his  opinion  on  the  study  of  4000  cases  and  his  researches  are 

'  Medical  Times,  February,  1895. 
*  Die  Epilepsie  im  Kindesalter,  1892. 
'  Nervous  Diseases,  by  American  Authors. 

'"  New  York  Medical  Journal  and  Philadelphia  Medical  Journal,  January  30, 
1904. 

50 


786  THE    EYE    AND    NERVOUS    SYSTEM 

certainly  worthy  of  careful  attention.  One  hundred  and  ninety-two  of 
the  4000  cases  exhibited  positive  signs  of  non-development  of  the  brain, 
68  per  cent.,  signs  of  epilepsy  before  twenty  years  of  age,  the  period  of 
brain  development.  The  most  frequent  cause  was  heredity,  and  the 
next  in  frequency,  trauma  of  the  head.  That  the  latter  was  a  more 
potent  cause  than  wounds  of  other  parts  of  the  body,  was  well  shown  by 
the  statistics  of  the  Franco-Prussian  war.  These  were  conclusive.  Out 
of  8889  soldiers  wounded  in  the  head,  46  became  epileptic;  whereas, 
out  of  77,463  persons  wounded  elsewhere,  only  17  became  epileptic. 
Fright  was  undoubtedly  a  cause,  as  119  of  the  patients  tabulated  had  the 
first  fit  after  a  fright. 

C.  L.  Dana,  in  discussing  Starr's  paper,  said,  "At  least  ten  years 
ago  he  had  spoken  of  epilepsy  as  a  progressive  degenerative  disease  of 
the  brain,  but,  as  we  did  not  yet  know  always  what  this  degeneration 
was  or  where  it  was,  and  could  not  give  a  morphological  description  of 
the  diseased  cells,  it  was  probably  wise  still  to  describe  epilepsy  as  a 
disease  without  definite  organic  basis.  lie  also  doubted  if  the  term 
disease  was  the  proper  one  to  use  in  connection  with  epilepsy.  There 
was  certainly  an  underlying  organic  degeneration,  but  it  seemed  to  him 
that  in  many  cases  it  was  rather  a  morbid  condition  than  a  pathologic 
process,  rather  a  teratological  defect  than  an  actual  disease.  So  far 
as  we  know,  it  was  probable  that  the  most  important  factor  underlying 
epilepsy  was  the  tendency  of  large  groups  of  nerve-cells  to  undergo  a 
rather  progressive  degenerative  process.  With  regard  to  the  incurability 
of  epilepsy,  he  expressed  the  opinion  that  the  stand  taken  by  Starr  was 
not  only  incorrect  but  discouraging.  He  absolutely  knew  of  cases  in 
which  epilepsy  had  been  cured.  He  knew  personally  of  a  young  man  of 
seventeen,  who  had  nocturnal  epilepsy  for  one  or  two  years,  the  attacks 
coming  as  often  as  two  or  three  times  a  month.  He  had  seen  the  attacks 
himself  and  they  were  characteristic.  Under  the  bromide  treatment 
this  man  recovered  and  was  now  forty-five  years  of  age,  and  had  never 
had  an  attack  since  completing  the  treatment.  There  was  now  none 
of  the  interconvulsive  symptoms  of  epilepsy,  and  this  disease  should  be 
looked  upon  as  cured,  just  as  much  as  pneumonia  should  be  considered 
cured.  The  very  theory  of  the  disease,  assuming  it  to  be  organic,  would 
make  a  cure  possible.  We  know  perfectly  well  that  there  are  certain 
degenerative  processes  that  we  may  arrest;  for  example,  tabes  and  de- 
generative processes  affecting  the  kidney  or  liver,  for  degenerative  pro- 
cesses were  not  always  progressive."  W.  H.  Thomson,  in  the  discussion, 
said  that  he  considered  functional  nervous  diseases  as  true  derange- 
ments of  the  source  of  nerve  function,  independently  altogether  of  the 


LEUKOSES  OCCASIONED  BY  EYE-STKAIX         787 

structure  of  the  brain, — i.e.,  the  blood.  Functional  disease  might  be  so 
serious  as  to  terminate  life,  and  yet  no  nerve  cell  or  fibre  could  be 
demonstrated  to  be  diseased,  the  disorder,  perhaps,  having  been  alto- 
gether due  to  a  toxin  in  the  blood.  He  could  not  believe  that  a  disease 
so  strikingly  characterized  by  intermittency  coiild  consist  essentially 
in  a  structural  lesion,  nutritive  or  otherwise.  He  fully  agreed  with 
Dr.  Starr  that  epilepsy  was  not  curable  by  the  surgeon,  and  stated  on 
the  contrary  that  a  surgical  operation  on  the  brain  was  apt  to  be  fol- 
lowed by  epilepsy.  A  polypus  of  the  nose  sometimes  gave  rise  to  an 
intermittent  asthma.  He  had  had  a  number  of  cases  in  which  the 
removal  of  peripheral  irritation  had  been  followed  by  the  permanent 
disappearance  of  epilepsy.  With  regard  to  the  pathology  of  epilepsy, 
we  were  often  misled  in  regarding  epilepsy  as  due  to  a  discharge  in  the 
cerebral  cortex.  His  definition  of  epilepsy  was  a  "  sudden  derange- 
ment of  the  normal  inhibition  of  cortical  centres  upon  one  another, 
dependent  in  the  first  instance  upon  an  abnormal  afferent  impression." 
R.  H.  jMacAllister  ^^  analyzed  250  cases  of  epilepsy  treated  in  C.  L. 
Dana's  clinic  and  private  practice,  and  does  not  mention  ocular  affec- 
tion among  the  etiological  factors. 

Published  cases  of  the  cure  of  epilepsy  by  the  removal  of  peripheral 
irritations  are  not  uncommon,  and  include  many  varieties  of  lesions. 
Pecho  ^  ^  saw  patients  recover  after  enucleation  of  an  atrophied  ball, 
following  an  injury  in  early  life.  Galeowski  ^^  records  the  history  of 
a  man,  aged  forty,  whose  right  eye  was  injured  six  years  before,  and 
for  which  abscission  of  the  anterior  part  of  the  eye  had  been  performed. 
The  stump  of  the  right  eye  was  still  very  sensitive.  For  six  months 
before  he  came  under  the  care  of  Galezowski  he  had  frequent  epileptic 
seizures,  and  vision  in  the  left  eye  had  been  failing.'  The  ophthalmo- 
scope showed  left  neuroretinitis,  venous  congestion,  perivascular  infil- 
tration, and  retinal  hemorrhages.  The  stump  of  the  right  was  sensitive 
to  pressure.  After  enucleation  of  the  stump,  the  epileptic  attacks  dis- 
appeared and  vision  in  the  left  eye  improved.  Stoewer,  reported  the 
case  of  a  married,  healthy  man  thirty-two  years  old,  who  had  been 
all  his  life  myopic.  In  1891  influenza  had  been  followed  by  an  in- 
tractable iritis  and  that  in  turn  by  epileptic  seizures  every  few  days, 
headache  and  eyeache.  The  eye  was  blind  from  closure  of  the  pupil. 
He  had  tonic  and  clonic  spasms,  in  which  he  became  unconscious,  and 
after  recovery  was  extremely  forgetful.     He  was  treated  by  suggestion, 

"  Xew  York  Medical  Journal,  January  29,  1894. 
"Rec.  d'Ophthal.,  January,  1886. 
"Rev.  d"Ophthal.,  January,  1886. 


788  THE    EYE    AND    XERVOUS    SYSTEM 

electricity,  and  bromides,  without  benefit.  On  the  contrary,  the  attacks 
became  more  frequent  and  violent.  The  patient  became  exhausted  and 
emaciated.  .  The  attacks  immediately  ceased,  and  improvement  in 
health  marked,  after  enucleation  of  the  left  eye.  Knapp  ^*  says, 
"  There  is  a  class  of  cases,  however,  in  which  the  reflex  origin  seems 
fairly  probable, — the  cases  of  so-called  epilepsy.  A  critical  examina- 
tion of  the  cases  reported  under  this  head  would  eliminate  many  of 
them.  A  considerable  number  of  them  are  undoubtedly  hysteria,  and 
in  others  the  dependence  of  epilepsy  ujwn  the  alleged  source  of  irrita- 
tion is  certainly  doubtful.  Xevertheless,  the  theory  that  a  remote  irrita- 
tion may  give  rise  to  convulsive  seizures  is  supported  by  the  well-known 
experiments  of  Brown-Sequard,  who  produced  epilepsy  in  the  guinea-pig 
by  injury  of  the  sciatic,  and  by  a  limited  number  of  authentic  cases  in 
man.  In  most  cases  cited,  the  source  of  the  epilepsy  has  been  of  trau- 
matic origin,  and  upon  removal  of  the  source  of  irritation,  the  epilepsy 
has  ceased.     These  cases  are,  however,  extremely  rare." 

That  heredity  is  an  important  predisposing  factor  is  shown  by 
Hare,^^  who  found,  after  examination  of  many  cases  of  epilepsy,  that  in 
25  per  cent,  a  distinctly  neurotic  family  history  could  be  traced. 

The  above  extracts  are  sufficiently  numerous  and  trustworthy  to 
prove  that  the  symptom  complex  known  as  epilepsy  may  be  caused  by 
peripheral  lesions  in  persons  who  have  neurotic  tendencies. 

Science  has  been,  as  yet,  unable  to  discover  the  post-mortem  lesions, 
or  conditions  which  explain  the  symptoms  of  epilepsy  in  the  so-called 
functional  forms.  If  it  were  possible  to  distinguish  between  func- 
tional and  organic  epilepsies,  the  whole  subject  would  be  elucidated. 
And  yet,  no  description  of  epilepsy  has  been  considered  complete  with- 
out a  detailed  account  of  the  post-mortem  findings.  The  pathology  of 
epilepsy  is  that  of  the  disease  which  causes  the  convulsions  and  is,  in 
many  cases,  unknown.  Gowers  well  says  that  "  the  changes  in  the 
nerve-centres  are  probably  of  that  kind  which  is  revealed  only  by 
altered  function  and  eludes  the  most  minute  search.  In  the  absence  of 
any  help  from  anatomy  and  histology,  the  pathology  of  idiopathic 
epilepsy  is  a  matter  of  hypothesis."  With  the  organic  form  of  the 
affection  we  have  nothing  to  do  in  this  paper,  and  confine  our  observa- 
tions to  functional  neuroses  as  indications  of  peripheral  irritation. 
In  such  affections  there  is  primarily  no  pathology-;  secondarily,  as  the 
result  of  the  habit  of  convulsions,  associated  with  unconsciousness,  the 


"American  Jotirnal  of  the  Medical  Sciences,  Octolier,  1805. 
"  Quoted  by  Wood  in  Pepper's  Text- Hook. 


LEUKOSES  OCCASION^ED  BY  EYE-STRAIX         789 

cortex  may  undergo  changes  which  later  develop  into  appreciable  ana- 
tomical lesions. 

Although  the  vasomotor  theory  of  the  causation  of  the  nerve  ex- 
plosions has  been  given  up  as  explanatory  of  epilepsy,  it  is  still  more 
applicable  than  any  other  theory  to  explain  the  condition  of  reflex 
epilepsy.  The  influence  of  the  vasomotor  nerves,  originating  or  passing 
through  the  ganglia  of  the  cervical  sympathetic,  is  not  thoroughly  un- 
derstood. The  effect  of  exsection  of  the  superior  ganglion  upon  the 
ocular  tissues,  however,  is  sufficiently  pronounced  to  warrant  the  in- 
ference that  the  nerve  impulse  that  passes  out  of  the  ganglion  to  the 
cerebral  vessels,  is  modified  by  the  condition  of  the  ganglion,  whether 
diseased  or  healthy.  It  may  be  here  incidentally  stated  that  in  seeking 
to  find  a  cure  for  idiopathic  epilepsy  surgeons  have  removed  the  ganglia 
in  order  to  note  the  effect  of  its  absence  upon  the  calibre  of  the  cerebral 
blood-vessels.  The  results  of  sympathectomy  upon  the  eye  are  thus 
summarized  by  De  Schweinitz :  ^®  "  Myosis,  narrowing  of  the  palpebral 
aperture,  projection  of  the  nictitating  membrane,  diminished  intra- 
ocular tension,  certain  ophthalmoscopic  and  microscopic  lesions  in  the 
eye-ground,  and  possibly  trophic  disturbances." 

The  causative  relations  of  derangements  of  the  ocular  apparatus  to 
epilepsy  have  been  the  subject  of  no  little  controversy  among  neurolo- 
gists and  o'phthalmologists.  Since  the  publication  of  Stevens's  prize 
essay,  and  the  investigation  of  Stevens's  claims  by  the  ^NTew  York 
Xeurological  Society,  the  attention  of  the  profession  has  been  directed 
toward  eye-strain  as  a  cause  of  epilepsy,  the  relief  of  the  convulsions 
by  correction  of  optical  errors,  the  restoration  of  balance  of  the  extra- 
ocular muscles,  and  of  the  normal  relationship  between  the  intraocular 
and  the  extraocular  muscles.  The  value  of  Stevens's  labors  has  not 
been  fully  appreciated  by  the  general  practitioner,  or  even  by  the 
neurologist,  and  the  ophthalmologist's  efforts  in  this  field  are  limited, 
except  as  employed  in  conjunction  with  his  colleagues.  The  same  idea 
is  more  forcibly  expressed  by  R.  H.  Gibbons :  ^^  "I  know  a  young  man 
cured  of  epilepsy  in  a  bad  form  by  Ranney,  and  I  know  of  four  cases 
cured  by  Stevens.  !N^one  of  these  cases  has  been  reported  and  one  of 
them  has  been  cured  for  five  years.  Stevens  and  Ranney  have  been  mis- 
quoted, or  worse, — they  have  been  misjudged  by  ophthalmologists  in 
general  and  by  neurologists  in  particular."  The  laity  has  already 
learned  by  experience  to  apply  to  the  oculist  for  relief  of  headache,  but 

^'The  Relation  of  the  Cervical  Synjpathetic  to  the  Eye,  Ophthal.  Sec.,  Amer. 
]Med.  Asso.,   1903. 

"  Trans.  Penna.  State  Med.  Soc,  1902. 


TOO  THE    EYE    AND    NERVOUS    SYSTEM 

is  not  sufRcientlj  advanced  to  appreciate  that  other  neuroses  may 
originate  in  eye-strain.  The  general  practitioner  must  take  the  initia- 
tive, and,  in  the  treatment  of  epilepsy,  should  endeavor  to  discover  and 
probe  every  possible  cause,  including  the  ocular  apparatus,  and  not 
wait  until  all  the  text-books  on  etiology  have  been  exhausted  before 
turning  the  eyes  in  the  right  direction.  The  writer  has  natural  hesita- 
tion in  expressing  himself  in  a  definite,  practical  manner  as  to  the 
relation  that  eye-strain  bears  to  the  causation  of  epilepsy.  Evidence 
culled  from  books  and  monographs  is  conflicting.  It  must  be  admitted 
that  advocates  of  this  theory  of  causation  have  not  been  successful  in 
carrying  conviction  to  the  minds  of  men  who  have  had  most  to  do  with 
epileptics.  The  theory  has  undoubted  claims  to  careful  and  impartial 
consideration,  and  the  work  done  along  these  lines  is  of  sufficient  weight 
to  compel  belief  or  refutation.  The  writer's  o\vn  belief  is,  that  a  small 
proportion  of  cases  of  epilepsy,  and  of  epileptiform  convulsions,  has 
been  cured  by  eye  treatment  alone,  and  that  the.  proportion  equals,  in 
percentage  of  cures,  that  of  any  other  single  surgical  or  mechanical 
means.  He  strongly  advocates,  moreover,  a  thorough  and  systematic 
ocular  examination  in  all  patients  in  whom  the  etiology  of  the  convul- 
sions is  unknown.  In  forming  this  opinion,  he  is  guided,  not  by  his  own 
experience,  which  is  most  limited,  but  by  the  cases  and  histories  re- 
ported in  recent  literature.  Stevens  ^*  examined  the  ocular  conditions 
in  140  cases  of  epilepsy.  "  The  general  result  has  been  to  reveal  the 
existence,  in  these  cases,  of  refraction  anomalies  in  a  considerably  greater 
proportion  than  has  been  found  by  Cohen  in  his  examination  of  the 
eyes  of  school  children,  or  by  other  observers  in  similar  investigations 
prosecuted  in  Germany,  Russia,  and  America.  In  100  consecutive 
cases,  hyperopia  was  present  in  59,  myopia  23,  emmetropia,  or  refrac- 
tion less  than  1  D.,  18.  Twenty-nine  cases  were  treated  only  by  the 
removal  of  optical  defects.  Of  these,  14  may  be  considered  well,  2 
(still  under  observation)  are  believed  to  be  permanently  relieved;  3, 
still  under  treatment,  have  received  such  marked  relief  that  it  is  be- 
lieved an  entire  discontinuance  of  the  malady  may  be  expected.  One 
died,  7  received  temporary  relief,  and  2  manifested  no  improvement. 
Some  remarkable  histories  and  striking  photographs  are  appended  to 
the  histories  of  a  few  of  the  most  pronounced  cases.  Ranney's  sum- 
mary of  20  cases'^  is  as  follows:  Four  abandoned  treatment.  Of  the 
22  remaining  cases,  10,  or  45  per  cent,  may  be  considered  as  well;  7 
being  completely  cured  and  3  being  practically  cured ;    amelioration  of 

"  Functional  Nervous  Diseases,  1887. 

"Reported  in  "Eye-Strain  in  Health  and  Disease,"  1897. 


LEUKOSES  OCCASIONED  BY  EYE-STRAIN         791 

the  attacks  has  been  afforded  by  eye  treatment  in  9  cases,  or  nearly  42 
per  cent. ;  no  improvement  has  been  observed  in  3  cases,  about  13  per 
cent.  Ranney's  collection  of  case  histories  is  truly  a  remarkable  one, 
and,  viewed  from  an  unprejudiced  stand-point,  convincing.  As  he  says 
in  the  general  resume,  "  I  have  brought  forward  here  some  very  strong 
written  testimony  from  physicians  of  repute,  and  the  patients  them- 
selves, to  prove  that  the  histories  published  by  me  are  those  of  genuine 
epileptics,  that  the  results  were  as  I  stated,  and  that  benefit  followed 
the  relief  of  eye-strain  after  a  failure  of  medicines  or  diet  to  control  the 
seizures."  J.  E.  Colburn  ^°  examined  43  cases  of  well-marked  epilepsy, 
and  from  this  number  selected  14  which  presented  subjective  and  ob- 
jective symptoms  of  eye-strain.  Those  rejected,  29  in  number,  pre- 
sented other  causes  for  the  epilepsy  or  failed  to  afford  evidence  that  the 
eyes,  or  their  function,  were  abnormal.  The  patients  who  submitted  to 
the  eye  treatment  had  refractive  or  muscular  anomalies,  or  both,  and 
were  treated  by  glasses,  prisms,  or  tenotomies.  His  results  were  as 
follows :  8  cases  had  no  return  of  the  convulsions  or  other  indica- 
tions of  a  continuance  of  the  disease  for  a  period  of  from  two  to  six 
years ;  6  were  benefited  in  a  general  way  and  the  attacks  rendered  less 
frequent ;  one  relapsed  as  soon  as  the  glasses  were  withdrawn ;  one 
received  no  benefit ;  one,  a  case  aggravated  in  every  respect,  could  not 
be  held  under  observation.  The  same  author  reports  ^^  a  case  of 
epilepsy  cured  by  correcting  an  error  of  refraction,  the  inherited  neu- 
rotic taint  being  especially  prominent  in  the  family  of  the  patient.  A  man, 
aged  twenty-four,  complained  of  mental  confusion,  flushing  of  the  face 
and  vertigo  in  attempting  to  read,  and  remarked  that  the  symptoms  dis- 
appeared when  the  eyes  were  closed.  At  times  he  was  dull  and  morose, 
and  at  others  brilliant  and  exemplary.  The  diagnosis  was  that  of  "  petit 
mal  with  impulses."  The  patient  had  been  confined  in  an  asylum  at 
intervals  for  two  years  on  account  of  epileptic  seizures.  Examination 
of  the  refraction  showed  hyperopia  .50  D.  of  manifest,  2  D.  of  latent; 
esophoria  8  degrees.  By  restoration  of  the  balance  of  the  ocular 
muscles  by  prism  exercise  and  correction  of  the  total  H.,  the  man  was 
relieved  of  his  pitiable  condition  and  was  made  strong  and  well.  The 
patient's  mother  suffered  from  neurasthenia  and  "  masked"  epileptic 
convulsions,  vertigo  and  unsteadiness  of  gait.  She  had  H.  2  D.,  HAs.  1 
D.,  and  a  muscular  error.  After  the  optical  defects  were  fully  corrected 
she,  also,  became  well.  The  patient's  sister  was  emmetropic  with 
neurotic  tendencies.     A  brother  had  been  an  inmate  of  an  asylum  for 

=»  Chicago  Medical  Record,  1894. 

-^  Peoria  Medical  Monthly,  February,  1888. 


792  THE    EYE    AND    NERVOUS    SYSTEM 

some  form  of  insanity.  Colburn  says,  "  The  hereditary  hyperopia 
error  of  refraction  was  responsible  for  all  the  symptoms  of  progressive 
cerebral  disease,  up  to  insanity."  C.  M.  Capps  records  two  cases.^^ 
One  was  that  of  a  girl  of  nine  who  consulted  him  one  year  before  re- 
porting the  result  of  the  treatment.  She  had  three  to  six  epileptic 
convulsions  every  month  since  four  years  of  age.  Since  entering 
school  the  seizures  had  become  more  frequent  and  violent.  The  seizures 
ceased  while  the  accommodation  was  paralyzed  with  atropin,  and  re- 
mained absent  during  the  year,  while  wearing  correction  for  M.  and 
MAs.  The  second  case  was  a  girl  of  eight  who,  after  correction  of  II. 
and  IIAs.,  had  complete  cessation  of  epileptic  attacks.  W.  F.  Connors 
describes  the  interesting  case  of  a  young  man  ^^  who  was  cured  by 
wearing  correction  for  high  II.  Removal  of  the  glasses  was  imme- 
diately followed  by  the  prodromes  of  an  epileptic  convulsion.  Reber  ^* 
narrates  four  cases  that  are  worthy  of  brief  description.  Case  1,  mar- 
ried woman,  aged  thirty,  high  hyperopic  refraction  combined  with  mus- 
cular imbalance,  treated  by  spherocylinders  and  prisms ;  result,  "  with- 
out any  suggestion  that  her  epilepsy  might  be  benefited  and  with  the 
withdrawal  of  all  drugs,  this  woman  was  rescued  from  a  future  that 
was  very  gloomy  at  best.  She  had  been  having  two  or  three  seizures 
weekly,  when  she  would  fall  to  the  floor  unconscious  and  remain  in  a 
stupor  for  an  hour  or  more.  To  be  brought  from  that  condition  to  the 
present  one  (having  one  fleeting  fit  a  month,  without  loss  of  conscious- 
ness) is  practically  a  cure."  Case  2,  unmarried  woman,  aged  twenty, 
in  apparently  perfect  health  with  the  exception  of  nocturnal  epilepsy, 
having  had  for  a  long  time  12  to  14  seizures  every  week.  The  fre- 
quency of  the  attacks  was  reduced  to  one  per  week  by  wearing  myopic 
cylinders.  Later,  prisms  were  added  to  her  glasses.  After  wearing  the 
combination  for  three  months,  she  reported  that  she  was  virtually  free 
from  attacks,  having  had  them  only  at  great  intervals  and  very  lightly." 
Case  3,  a  strong,  active  youth  of  twenty.  After  reading  several  hours 
in  the  evening  he  would  suffer  on  the  next  day  several  attacks  of  petit 
mal.  For  several  years  he  had  the  attacks  at  irregular  intervals,  some- 
times one  or  two,  sometimes  several  weeks  apart.  Treatment  of  mus- 
cular imbalance  by  tenotomies  reduced  the  seizures  to  one  or  two  slight 
attacks  in  over  two  years,  so  that,  to  all  intents  and  purposes,  he  is  a 
cured  man.  Case  4,  a  man  of  twenty-one,  one  of  a  family  of  eight 
children.     "  There  are  several  neurotics  in  the  family,  and  the  general 

"Xew  York  Medical  Journal,  September  IG,  1899. 

"  Medical  News,  November  4,  1893. 

"  Pennsylvania  Medical  Journal,  November,  1902. 


XEUKOSES  OCCASIOXED  BY  EYE-STKAIN         793 

ancestral  history  is  studded  with  neurasthenia,  hysteria,  and  chorea, 
but  no  epilepsy  or  alcoholism.  At  seventeen,  he  commenced  to  have 
epilepsy,  having  two  seizures  within  24  hours  of  each  other  every  three 
or  four  weeks.  Under  partial  prismatic  correction  of  exophoria  and 
withdrawal  of  drug  treatment  the  attacks  ceased  for  four  months,  when 
two  very  light  attacks  were  induced  by  excessive  reading  several  nights 
in  succession.  Instead  of  the  8  to  10  attacks  he  would  have  had  within 
the  19  months,  taking  all  the  time  heavy  doses  of  bromides,  he  had  two 
very  light  seizures,  almost  imperceptible,  and  that  without  any  drug 
treatment  at  all."  F.  G.  Murphy  writes  ^^  that  in  the  past  two  years 
he  had  five  epileptics  under  his  care,  four  of  whom  had  defective 
eyes.  Two  of  these  were  completely  relieved  of  all  their  epileptic 
symptoms  by  the  correction  of  their  optical  defects.  The  remaining 
two  wore  their  correction  with  relief  of  ocular  symptoms,  but  none 
whatever  from  epileptic  seizures.  Ranney  reports  ^®  results  of  eye  treat- 
ment in  29  cases :  7  completely  cured,  3  practically  cured,  9  improved,  3 
not  improved,  4  not  counted.  Gould  ^"  claims  to  have  cured  six  cases,  and 
in  all  "  the  cure  was  immediate,  certain  and  complete  and  persisted  for 
several  years."  He  agrees  with  most  ophthalmic  surgeons  that  in  ex- 
ceptional cases  the  ocular  condition  may  be  the  pernicious  factor  that 
maintains  the  epileptic  manifestations.  The  obligation  is  imperative 
that  in  every  case  of  epilepsy  the  condition  of  the  eyes  should  be  care- 
fully considered.  In  the  Spratling  and  Gould  report  of  the  eye  ex- 
aminations and  results  of  eye  treatment  in  78  selected  cases  of  epilepsy, 
who  were  patients  in  the  Craig  Colony ,^^  the  same  differences  of  opinion 
that  characterizes  similar  reports  are  found.  Marlow  ^®  corrected  the 
refraction  of  a  neurotic  patient.  The  patient  gained  20  pounds  in 
weight  as  a  result  of  wearing  the  glasses,  and  at  the  end  of  five  years 
continued  free  from  the  intense  nervousness  and  occasional  epilepsy 
that  had  baffled  many  physicians  for  some  years.  Coover  details  15 
cases  ^"  of  reflex  irritation  accompanying  eye-strain.  Among  the  promi- 
nent symptoms,  all  of  which  were  relieved  by  correction  of  refractive 
error,  were  attacks  of  convulsions  resembling  epilepsy  caused  by  con- 
tinued use  of  the  eyes  at  the  near  point.  Elmer  ^^  recited  instances  of 
patients  afflicted  with  epileptic  seizures,  of  mental  conditions  approach- 

-'  Trans.  Iowa  State  Med.  Soc,  vol.  xvi.,  1898. 

-"  New  York  Medical  Journal,  January  2  and  January  9,  1897. 

"  Journal  of  the  American  Medical  Association,  July  o,  1902. 

^  American  Medicine,  April  9,  1904. 

=^  Medical  News,  July  4.  1897. 

=»  Medical  News,  October  26,  1895. 

='^Amer.  Jour.  Ophthal.  and  Otol.,  April,  1896. 


794  THE    EYE    AND    NERVOUS    SYSTEM 

ing  insanity,  of  chorea,  and  of  petit  mal,  who  were  entirely  relieved 
by  correcting  glasses. 

Ilaving  thus  presented  some  of  the  evidence  published  in  advocacy 
of  the  treatment  of  the  eyes  as  a  means  of  cure  of  epilepsy,  the  other  and 
negative  side  claims  attention.  Peterson  ^^  does  not  remember  to  have 
seen  among  several  hundred  epileptics  a  single  case  of  genuine  reflex  epi- 
lepsy. Gowers  '^  says,  "  It  is  extremely  rare  to  meet  with  any  evidence 
of  a  reflex  cause  in  the  system  of  the  cerebrospinal  nerves  and  in  the 
few  recorded  cases  there  has  been  a  manifest  and  strong  predisposition." 
Starr  believes  that  reflex  neuroses  of  an  epileptiform  type  are  ex- 
ceedingly rare,  and  Dana  says  "  the  importance  of  reflex  irritation  has 
been  much  overestimated."  Sachs  does  not  even  mention  reflexes  among 
the  causes  of  epilepsy,  except  in  a  footnote,  where  he  says  the  disease 
is  rarely  established  by  peripheral  irritation.  Concerning  eye  con- 
ditions, he  says  that  they  may  cause  single  attacks,  but  they  surely  are 
not  the  cause  of  epilepsy.  He  asserts  that  Kanney  does  not  cure  cases, 
and  makes  the  statement  on  the  ground  that  Ranney  has  not  observed 
them  long  enough.  Both  Dana  and  Sachs  reiterate  these  opinions  in 
their  latest  published  articles.^^  Fere  ^^  as  late  as  1897,  in  his  full 
chapter  on  epilepsy,  says  that  "  errors  of  refraction,  necessitating  as 
they  do  efforts  of  accommodation,  may  cause  epilepsy,"  and,  in  the 
section  devoted  to  treatment,  "  anomalies  of  refraction  must  be  cor- 
rected." He  does  not  include  anomalies  of  the  ocular  muscles  among 
the  causes.  Gowers,  in  his  monograph,^*^  is  silent  on  the  subject.  Sink- 
ler  and  Pearce,^^  in  their  analysis  of  315  cases,  also  pass  ocular  causes 
by  without  comment.  H.  A.  Hare  ^*  quotes  Stevens  as  to  the  relation 
of  muscular  anomalies  and  refraction  errors  to  the  production  of  epi- 
lepsy, and  says  that  thus  far  Stevens's  remarkable  cases  have  adduced 
no  evidence  in  rebuttal,  but  in  the  section  on  treatment,  he  does  not 
refer  to  the  ocular  treatment,  as  one  of  the  means  of  relieving  epilepsy. 

Thus,  it  will  be  seen  that  the  proof  of  the  causal  relations  of  affec- 
tions of  the  eye  to  epilepsy  rests  entirely  with  the  ophthalmologists. 
The  ablest  among  the  neurologists  barely  refer  to  the  eye  as  a  cause,  or 
omit  it  altogether.  An  unbiased  opinion  formed  from  a  study  of  the 
literature  on  both  sides  of  the  question,  and  expressed  tersely,  would  be 

"  New  York  Medical  Journal,  July  4,  189G. 

"  Mental  Diseases. 

**  Medical  News,  July  30,  1904. 

**  Twentieth  Century  Practice,  vol.  x. 

*•  Epilepsy  and  other  Convulsive  Diseases,  1901. 

"Trans.  Med.  Soc.  State  of  Penna.,  1897. 

•*  Epilepsy,  its  Pathology  and  Treatment,  1890. 


NEUEOSES  OCCASIONED  BY  EYE-STRAIN         795 

that  exceptionally  the  so-called  reflex  and  idiopathic  epileptic  convul- 
sions may  be  cured  by  correction  of  ocular  anomalies. 

Treatment. — Ocular  examination  conducted  with  patience,  ability, 
and  thoroughness  should  be  made  in  every  case  of  epilepsy,  including 
all  its  forms.  In  a  large  proportion,  the  result  of  ocular  remedies,  both 
on  the  violence  and  frequency  of  the  seizures,  will  demonstrate  only 
its  negative  value;  in  a  smaller  proportion,  the  seizures  will  be  per- 
manently arrested.  It  would  seem  to  the  writer  that  a  distinction  be- 
tween epilepsy,  epileptiform  convulsions,  petit  mal  and  grand  mal  need 
not  be  drawn  when  we  are  face  to  face  with  the  problem,  What  shall 
we  do  with  this  case  of  convulsions  ?  The  prognosis  of  the  affection  is 
universally  admitted  to  be  unfavorable,  and  why  should  we  decline  to 
investigate  and  remove  all  possible  sources  of  peripheral  irritation, 
whether  the  symptoms  are  said  to  belong  technically  to  the  so-called 
"  true"  epilepsy  or  are  only  epileptiform  in  character  ?  The  therapy  of 
drugs  has  failed  to  cure.  The  bromide  treatment,  that  most  generally 
resorted  to,  is  admittedly  palliative  only.  By  dulling  the  cerebral 
forces,  outbreaks  or  crises  of  cerebral  activity  are  held  in  abeyance. 
The  advocates  of  the  bromide  treatment  recognize  its  value  in  reliev- 
ing symptoms,  but  do  not  claim  for  it  curative  power.  Brain  surgery 
accomplishes  a  little.  Da  Costa  says  that  4  per  cent,  of  all  epileptics 
are  cured  by  operation  on  the  skull.  The  removal  of  reflex  irritation 
arising  from  atrophied  eyeballs,  nasal  disease,  old  cicatrices,  degen- 
erated testes,  and  other  lesions,  has  been  followed  by  relief  of  the  con- 
vulsions, and,  if  well  authenticated  reports  of  cure  by  eye  treatment 
are  to  be  credited,  a  knowledge  of  the  ocular  conditions  will  suggest 
methods  of  treatment  that  we  cannot  afford  to  neglect. 

The  examination  of  the  eye  includes,  first,  refraction,  and,  second, 
the  extraocular  muscles. 

1,  Refraction. — (For  detailed  method  of  diagnosis  and  treatment  of 
refraction  errors,  the  reader  is  referred  to  one  of  the  text-books  on  that 
subject.)  (a)  Hyperopia  and  hyperopic  astigmatism.  Under  full 
paralysis  of  accommodation  in  individuals  less  than  forty-five  years 
old  the  total  error  is  determined,  and  as  near  a  full  correction  as  will 
be  tolerated,  ordered,  and  worn  constantly.  Thus,  the  unusual  exer- 
cise of  accommodation,  and  the  undue  stimulation  of  the  extraocular 
muscles  dependent  upon  it,  are  removed ;  the  congestion  of  the  uveal 
tract  so  commonly  associated  with  accommodative  asthenopia,  reduced; 
the  circulation  of  blood  and  lymph  restored  to  the  normal  and 
the  excessive  stimulation  of  the  ciliary  nerves,  and  consequent  over- 
action  of  the  other  branches  of  the  third  nerve,  relieved.      In  those 


796  tup:  eye  and  nervous  system 

cases  in  which  the  accommodation  conceals  a  latent  error  of  refraction, 
suggested  by  the  lack  of  harmony  between  the  degree  of  refraction  as 
determined  by  the  ophthalmoscope,  or  ophthalmometer,  or  a  difference 
of  refraction  between  the  near  and  far  points,  full  paralysis  of  the 
ciliary  muscle  should  be  obtained  by  stronger  solutions  of  the  cyclo- 
plegic  and  its  continued  application.  By  these  means  the  eye  is  fully 
rested,  the  degree  of  the  true  static  error  determined,  and  the  causes, 
resident  in  the  ocular  tissues  or  the  result  of  overuse,'  which  con- 
tribute to  tlie  production  of  reflex  symptoms,  eliminated. 

(6)  Myopia  and  myopic  astigmatism.  The  influence  of  increased 
refractive  power,  in  causing  reflex  phenomena,  has  already  been  alluded 
to.  By  spasm  of  the  accommodation,  by  the  unconscious  non-use  of 
the  accommodation  or  the  disruption  of  the  relation  of  accommodation 
and  convergence,  local  and  constitutional  symptoms  of  eye-strain  may 
be  produced.  Myopia  is,  however,  seldom  named  as  a  cause.  As  a 
rule  myopic  patients  do  not  complain  of  asthenopia  or  other  evidence 
of  eye-strain,  but  only  of  indistinct  distant  vision.  There  is  a  gradual 
increasing  tendency  to  divergence  of  the  visual  axes  which  is  to  be  con- 
trolled, and  its  further  development  into  an  actual  divergence  prevented, 
by  the  muscles  of  convergence.  The  maintenance  of  binocular  single  vis- 
ion by  the  ever-diminishing  power  of  convergence,  leads  either  to  the 
reflex  neuroses  in  neurotic  patients,  or  to  dissociation  of  the  eyes  in 
the  act  of  vision.  The  real  defect,  as  in  hyperopia,  can  be  detected  only 
through  cycloplegia,  but  unlike  hyperopia,  the  correction  selected  with 
mydriasis  is  apt  to  be  stronger  than  the  actual  degree  of  myopia.  Post- 
mydriatic  examination  and  the  ordering  of  glasses  after  complete  return 
of  the  accommodation,  in  order  that  its  power  may  be  measured  and 
allowance  made  when  advisable,  is  essential  to  a  practical  and  useful 
correction.  In  all  cases,  the  examination  should  be  repeated,  at  in- 
tervals of  one  or  two  years,  and  changes  in  the  refraction  followed  by 
corresponding  changes  in  the  glasses. 

(c)  Presbyopia.  Comparatively  few  cases  of  neuroses  are  to  be 
attributed  to  the  strain  of  the  eyes  from  failure  of  the  accommodation 
due  to  stiffness  or  loss  of  elasticity  of  the  lens,  or  to  the  failure  of  con- 
tractility of  the  ciliary  muscle.  Yet  such  cases  have  been  reported,  and 
marked  amelioration  of  distressing  symptoms  has  followed  the  use  of 
carefully  selected  glasses.  The  strain  arises  from  the  expenditure  of 
nervous  energ;v'  in  the  effort  to  focus  the  eyes  for  small  objects  at  33  cm. 
distance,  and  because  of  the  excessive  innervation  to,  and  response  of, 
the  muscles  of  convergence  without  an  equivalent  response  of  the  ciliary 
muscles.    The  strain  may  induce,  in  individuals  predisposed  to  nervous 


NEUROSES  OCCASIOXED  BY  EYESTRAIN         797 

affections,  the  identical  phenomena  that  are  witnessed  in  younger  per- 
sons who  suffer  from  "eye-strain.  The  correction  of  presbyopia  is  a 
simple  matter.  Having  learned  the  refraction  of  the  eye,  glasses  should 
be  given  which  supply  the  deficiency  of  accommodation  so  that  the 
patient  will  read  without  discomfort  at  33  cm. 

2.  The  Extraocular  Muscles. — In  all  cases  of  insufficiency  of  the 
extraocular  muscles,  readily  determinable  by  the  usual  tests,  and  by  the 
relations  which  the  findings  of  the  tests  bear  to  the  relative  strengths  of 
adduction  and  abduction,  the  true  static  refraction  must  be  learned  and 
the  correction  of  it  worn  long  enough  both  to  make  certain  that  the 
symptoms  are  not  due  to  ametropia  and  to  exert  its  beneficent  influence 
on  the  muscular  tensions.  In  persons  too^'oung  to  wear  glasses,  long 
continued  atropinization  may  be  effective.  Such  preliminary  treat- 
ment of  muscular  anomalies  before  resorting  to  prisms  or  operations  is 
imperative. 

(a)  Esophoria  is  a  tendency  to  cross  the  visual  axes.  The  rela- 
tively weak  divergent  power  is  insufficient  to  maintain  binocular  single 
vision  for  all  distances  without  undue  stimulation,  and  in  order  to 
obtain  relief,  treatment  should  be  directed  to  the  reduction  of  the 
demand  on  divergence.  This  may  be  accomplished  by  (1)  the  internal 
exhibition  of  nerve  sedatives  and  simultaneous  exercise  of  the  muscles 
of  abduction  by  prisms  and  the  stereoscope,  or  the  amblyoscope  of 
Worth;  and  (2)  by  prisms,  bases  out,  the  degree  of  the  prism  to  be 
determined  by  the  amount  of  esophoria.  The  prisms  may  be  incorpor- 
ated into  the  ametropic  correction,  or  the  latter  may  be  decentred  so 
that  it  has  prismatic  action,  or  they  may  be  worn  separately.  Although 
this  method  is  occasionally  serviceable,  it  is  open  to  the  objection  that 
it  can  only  be  useful  by  allowing  the  visual  axes  to  tutm  inward — 
esotropia.  It  is  only  a  crutch  for  the  disabled  muscles,  and  is  not  instru- 
mental in  restoring  the  muscles  to  equilibrium.  Moreover,  the  limit  of 
the  usefulness  of  prisms  is  soon  reached.  Esophoria  develops  under  their 
action,  either  by  uncovering  a  part  of  the  defect  hitherto  latent,  or  by 
actually  creating  a  greater  defect.  Notwithstanding  this  theoretic 
objection,  cases  that  resist  cure  by  complete  and  continued  rest  of  ac- 
commodation, sedatives  and  abduction  exercise,  should  wear  prisms 
before  resorting  to  the  final  means  of  cure.  (3)  Operation.  Shall 
tenotomy  or  advancement  be  chosen  ?  Esophoria,  dependent  upon  hy- 
peropic  refraction,  is  not  primarily  induced  by  weakness  of  divergence, 
but  by  excess  of  convergence,  a  physiologic  sequence  of  the  inordinate 
action  of  the  accommodation.  Hence  tenotomy  of  one  or  both  of  the 
interni  is  the  logical  procedure.     The  operation  should  be  performed 


798  THE    EYE    AND    NERVOUS    SYSTEM 

with  great  care  and  frequently  interrupted  to  note,  by  testing  with  the 
Maddox  rod  or  other  means,  the  effect  of  work  already  performed. 
Free  division  of  the  tendon  and  adjacent  structures  is  unjustifiable. 
Partial  or  graduated  tenotomy  only,  as  recommended  by  Stevens  and 
Kanney,  is  the  proper  operation.  These  surgeons  state  that  equilibrium 
can  seldom  be  secured  after  a  single  operation,  and  claim  that,  should 
more  defect  be  uncovered  as  time  goes  on,  a  second,  third,  or  more 
graduated  tenotomies  should  be  performed,  the  necessity  for  the  subse- 
quent operations  being  indicated  by  a  return  of  the  symptoms. 

(Z>)  Exophoria  is  a  tendency  of  the  visual  lines  outward.  The  cor- 
rection of  ametropia,  the  use  of  nerve  stimulants  in  large  doses,  prism 
exercise  of  the  weakened  divergence,  the  restriction  of  the  time  de- 
voted to  the  near  use  of  the  eyes,  and  other  hygienic  measures  referred 
to  under  esophoria,  will,  in  the  majority  of  cases  of  exophoria,  obviate 
the  necessity  for  operation.  Should,  however,  the  symptoms  resist  this 
treatment  and  the  wearing  of  prisms,  advancement  or  resection  of  one 
or  both  intern i  should  be  preferred  to  tenotomy  of  the  externi.  Thus, 
deficient  adduction  may  be  strengthened  and  made  to  compensate  for 
the  relatively  stronger  abduction. 

(c)  Hyperphoria  is  a  tendency  of  the  visual  axes  to  separate  ver- 
tically, or  of  one  visual  axis  to  assume  a  plane  higher  than  that  of  its 
fellow\  This  is  the  most  frequent  of  the  anomalies  of  the  muscles  of 
the  eye  to  cause  reflex  neuroses,  and  to  Stevens  is  due  the  credit  of 
scientifically  and  practically  elaborating  the  causal  relations,  and  of 
bringing  the  subject  impressively  to  the  notice  of  the  profession.  As 
in  the  other  muscular  anomalies,  this  is  dependent  in  the  large  ma- 
jority of  cases  upon  errors  of  refraction.  The  principles  of  treatment 
are  essentially  those  of  other  defects  of  co-ordination. 

CHOREA. 

Involuntary  tonic  or  clonic  contraction  of  a  muscle,  or  of  groups  of 
muscles,  is  a  symptom  of  an  organic  disturbance  of  the  cerebrospinal 
system,  or  a  reflex  perversion  of  function  of  one  or  more  of  the  organs 
of  the  body.  The  former,  or  "  true"  chorea,  is,  according  to  some 
authorities,  an  acute  infectious  disorder,  variable  in  its  limitations, 
nature  and  course,  according  to  the  character  of  the  basic  disease.  The 
latter  "  habit,"  reflex  or  symptomatic  chorea,  is  a  purely  functional 
affection,  the  development  of  a  peripheral  irritation. 

The  word  chorea  has  been  interchangeably  used  by  writers  in  de- 
scribing true  chorea  and  habit  chorea,  affections  that  are  essentially 
different  in  their  etiology,  prognosis,  and  treatment.     In  consequence 


LEUKOSES  OCCASIONED  BY  EYE-STIlAi:>^         799 

of  this  vagueness  of  definition,  much  confusion  has  arisen  as  to  the 
true  significance  of  chorea,  and  unprofitable  controversies,  more  or  less 
bitter,  have  been  waged  in  medical  journals. 

The  relation  of  eye-strain  to  the  causation  of  true  chorea  is  prac- 
tically denied  by  authors  of  wide  experience — men  whose  opinions  are 
worthy  of  the  greatest  consideration.  Thus,  Lees,^^  in  an  accurate  and 
presumably  full  description  of  the  pathology  and  treatment  of  this 
disease,  makes  no  mention  of  eye-strain  and  this  possible  cause  is  not 
alluded  to  by  the  speakers  who  discussed  his  paper.  M.  Allen  Starr 
scientifically  studied  1-iOO  cases  treated  in  the  Vanderbilt  clinic,  and 
Krafft-Ebing  200  cases,*^  without  finding  a  single  subject  in  which  the 
cause  could  be  attributed  to  eye-strain.  Aldrich,^^  in  an  inapartial 
study  of  the  subject,  criticises  Stevens's  and  Kanney's  reported  cases 
of  the  cure  of  chorea  by  eye  treatment,  by  stating  that  their  cases  were 
habit  spasm,  or  possibly  hysterical  chorea,  and  not  one  case  was  true 
chorea.  "  It  was  merely  a  question  of  mistaken  diagnosis."  Baker's 
report  of  his  cases  ■*-  was  also  criticised  by  Aldrich  ^^  in  these  words : 
"  He  did  not  believe  that  the  winking  and  grimacing  of  children  as 
described  by  Baker  to  have  been  relieved  by  glasses  was  chorea,  and 
that  chorea  proper  could  not  be  caused  by  eye-strain  since  it  was  un- 
doubtedly an  acute  specific  nervous  affection."  Starr  further  says 
"  while  it  is  possible  that  local  twitching  of  the  muscles  of  the  eyes  or 
face  or  neck  may  be  produced  by  eye-strain  or  irritation  of  the  naso- 
pharynx, true  chorea  is  never,  in  my  opinion,  produced  by  these  causes, 
and  treatment  directed  to  the  relief  of  the  anomalies  of  the  eye  muscles 
is  useless." 

The  chorea  of  eye-strain  is  essentially  that  described  years  ago  by 
S.  Weir  Mitchell  as  "  habit  chorea,"  and  more  recently  by  Osier  ■**  as 
"  simple  tic"  or  habit  spasm.  According  to  the  latter  authority,  it  is 
to  be  carefully  differentiated  from  chorea  with  superadded  psychical 
phenomena,  and  from  complex  and  co-ordinated  chorea.  Further,  as 
Wilbrand  and  Saenger  have  pointed  out,^^  habit  chorea  should  not  be 
confounded  with  or  mistaken  for  the  reflex  chorea,  or  choreic  move- 
ments, of  the  orbicularis  palpebrarum  (blepharospasm),  of  the  frontalic 
and  the  corrugator  supercilii,  which  are  dependent  upon  irritative  or- 

^»  British  Medical  Journal,  1903,  11. 
"  Festschrift  in  honor  of  A.  Jacobi. 
"  Arch.  Pediat.,  1903,  xx. 
"American  Medicine,  March  14,  1903. 
"American  ^Medicine.  May  9.   1903. 
"Arch.  Ped.,  January,  1897. 
"  Die  Xeuroloofie  des  Auges. 


800  THE    EYE    AND    NERVOUS    SYSTEM 

ganic  lesions  of  the  cortical  centre,  nucleus,  or  of  the  seventh,  fifth,  or 
optic  nerve  trunks. 

Choreic  movements,  the  result  of  eye-strain,  involve  primarily  the 
muscles  of  the  lids,  secondarily,  those  of  the  face  and  neck,  and  in  ex- 
ceptional cases,  distant  groups  of  muscles  may  be  invaded.  The  extent 
of  the  spread  of  the  movement  and  its  violence,  are,  in  a  measure, 
independent  of  the  source  of  the  strain,  whether  it  be  due  to  an  error 
of  refraction  or  to  a  muscular  anomaly.  The  separation  of  these  two 
etiological  factors,  is,  in  a  majority  of  cases,  not  practicable.  As  has 
already  been  shown,  muscular  anomalies  rarely  exist  except  in  connec- 
tion with,  or  dependent  upon,  errors  of  refraction,  which  are  rarely 
found  in  association  witli  perfect  muscular  balance.  The  character 
and  intensity  of  the  reflex  phenomena  of  eye-strain  are  determined  less 
l)y  the  special  ocular  error  than  by  the  impressionability  of  the  nervous 
system,  the  age,  occupation,  and  environment  of  the  individual. 

The  credit  of  the  discovery  that  astigmatic  and  other  optical  errors 
may  he  responsible  for  reflex  nervous  phenomena  belongs  to  William 
Thomson,  who,  in  1879,  published  ^®  a  clinical  lecture  delivered  in  the 
Jefferson  Medical  College  Hospital,  in  which  he  says,  "  More  than  ten 
years  ago  I  became  aware  that  persons  who  consulted  me  for  imperfect 
sight  presented  symptoms  not  fairly  .described  in  the  text-books  under 
the  general  head  of  asthenopia  and  which  were  indicative  of  some 
serious  disturbance  of  the  nerve  centres."  Among  other  symptoms  he 
mentions  insomnia,  loss  of  appetite,  a  fear  of  some  impending  disaster, 
apoplexy  or  epilepsy,  general  nervous  prostration,  choreic  twitching  of 
the  muscles  of  the  head  or  face.  (Italics  mine.)  Finding  these  persons 
astigmatic  and  seeing  that  their  symptoms  yielded  to  the  supporting 
effect  of  properly  selected  cylindric  glasses,  the  attention  of  S.  Weir 
Mitchell  was  called  to  this  aspect  of  these  sufferers,  and  to  the  latter  dis- 
tinguished physician  Thomson's  discovery  seemed  of  sufficient  impor- 
tance to  bring  it  to  the  notice  of  the  general  profession  in  a  paper  in  the 
Medical  and  Surgical  Reporter,  1874,  and  the  American  Journal  of  the 
Medical  Sciences,  1876. 

During  the  thirty  years  that  have  elapsed  since  Thomson's  dis- 
covery and  pioneer  work  in  this  great  department  of  ophthalmology, 
tens  of  thousands  of  patients  have  been  successfully  treated  for  nervous 
disorders  by  remedies  directed  only  to  a  readjustment  of  the  focussing 
and  co-ordinating  apparatus  of  the  eyes.  Stevens's  study  of  the  ocular 
musculature  and  his  philosophical  deductions  and  practical  application 


**  Medical  News  and  Library.  .T>inp.  1897. 


XEUKOSES  OCCASIONED  BY  EYE-STRAIN         801 

of  them  to  the  correction  of  muscular  anomalies  ^^  marks  another  epoch- 
making  period  in  this  branch  of  science.  These  men  have  been  followed 
by  a  host  of  workers  along  the  same  lines,  who  have  reiterated  with 
increasing  emphasis  the  importance  of  eye-strain  as  a  factor  in  the 
causation  of  functional  nervous  diseases. 

Facial  chorea,  while  not  one  of  the  common  symptoms  of  eye-strain, 
is  yet  so  frequently  observed  that  oculists  accept  its  presence  as  evidence 
of  disturbed  ocular  function.  Its  only  resemblance  to  true  chorea  con- 
sists in  an  involuntary  contraction  of  voluntary  muscles.  Its  appear- 
ance, unlike  that  of  true  chorea,  is  independent  of  season  or  age  of  the 
individual.  It  has  a  distinct  relation  to  school  life,  in  the  young,  and  to 
certain  occupations,  because,  in  the  acquisition  of  education,  or  study  of 
a  profession,  as  in  other  pursuits  of  life,  the  defective  eyes  cannot 
respond  to  the  demand  upon  them  without  strain.  The  child  is  taken 
from  school,  the  professional  man  from  his  books,  the  clerk  from  his 
ledger,  and  a  cure  results — a  cure  too  likely  to  be  credited  to  outdoor 
life,  change  of  environment,  rest  from  brain-fag,  etc.  Rest,  recreation, 
€xercise,  tonics  and  the  like,  are  of  distinct  value;  but,  on  the  other 
hand,  too  much  importance  should  not  be  attached  to  their  influence. 
Resumption  of  work  means  return  of  symptoms.  The  remedies  are  of 
■only  temporary  benefit.  The  fault  lies,  not  in  brain-fag,  too  much 
Avork,  too  much  reading,  too  much  indoor  life,  but  in  the  use  of  the 
■eyes  under  adverse  conditions.  Before  reaching  the  decision  that  the 
patient  is  "  run  down"  and  needs  winding  up  by  the  rest  treatment  or 
B.  course  of  tonics,  or  other  hygienic  measures,  examination  of  the  eyes 
should  be  made,  in  order  to  determine  whether  an  optical  error  or  func- 
tional derangements  of  the  muscles  may  be  responsible.  Wood's 
iidvice,^^  "  Especially  are  eye-strain  and  nasal  difficulties  apt  to  cause,  in 
childhood,  persistent  chorea,  and  it  is,  therefore,  essential  in  every  case 
which  resists  treatment  to  thoroughly  examine  these  organs,"  might  be 
advantageously  modified  into  a  recommendation  that  the  ocular  and 
nasal  possible  causes  be  excluded  before  treatment  directed  to  other 
organs  be  tried. 

Eye  treatment  is,  in  a  sense,  experimental.  It  is  quite  impossible 
to  predict  with  certainty  in  any  case  that  it  will  be  efficacious.  It  is, 
however,  reasonable  and  logical  to  assume  from  knowledge  based  on 
previous  experience,  or  acquired  through  reading,  that  the  ocular  defect 
found  in  any  given  case  is  the  source  of  the  trouble,  or  in  the  absence 
of  a  defect  that  the  ocular  apparatus  is  not  a  causative  factor.    Wearing 

"  Arch.  Ophthal.,  1884. 
**  Pepper's  Amer.  Text-Book. 
51 


802  THE    EYE    AND    NERVOUS    SYSTEM 

of  a  needed  glass  or  the  correction  of  a  muscular  error  will  soon  decide 
the  etiology.  A  large  proportion  of  choreic  patients  have  hyperopia 
and  astigmatism,  but  95  per  cent,  of  all  individuals  have  an  error  of 
refraction;  therefore,  it  would  appear  that  frequency  of  ametropia  in 
choreics  has  little  or  no  bearing  on  the  relation  of  cause  and  effect. 
Cheney  *^  examined  23  cases  of  chorea,  and  found  emmetropia  2, 
hyperopia  15,  hyperopic  astigmatism  3,  myopia  1,  myopic  astigma- 
tism 1,  unknown  1,  esophoria  8.  His  conclusion  was  that  "  patients 
frequently  recover  while  undergoing  no  treatment  whatever,  but  the 
marked  improvement  or  rapid  recovery  following  the  correction  of  re- 
fractive or  muscular  errors  in  some  of  these  cases  makes  it  seem  more 
than  probable  that  the  ocular  defects  were  the  exciting  cause  of  the 
disease."  The  same  writer  reports  the  case  of  a  boy,  eight  years  of  age, 
who,  since  his  fifth  year,  had  never  been  free  from  chorea,  although  the 
twitchings  were  slight  at  all  times.  The  patient  complained  of  head- 
aches for  a  number  of  years.  Two  months  previous  to  Dr.  Cheney's 
examination  it  was  noticed  that  the  left  eye  turned  inward;  inci- 
dentally the  twitchings  became  considerably  aggravated,  the  movements 
involving  both  the  upper  and  lower  extremities,  neck,  and  face.  An 
examination  of  the  eyes  under  atropia  revealed,  in  addition  to  the  con- 
vergent strabismus,  hyperopia  of  1.50  D.  in  both  eyes.  A  cure  was 
effected  by  correcting  the  refractive  error  and  tenotomizing  the  internal 
rectus.  De  Schweinitz  examined  50  cases  of  chorea  in  the  Orthopedic 
Hospital  and  Infirmary  for  Nervous  Diseases,  and  in  77  per  cent,  of 
these  patients  hyperopia  and  hyperopic  astigmatism  were  the  pre- 
dominating condition  of  the  refraction,  but  "  evidence  seems  lacking 
that  the  refraction  error  is  the  basal  cause  of  the  chorea."  Of  118 
cases  examined  by  Stevens  in  his  private  practice,  all  but  5  had  defective 
refraction,  and  a  considerable  number  muscular  insufficiencies.^® 

Eoosa  ^^  says  that  of  118  cases  of  chorea,  78  had  hyperopia,  13 
had  hyperopic  astigmatism,  5  had  mixed  astigmatism,  6  were  myopic, 
and  11  had  myopic  astigmatism. 

These  figures,  and  others  of  like  import,  simply  show  that  refrac- 
tion errors  are  extremely  common  among  choreics,  but  they  do  not  prove 
that  ametropia  is  the  primary  causative  factor  in  producing  chorea. 
It  is,  however,  reasonable  to  suppose  that  accommodative  or  extrinsic 
muscular  strain,  like  irritation  in  any  other  part  of  the  body,  may 


"Boston  Medical  and  Surgical  Journal,  February  20,  1890. 

"The  reader  is  referred  to  the  remarkable  array  of  case  histories  in  his  book 
on  Functional  Nervous  Diseases. 

"  Transactions  of  the  New  York  Academy  of  Medicine. 


XEUEOSES  OCCxVSIONED  BY  EYE-STKAI:N'         803 

aggravate  the  symptoms  of  chorea  or  any  other  nervous  affection.  Eye- 
strain is  a  waste  of  nerve  force  and  escape  of  energy,  a  consumption  of 
power  so  easily  prevented,  so  amenable  to  treatment,  that  whenever  even 
a  suspicion  of  its  presence  exists  an  examination  should  be  demanded. 

VERTIGO. 

Vertigo,  subjective  or  objective,  is  a  symptom  of  many  diseases, 
organic  and  of  known  pathology,  and  of  functional  affections  which 
have  no  recognizable  or  defined  lesions.  It  is  found,  for  instance,  in 
disease  of  the  inner  ear,  irritative  intracranial  affections,  disease  of  the 
heart  and  blood-vessels,  of  the  blood  (toxaemia),  and  other  affections  by 
which  equilibration  is  temporarily  or  permanently  destroyed. 

Vertigo  may  be  reflex.  Mills  says,  "  Irritation  may  be  reflected  to 
the  apparatus  of  equilibration  from  almost  any  part  of  the  body,  near 
or  far.  Reflex  vertigos  are  termed  ocular,  aural,  nasal,  pharangeal, 
gastric,  gastro-intestinal  hepatic,  uterine,  or  ovarian,  according  to  the 
organ  which  seems  to  be  the  fountain-head,  but  if  they  are  truly  reflex 
disorders  the  mechanism  of  their  production  is  practically  the  same 
in  all  cases, — ^namely,  a  disturbance  of  equilibrium  produced  by  the 
reflection  of  an  abnormal  stimulus  upon  a  more  or  less  non-resisting 
equilibratory  apparatus."  It  will  be  seen  that  the  presence  of  vertigo 
or  dizziness  is  of  little  value  in  determining  the  nature  of  the  under- 
lying affection.  The  diagnosis  is,  necessarily,  often  one  of  exclusion. 
The  appearance  of  vertigo  incidently  with  the  performance  of  the  func- 
tion of  one  or  more  organs  of  the  body  will,  however,  often  indicate  its 
probable  origin. 

Ocular  vertigo  is  the  result  of  causes  identical  with  those  that  are 
active  in  giving  rise  to  other  symptoms  of  irritation  or  exhaustion  of 
the  nervous  system, — ^namely,  accommodative  strain  and  muscular  insuf- 
ficiencies. Any  uncorrected  error  of  refraction,  or  presbyopia,  or  hetero- 
phoria  in  an  individual  predisposed  to  nervous  affections  may  be  respon- 
sible. Vertigo  occurs  in  about  12  per  cent,  of  all  cases  of  asthenopia, 
including  both  accommodative  and  muscular,^ ^  in  which  no  disease  of 
the  visual  apparatus  can  be  shown.  It  is  common  also  in  those  that 
have  asthenopia  from  wearing  inaccurately  adapted  lenses,  either  in 
the  strength  of  the  lens  or  in  the  adjustment  of  the  axis  of  the  cylinder. 

That  vertigo  may  be  a  symptom  of  eye-strain  was  shown  by  Thom- 
son and  later  by  the  joint  papers  of  Thomson  and  Weir  Mitchell. 
Their  statements  have  been  verified  in  thousands  of  cases,  and  are 
being  daily  shown  to  be  true  in  the  routine  work  of  the  oculist. 

"  Marlow,  New  York  Medical  Journal,  July  13,  1897,  gives  29  per  cent. 


804  THE    EYE    AND    NERVOUS    SYSTEM 

Ocular  vertigo  is,  with  few  exceptions,  associated  with,  or  conse- 
quent upon,  the  use  of  the  eyes  for  near  work.  It  is  purely  suhjective, 
the  patient  becoming  dizzy  and  feeling  as  though  he  would  fall.  Ob- 
jective vertigo,  or  the  apparent  movement  into  unreal  or  unnatural 
positions  and  relations  of  stationary  objects,  is  rare.  Ocular  vertigo  is 
momentary,  disappearing  with  the  cessation  of  the  use  of  the  eyes  and 
recurring  with  resumption  of  work.  The  relation  of  its  oncoming  to 
the  performance  of  close  work  depends,  as  in  other  forms  of  asthenopia, 
upon  the  relative  strength  of  the  accommodation  and  of  the  extrinsic 
muscles,  as  compared  to  the  degree  of  the  ocular  defect.  The  condition 
of  the  general  health  has  also  much  to  do  with  the  advent  and  fre- 
quency of  the  attacks  of  dizziness.  The  attacks  recur  with  greater 
frequency  in  those  who  are  weakened  from  disease  or  overwork,  who 
lead  sedentary  lives,  who  are  confined  many  consecutive  hours  to 
their  desk  or  sewing-machine,  or  who  have  some  other  affection 
than  asthenopia.  And  conversely,  the  attacks  are  less  severe  and  fre- 
quent in  the  healthy  and  well-fed  and  among  those  who  observe  the 
rules  of  hygiene. 

Dizziness  seldom  exists  alone  as  a  symptom  of  eye-strain,  but  is 
associated  with  local  asthenopia  and  other  reflexes,  particularly  nausea. 
The  gastric  disturbance  may  be  secondary  to  the  v?rtigo  or  primarily 
induced  by  the  cause  of  the  vertigo.  The  combination  of  dizziness, 
nausea,  and  vomiting  is  not  accidental,  but  an  intimation  of  the  asso- 
ciation of  eye-strain  with  functional  stomach  disorder.  All  these  symp- 
toms may  be,  of  course,  the  result  of  eye-strain  alone,  particularly  when 
they  occur  with,  or  after,  use  of  the  eyes. 

Immediately  preceding  and  during  the  attacks  of  vertigo,  the  con- 
dition of  the  cerebral  circulation  is  doubtful.  It  is  i)robably  not  the 
same  in  all  sufferers,  and  not  always  the  same  in  one  individual. 
^Miether  it  be  one  of  anaemia  or  hypera?mia,  the  circulatory  disturbance 
is  momentary  and  probably  Umited  to  an  extremely  small  central  area. 
UjKjn  removal  of  the  cause  or  cessation  of  the  use  of  the  eyes,  the  dizzi- 
ness is  allayed,  l)ecause  the  cerebral  disturbance,  whatever  it  may  be, 
is  removed.  The  effect  of  repeated  irritation  of  the  centre  of  equilibra- 
tion is  deleterious,  and  while  for  a  time  the  affection  may  be  functional, 
constant  repetition  must  lead  to  organic  change  with  permanent  loss 
of  normal  function  and  deterioration  of  the  mental  faculties. 

Diagnosis. — The  characteristic  features  of  ocular  vertigo  are  its 
subjectiveness,  transitoriness,  and  association  with  the  act  of  vision. 
In  order  to  test  its  ocular  origin,  the  patient  should  abstain  from  all 
sources  of  eye-strain,  either  by  voluntarily  giving  up  the  use  of  the 


LEUKOSES  OCCASIONED  BY  EYE-STRAm         805 

eyes  or  by  induced  paralysis  of  accommodation.  Should  the  vertigo 
persist,  the  cause  must  be  looked  for  elsewhere  than  in  the  visual  appa- 
ratus. The  subsidence  of  the  vertigo,  on  the  other  hand,  indicates  that 
it  is  of  ocular  origin.  Vertigo,  dependent  upon  errors  of  refraction 
and  functional  anomalies  of  the  muscles,  is,  strictly  speaking,  ocular 
vertigo,  and  should  not  be  confounded  with  the  vertigo  of  organic  dis- 
ease of  the  ocular  nerves,  in  which  the  lesion  is  orbital  or  of  the  cerebro- 
spinal system.  For  example,  hemianopsia,  limited  fields  of  vision, 
dijDlopia,  the  apparent  distortion  of  objects  by  imperfect  retinal  images, 
hallucination  of  vision,  etc.,  frequently  are  associated  with  vertigo. 
Vertigo  of  brain  tumor,  in  which  the  optic  or  other  ocular  nerves  are 
involved,  is  also  not  to  be  classified  as  ocular  vertigo. 

Treatment. — Correction  of  errors  of  refraction  and  the  restoration 
to  balance  of  the  extrinsic  muscles  are  imperative.  Rest  of  the  eyes 
by  abstinence  from  the  usual  occupations,  tonics,  and  other  general 
measures,  will  give  temporary  relief,  but  permanent  cure  can  be  estab- 
lished only  hy  i-emoval  of  the  defect  of  the  visual  apparatus  which  is 
the  cause  of  the  eye-strain  and  vertigo. 

A  few  illustrative  examples  from  the  journals  and  from  my  own 
histories  are  appended. 

Colburn  ^^  quotes  the  case  of  a  farmer  who  suffered  from  severe 
headaches,  vertigo,  and  nausea.  He  was  examined  under  atropia,  and 
was  found  to  have  a  hypermetropic  astigmatism  in  each  eye,  besides  a 
hyperphoria  of  one  degree  and  a  manifest  esophoria  of  eight  degrees 
in  the  right  eye.  He  was  given  his  correction  and  advised  to  return 
if  the  symptoms  did  not  abate.  Four  and  a  half  years  later  he  appeared, 
not  having  complained  of  any  discomfort  until  recently.  Xo  change 
was  found  necessary  in  his  glasses.  The  hyperphoria  had  increased, 
however,  to  four  and  a  half  degrees,  but  the  esophoria  remained  the 
same.  The  right  superior  rectus  was  tenotomized  and  the  inferior  of 
the  same  eye  advanced,  the  result  being  that  a  hyperphoria  of  one  degree 
remained  and  the  esophoria  almost  entirely  disappeared.  He  was  seen 
two  months  later,  when  he  stated  that  the  symptoms  from  which  he 
suffered  had  not  returned. ' 

The  same  author  ^*  gives  details  of  four  classes  of  intractable  asthen- 
opic  headaches,  with  insomnia,  vertigo,  indigestion,  and,  in  one  case, 
petit  mal,  persisting  in  spite  of  careful  refraction.  Correction  of  mus- 
cular anomalies  gave  relief  in  four  cases. 

Eulton  (in  North westerii  Lancet,  1900)  reports  the  case  of  a  busi- 

'•^  Clinical  Lectures  on  Diseases  of  the  Eye. 
"Clinical  Review,  May,  1901. 


806  THE    EYE    AND    NERVOUS    SYSTEM 

ness  man  who  suffered  from  almost  constant  vertigo.  For  two  years 
lie  was  treated  at  sanitariums  in  America  and  Europe  without  obtaining 
relief.  Examination  showed  a  heterophoria.  A  tenotomy  of  the  in- 
ferior rectus  was  done,  with  the  result  that  all  symptoms  of  vertigo 
completely  disappeared,  and,  up  to  one  j-ear  after  the  operation,  had 
not  returned. 

Carter  ^^  reports  the  case  of  a  young  man,  the  son  of  a  wealthy 
banker,  who  was  obliged  to  relinquish  his  studies  at  Oxford  because  of 
his  inability  to  read  without  incurring  diplopia,  followed  by  vertigo, 
and,  if  continued  efforts  were  made  to  read,  by  sickness,  palpitation  of 
the  heart,  and  intense  headache.  Both  eyes  were  found  to  be  myopic, 
but  otherwise  the  media  and  fundi  were  normal.  He  was  refracted 
and  suitable  glasses  adjusted,  with  the  result  that  all  symptoms  froia 
which  he  had  suffered  disappeared  almost  immediately  and  had  not 
returned  when  seen  about  three  months  later. 

Case  1.  Mrs,  T.,  aged  29  years.  Complained  of  attacks  of  ver- 
tigo, lasting  thirty  minutes,  followed  by  severe  headache  and  occasion- 
ally by  vomiting.  The  dizziness  commenced  at  15  years  of  age, 
and  recurred  every  week  or  twice  a  week  for  the  past  12  years.  The 
attacks  were  attended  with  numbness  of  the  fingers.  V=G/6.  R.  hyper- 
phoria 1/2°,  esophoria  1/0°.  She  was  relieved  by  full  correction  of 
HAs. 

Case  2.  Mrs.  T.,  48  years,  momentary  dizziness  while  working 
as  a  weaver  or  while  walking.  The  trouble  commenced  simultaneously 
with  the  wearing  of  glasses  for  the  correction  of  compound  HAs.,  and 
presbyopia.  Cure  was  effected  by  addition  of  the  prismatic  correction 
of  2°  of  hyperphoria. 

Case  3.  Mrs.  K.,  46,  nearly  constant  headache;  efforts  to  see  near 
objects  invariably  induced  vertigo,  nausea,  and  vomiting.  Correction 
of  high-grade  mixed  astigmatism  and  presbyopia  gave  entire  relief 
from  her  distressing  symptoms. 

Case  4.  Mrs.  I.,  27.  Could  not  read  half  an  hour  without  head- 
ache and  dizziness.  She  had  always  considered  herself  nervous  and 
excitable.  Headache  and  vertigo  were  cured  by  25  D.  less  than  full 
correction,  which  under  cyclopegia  was  found  to  be 

R.  +  .750+  .25  ax.    IS" 
L.  +  .50  O  +    1.  ax.  165° 

Case  5.  Mary  F.,  11.  Neuralgia  in  left  side  of  face  and  dizziness, 
aggravated  by  near  use  of  the  eyes.     Print  seemed  to  grow  small  and 

"  Transactions  of  the  Clinical  Society  of  London,  vol.  viii. 


LEUKOSES  OCCASIONED  BY  EYE-STRAI^^         807 

recede.     She  also  suffered  from  constipation,  pain  in  back  of  neck  and 
across  the  shoulders.     These  symptoms  were  cured  by : 

R.  +  2.25  ax.  90° 

L.  +    .50  0  +  2.25  ax.  90° 

Multiplication  of  cases  is  unnecessary.  It  is  universally  admitted 
that  vertigo  may  be  a  symptom  of  ocular  strain,  and  it  is  cured  by 
lenses  to  correct  refraction  and  presbyopia,  by  prisms,  and  by  opera- 
tions designed  to  overcome  abnormal  tendencies  of  the  ocular  muscles. 
It  is,  however,  interesting  to  note  that  practically  identical  ocular  errors 
give  rise  to  no  reflexes  in  some  patients,  while  in  others  they  may  cause 
vertigo,  gastric  disturbances,  or  a  complexity  of  symptoms,  which  ap- 
parently have  no  connection  with  the  use  of  the  eyes.  For  example, 
May  F.,  a  sister  of  Case  5,  had  much  the  same  error  with  entirely 
different  symptoms.  Her  refraction  was  R.  -]-  150  O  +  1.50  ax.  90°  ; 
L.  -|-  1  O  -)-  3  ax.  105°.  She  complained  only  of  occasional  head- 
ache and  confusion  of  print  in  reading.  Edward  F.,  a  brother,  made 
no  complaint  at  all  except  imperfect  vision,  and  his  defect  was 

R.  — .50  o  +  450  ax.  100° 
L.  —.50  o  +      1  ax.    90° 

To  revert  to  a  principle,  already  mentioned,  the  optical  or  muscular 
defect  does  not  decide  the  character  of  the  reflexes. 

GASTRIC    DISTURBANCES. 

The  gastric  symptoms  of  ocular  strain  are  those  of  so-called 
functional  stomach  affections, — namely,  flatulency,  delayed  digestion, 
hyperacidity,  nausea  and  vomiting,  and  the  natural  sequence  of  indi- 
gestion —  constipation.  They  manifest  themselves  in  different  in- 
tensity, according  to  the  kind  and  degree  of  optical  defect  and  accord- 
ing to  the  individual  disposition.  They  may  develop  in  persons  whose 
digestive  organs  would  be,  under  normal  ocular  conditions,  in  perfect 
functional  activity,  or  in  those  whose  digestive  tract  is  disordered,  as  a 
result  of  other  cause  than  ocular.  They  also  occur  as  the  result  of 
disease  of  the  digestive  tract,  and  are  aggravated  by  the  use  of  the 
eyes.  The  history  of  their  appearance,  severity,  continuance,  and 
recovery  corresponds  in  every  particular  to  the  history  of  other  forms 
of  asthenopic  and  reflex  neuroses,  appearing  simultaneously  with  the 
commencement  of  close  (or  far)  application  of  the  eyes,  and  in  a  few 
minutes  increase  in  intensity  until  the  occupation  is  discontinued  and 
the  patients'  eyes  are  put  at  rest.  The  symptoms  persist  during  the 
period  of  eye-strain,  and  recover  only  after  abstinence  from  work  and 
possibly  seclusion  in  a  dark  room. 


808 


THE    EYE    AND    NERVOUS    SYSTEM 


Ophthalmic  ganglion 
Carotid  plexus 
Infraorbital 


Second  division 
lifth  nerve 


First  division  fifth  nerve 

,  First  division  third  nenre 
,  First  division  fifth  nerve 

Nucleus  third  nerve 

Nucleus  fifth  nerve 


Meclcel's  ganglion 


Superior  cervical 
ganglion 


Middle  cervical  ganglion 


Inferior  cervical  ganglion 


Ganglion  of  the 
root 
Medulla  of  pneumogas- 

tric  nerve 
First  cervical  nerve 
Second  cervical  nerve 


Spinal  cord 


Pneumogastric  nerve 


Stomach 


Fig.  1.— a  schematic  diagram  of  the  cerebrospinal  and  sympathetic  nerve  connections  between  the 

eye  and  the  stomach. 


IS^EUROSES  OCCASIONED  BY  EYE-STRAI:N'         809 

The  schematic  anatomical  explanation  upon  the  opposite  page 
(Fig.  1)  of  the  origin  of  the  gastric  disturbances  from  irritation 
of  the  fifth  pair  of  nerves  helps  to  elucidate  their  ocular  causes.  It 
is,  indeed,  applicable  to  lesions  occurring  anywhere  in  the  course  of 
the  fifth  nerve. 

That  indigestion  may  be  due  to  eye-strain  there  can  be  no  reason- 
able doubt.  Too  many  authentic  cases  are  on  record  to  permit  of  scepti- 
cism. Unquestionably,  hasty  eating,  imperfect  mastication,  ice-water, 
resumption  of  mental  or  physical  work  immediately  after  partaking 
of  heavy  meals,  indifference  to  the  laws  of  hygiene,  and,  in  a  word,  too 
little  thought  of  health,  are  contributory  causes.  Nerve  energy  that 
should  be  spent  in  stimulating  the  different  organs  of  the  body  to  per- 
form their  functions  is  diverted  to  the  visual  apparatus.  Focussing 
rays  of  light  from  near  or  distant  objects  and  the  adjustment  of  the 
musculature  of  both  eyes  for  single  binocular  vision  for  more  or  less 
prolonged  periods  demand  an  excess  of  nerve  force  that  cannot  be 
spared  without  sacrificing  the  functions  of  other  organs.  The  causative 
relation  of  eye-strain  is  seldom  suspected.  Treatment  directed  to  the 
abdominal  organs  gives  temporary  benefit  only.  Permanent  recovery 
ensues  only  after  correction  of  all  optical  defect. 

The  ocular  factor  in  the  etiology  of  gastric  disorder  has  been  insisted 
upon  by  oculists  for  many  years. 

The  following  histories  are  not  uncommon :  Tn  the  article  by  Thom- 
son,^^  already  alluded  to,  nausea  is  included  in  the  description  of 
asthenopia  induced  by  astigmatism. 

M.  X.,  lawyer,  aged  30,  complained  of  occasional  blurring  of  dis- 
tant vision,  photophobia,  chronic  conjunctivitis,  and  headache,  all  of 
which  symptoms  he  attributes  to  indigestion.  Under  mydriasis  the 
refraction  was  -|-2  O  -j-l°  ax.  90°.  Correction  of  this  defect  cured 
the  local  asthenopia,  headache,  and  indigestion.  The  patient  had  sub- 
mitted to  medicinal  treatment  for  indigestion  for  years,  and  was  not 
a  little  surprised  at  the  cure  by  glasses.  Miss  M.  complains  that  she 
cannot  use  her  eyes  day  or  night  without  pain.  Is  nervous,  irritable, 
and  dyspeptic.  After  correction  of  HAs.,  muscular  exercise  by  prisms, 
nerve  tonics,  and,  finally,  tenotomy  of  one  of  the  superior  recti  muscles, 
she  reported  that  she  could  read  or  sew  several  hours  comfortably  and 
felt  herself  "  a  new  creature." 

Gould  ^"^  gives  the  history  of  a  case  of  flatulent  dyspepsia  of  twenty 
years'  standing  cured  by  the  application  of  proper  lenses.     The  patient 

^°  Medical  News  and  Library. 

"  Aledical  and  Surgical  Reporter,  February.  1889. 


810  THE    EYE    AND    NERVOUS    SYSTEM 

complained  of  certain  ocular  and  cerebral  troubles,  asthenopia,  diffi- 
culty in  near  work  and  the  like,  besides  the  gastric  disorder,  though 
it  was  the  latter  which  gave  her  the  greatest  cause  for  worry.  Medi- 
cines never  gave  her  any  relief.  There  was  absolutely  no  hysterical 
element  in  the  case.  The  reflection  of  the  ophthalmoscopic  mirror  into 
the  eye  caused  such  an  instantaneous  and  frightful  paroxysm  of  eruc- 
tation that,  for  fear  that  violent  emesis  was  about  to  take  place,  the 
examination  had  to  be  deferred.  It  was  highly  interesting  to  note  that 
the  application  of  suitable  lenses  in  a  trial  frame  as  effectually  quieted 
the  gastric  volcano  as  the  ophthalmoscopic  mirror  had  caused  it. 
Glasses  were  prescribed,  with  the  result  that  the  patient  improved  each 
day.  She  stated  that  she  could  not  leave  the  glasses  off  a  minute  w^ith- 
out  gastric  symptoms.  That  the  condition  was  purely  a  reflex  neurosis 
was  clearly  evident,  because  removal  of  the  glasses  from  before  her  eyes 
while  fixing  an  object  caused  the  eructations  to  reappear  like  a  flash, 
and  again  disappear  when  the  glasses  were  put  in  place.  Knapp  says, 
in  the  treatment  of  so-called  gastric  troubles,  flatulent  and  other  dys- 
pepsias, indigestion,  etc.,  the  neutralization  of  errors  of  refraction  and 
restoration  of  normal  muscle  balance  yield  such  good  results  that  oph- 
thalmogists  are  justified  in  constantly  advising  ocular  treatment. 
Ayres  ^*  reports  a  case  of  a  healthy,  well-developed  boy  of  15  years, 
who  complained  of  nausea  when  in  school,  in  church,  or  in  any  assem- 
bly of  persons.  The  sensation  became  so  marked  in  school  that  he 
was  obliged  to  discontinue  his  studies.  Ophthalmoscopic  examinations 
revealed  a  hyperopia  of  1.5  D.  in  each  ,eye.  He  was  refracted  and 
ordered  a  +1  D.  to  be  worn  constantly.  The  results  were  immediately 
manifest.  The  nausea  was  at  once  relieved.  Parker  ^®  says  that  while, 
as  a  general  rule,  the  symptoms  produced  by  refractive  errors  and  ocular 
imbalance  are  varied,  the  headaches  are  characteristic.  The  migraine 
produced  by  refractive  errors  begins  as  frontal,  extends  to  the  top  of  the 
head,  and  finally  settles  in  the  back  of  the  neck.  He  quotes  several 
interesting  cases: 

1.  A  man,  aged  35,  complained  of  being  nauseated  and  of  having 
severe  headaches  for  two  or  three  days  each  time  he  audited  his  books, 
which  was  every  month.  So  severe  were  these  attacks  and  so  directly 
referable  to  his  near  work  that  he  was  obliged  to  refrain  from  excessive 
use  of  his  eyes  for  the  balance  of  the  month.  He  had  a  low  myopia 
for  which  he  used  his  correction  constantly.  Muscle  test  showed  one 
degree  of  exophoria.    By  orthopic  exercises  the  divergence  disappeared, 

"  Cincinnati  Lancet  Clinic. 
•Physician  and  Surgeon,  May,  1902. 


NEUROSES  OCCASIOXED  BY  EYE-STRAIN         811 

as  did  the  symptoms,  after  six  weeks  of  training.  This  patient  has 
been  under  observation  for  seven  years,  and  in  that  time  he  has  been 
entirely  free  from  his  okl  attacks. 

2.  A  girl,  aged  10,  suffered  daily  from  intermittent  headaches, 
nausea,  and  vertigo  for  six  years.  Examination  showed  an  exophoria 
of  9°  ;  although  glasses  were  given  to  correct  the  astigmatism  pres- 
ent, and  adduction  was  developed  by  exercise,  the  symptoms  continued. 
A  complete  tenotomy  of  the  external  rectus  reduced  the  muscle  error  to 
naught.  It  later  went  back  two  degrees,  but  no  symptoms  developed  and 
she  has  had  perfect  comfort  ever  since  the  day  of  operation. 

GENERAL    ASTHENOPIA. 

Asthenopia,  painful  use  of  the  eyes,  is  common  to  all  forms  of 
refractive  defects  and  muscular  anomalies,  and  is,  exceptionally,  present 
in  emmetropia  and  muscular  equilibrium.  One  or  the  other  of  these 
conditions  is  necessary  for  its  development,  but,  as  a  rule,  both  factors 
are  coexistent,  and  in  most  asthenopics, — namely,  a  defect  in  the  visual 
apparatus  and  an  impressionable  and  responsive  nervous  system.  The 
use  of  emmetropic  eyes  under  unhygienic  conditions  of  light,  improper 
physical  posture,  unusual  or  unnatural  direction  of  fixation  in  healthy 
individuals,  will  cause  asthenopia,  which  assumes  an  exaggerated  ex- 
pression in  those  who  are  infirm  or  predisposed  to  muscular  weak- 
ness. 

Asthenopia  is  accommodative  or  muscular.  (It  may  be  both  accom- 
modative and  muscular.)  In  the  former  case  the  power  of  accommo- 
dation is  vmable  to  cope  with  the  disadvantages  of  ametropie  refraction ; 
in  the  latter  the  nerve  stimulation  to  the  extra-ocular  muscles  or  the 
muscles  themselves  are  defective.  Both  forms  give  rise  to  local  and 
general  symptoms  which  may  be  logically  referred  to  ocular  causes  or 
which  may  be  so  independent  of  the  use  of  the  eyes  that  their  ocular 
origin  is  only  suspected  after  exclusion  of  other  possible  causes. 

The  Acuity  of  Vision  in  Accommodative  Asthenopia. — Lowered 
vision,  or  the  inability  to  perceive  and  name  letters  or  objects  that  are 
small  enough  to  be  included  in  the  5'  visual  angle,  in  any  distance  be- 
tween the  near-point  and  the  so-called  infinity  (6m.),  is  not  always, 
and  indeed  in  the  majority  of  cases,  associated  with  or  a  symptom  of 
asthenopia.  Its  presence  is,  however,  indicative  that  the  visual  appa- 
ratus is  imperfectly  functionating.  The  defect  may  lie  in  opacity  of 
the  media,  in  disease  of  the  optic  nerve,  from  its  deep  origin  in  the 
brain  to  its  expansion  at  the  fundus  of  the  eye,  or  in  an  error  of  refrac- 
tion.    Eliminating  disease,  as  irrelevant  to  the  subject  of  this  chapter, 


812  THE    EYE    AND    NERVOUS    SYSTEM 

we  are  limited  to  a  consideration  of  Hyperopia,  Myopia,  and  Astig- 
matism. 

(a)  Hyperopia  (II). — The  acuity  of  vision  depends  upon  the  de- 
gree of  II.,  the  power  of  accommodation,  and  the  distance  from  the 
eye  at  which  the  test  is  made.  Young  persons  in  good  health  may 
conceal,  by  ciliary  contraction,  2  to  3  D.  of  hyperopia  for  all  distances. 
3  D.  may  he  consistent  with  full  acuity  for  far,  but  not  for  near. 
Higher  hyperopia  than  3  I),  in  the  great  majority  of  persons,  even  the 
very  young,  means  lowered  vision,  and  is  therefore  one  of  the  symp- 
toms of  an  optical  defect.  In  advancing  years  the  contractility  of  the 
ciliary  muscle  becomes  reduced  and  the  elasticity  of  the  lens  lessened. 
Latent  II.  becomes  manifest  H.,  and  the  acuity  of  vision  appreciably  de- 
clines. Between  forty  and  forty-five  years  of  age  in  Em.  distant  V.  may 
remain  full,  but  the  near-point  has  receded,  and  full  acuity  for  the  ordi- 
nary reading  distance  is  no  longer  present,  or,  if  present,  is  maintained 
for  a  few  minutes  only.  In  H.  the  distant  V.  always  suffers  with  the 
receding  of  the  near-point.  In  m^'opic  refraction,  distant  and  near 
vision  are  practically  unchanged  by  failure  of  accommodation. 

{h)  Myopia  (M). — It  will  be  seen  from  these  statements  that  the 
acuity  of  vision  depends  in  part  upon  the  distances  at  which  the  tests 
are  made,  and  that  no  examination  is  complete  unless  the  determination 
of  the  position  of  the  near-point  is  included.  Lowered  acuity  for  near 
is  suggestive  of  hyperopic  refraction,  although  myopic  astigmatism  may 
also  be  a  cause.  In  M.  vision  is  always  less  than  Q>/Q.  The  ability 
to  see  small  objects,  such  as  test  types,  at  the  distance  at  which  they 
are  seen  by  emmetropic  eyes,  the  accommodation  being  abolished  either 
pathologically  or  therapeutically,  is  not  incompatible  with  myopia  of 
low  degree.  Myopes,  however,  usually  read  at  a  distance  nearer  than 
the  degree  of  M.  would  warrant,  since  the  convergence  necessary^ 
in  the  associated  function  of  both  eyes,  induces  contraction  of  the 
ciliary  muscles,  and  this,  in  turn,  increases  temporarily  the  M.  Re- 
laxation of  the  accommodation  may  not  be  complete  even  for  6  M., 
particularly  in  individuals  accustomed  to  long-continued  near  work. 
Ilencc  !M.  will  l)e  apparently  greater  than  the  degree  shown  under 
complete  cyclo])legia.  I  have  seen  a  remarkable  illustration  of  this 
fact.  A  woman,  aged  35,  had  binocular  V  =  6/GO  corrected  to  6/G 
by  — 3  D.  When  she  fixed  with  one  eye  and  allowed  the  other  to 
diverge,  V.  improved  to  6/6  without  a  glass. 

(c)  Astigmatism  (As). — Lowered  visual  acuity  is  found  in  all 
grades  of  !MAs.  and  IIAs.  of  1  D.  or  over.  In  spasm  of  accommodation, 
by  which  IIAs.  is  apparently  transformed  into  ^MAs.,  the  acuity  of  vision 


NEUROSES  OCCASIONED  BY  EYE-STRAIN         813 

is  below  the  normal  and  is  improved  by  a  minus  cylinder  before  cyclo- 
plegia,  a  plus  cylinder  during  cycloplegia.  In  mixed  As.  the  myopic 
meridian  may  be  made  more  myopic  and  the  hyperopic  meridian  less 
hyperopic  by  contraction  of  the  muscle  of  accommodation.  In  either 
case  the  acuity  of  vision  is  reduced. 

Full  acuity  of  vision  in  the  distance  is  present  in  most  cases  of 
accommodative  asthenopia.  Therefore,  the  test  so  often  made,  to  de- 
termine whether  the  eyes  are  at  fault  by  test  cards  as  6  M.,  is  misleading, 
and  the  exclusion  of  the  eyes  as  a  causative  factor  because  V  =  6/6 
is  incorrect.  Should,  however,  the  combination  exist  of  full  visual 
acuity  and  muscular  balance  for  all  distances,  it  is  probable  that  Em. 
is  present  and  the  symptoms  of  which  the  patient  may  complain  are 
not  of  ocular  origin. 

The  visual  acuity,  therefore,  is  no  criterion  of  the  presence  of 
accommodative  asthenopia,  since  asthenopia  may  be  a  symptom  either 
in  normal  or  defective  vision.  It  is  claimed  by  some  writers,  how- 
ever, that  asthenopia  is  more  frequently  present  in  the  former  than  in 
the  latter,  and  consequently  is  to  be  attributed  to  the  constant  effort 
of  accommodation  necessary  to  overcome  the  slighter  degrees  of  ametro- 
pia by  which  vision  is  maintained  at  the  normal  standard.  Yet  it 
should  be  borne  in  mind  that  one  additional  factor  enters  into  the 
eye-strain  in  individuals  who  have  eyes  so  defective  that  the  accommo- 
dation is  powerless  to  entirely  overcome  the  defect.  The  contraction 
of  the  ciliary  muscle  continues  as  persistently  in  the  higher  as  in  the 
lower  defects,  and  the  imperfect  vision  is  only  partly  or  not  at  all 
restored.  The  mental  effect  of  the  unclear  retinal  images  should  not 
be  disregarded.  The  mind  is  erroneously  informed  of  the  actual  size, 
form,  and  relation  of  objects,  and  is  led  to  form  wrong  conclusions, 
the  effects  of  which  are  far-reaching.  The  discovery  of  this  uncon- 
sciously misguided  attitude  towards  the  world  exerts  a  baneful  influ- 
ence upon  the  entire  system,  tends  to  melancholy  and  depression  of 
spirits,  social  exclusion,  and  helps  towards  the  development  of  a  mis- 
anthrope. 

Vision  in  Muscular  Asthenopia. — The  effect  on  vision  of  the  de- 
grees of  muscular  defect  is  analogous  to  that  of  the  degree  of  ametropia. 
Vision  may  be  single  (binocular)  for  all  distances  and  at  all  times; 
it  inay  be  single  ordinarily  and  blurred  occasionally,  Avhen  the  blurring 
may  be  taken  as  evidence  that  the  weaker  set  of  muscles  are  momentarily 
incapable  of  maintaining  the  tension  necessary  to  resist  the  opposing 
muscles.  Simultaneous  with  the  deviation  of  the  visual  lines,  the  object 
looked  at  becomes  indistinct  in  outline  or  appears  double.     Vision  may 


814  THE    EYE    AND    NERVOUS    SYSTEM 

be  permanently  double,  indicating  insuperable  deviation  from  each 
other  of  the  visual  lines.  Asthenopia  of  great  severity  usually  accom- 
panies the  first  and  second  or  latent  forms  of  insufficiency  and  dis- 
turbed mental  equilibrium,  from  the  presence  of  the  two  images,  is  the 
common  result  of  the  third  form. 

The  Facial  Expression  in  Asthenopia. — Stevens  and  others  have 
called  attention  to  the  modification  of  the  facial  expression,  induced 
by  the  conscious  or  unconscious  participation  of  some  of  the  facial  mus- 
cles in  the  accommodative  and  muscular  effort  to  secure  clear  and  single 
images.  The  facial  expression  is  determined  in  part  by  the  ocular 
defect.  For  example,  the  myope  by  contraction  of  the  orbicularis  lessens 
the  vertical  diameter  of  the  commissure,  and  by  cutting  off  the  light 
rays  that  enter  the  periphery  of  the  pupil,  the  circles  of  diffusion  that 
fall  upon  the  retina  are  reduced  in  size.  The  contracting  muscle  also 
modifies  the  curvature  of  the  cornea  by  pressure  upon  the  vertical  diam- 
eter. The  same  statement  holds  for  myopic  astigmatic  when  the  axis 
of  the  astigmatism  is  horizontal,  or  a  few  degrees  removed  from  the 
horizontal.  Should  the  axis  be  vertical,  or  nearly  so,  better  vision  is 
obtained  by  turning  the  head  on  its  vertical  axis.  In  heterophoria  the 
muscles  of  the  brow  are  contracted,  lining  the  forehead  with  vertical 
or  horizontal  furrows.  In  heterotropia  the  orbicularis  of  one  eye  is 
maintained  in  a  state  of  constant  contraction  in  order  to  exclude  that 
eye  from  the  act  of  vision,  or  the  head  is  rotated  in  its  vertical  or  hori- 
zontal axis  in  the  direction  of  the  weaker  set  of  muscles.  Thus,  in 
paralysis  of  the  third  nerve  of  the  left  eye,  the  eye  is  diverged,  and 
the  false  image  can  be  merged  into  the  true  by  turning  the  face  towards 
the  right. 

"  Wrinkles,"  or  "  crows'  feet,"  at  the  external  angles  of  the  com- 
missures are  in  many  persons  an  indication  of  eye-strain.  In  exagger- 
ated cases  of  ametropia  and  muscle  imbalance,  the  facial  muscular  con- 
tractions assume  the  proportion  of  deformities,  and  are  suggestive  of  the 
expression  of  horror,  fright,  astonishment,  or  other  emotion.  These 
lines  may  become  permanent,  and  are  often  misleading  indications  of 
character  and  disposition. 

Causes. — Errors  of  refraction  and  anomalies  of  the  extraocular 
nmscles  have  been  alluded  to.  They  are  by  far  the  most  frequent  causes 
of  asthenopia  ^^  and  affect  a  large  proportion  of  the  population  of  edu- 
cated communities.     The  causes  are  most  active  between  the  tenth  and 


"  For   correction   of   these   defects   and    the   treatment   of   accommodative   and 
muscular  asthenopia  the  reader  is  referred  to  special  works  on  ophthalmology. 


NEUROSES  OCCASIONED  BY  EYE-STRAIN         815 

fortieth  years  of  life,  although  no  time  of  life  is  exempt.  Hale  ®^  says 
that  muscular  asthenopia  usually  has  its  origin  in  the  brain,  depending 
upon  a  disturbed  innervation  or  possibly,  in  some  cases,  upon  a  weak- 
ness of  the  power  of  fusion.  In  a  small  percentage  of  cases  asthenopia 
depends  upon  either  the  absolute  or  relative  weakness  of  the  adductors 
or  obliques,  or  upon  the  position  of  their  insertions  into  the  sclera.  His 
own  observations  lead  to  the  belief  that  want  of  converging  power  is 
the  chief  cause  of  this  form  of  asthenopia.  Optical  defects  are  of  such 
vast  importance  to  health  and  intellectual  growth  that  it  is  incumbent 
upon  every  parent  or  guardian  of  youth  to  seek  an  examination  of  the 
eyes  of  those  under  their  care. 

Reflex  asthenopia  is  sufficiently  common  to  deserve  more  attention 
than  is  accorded  to  it.  Painful  use  of  the  eyes  may  be  due  to  nasal 
obstruction,  to  dental  caries,  to  throat  and  nose  affections,  and  to  the 
reflected  irritation  from  disturbed  function  of  the  organs  of  the  chest 
and  abdomen.  A  familiar  example  is  asthenopia  from  disease  of  the 
cavities  accessory  to  the  orbit,  such  as  ethmoiditis,  rhinitis,  and  frontal 
sinus  affections,  by  which  the  circulation  of  the  orbit  and  eye  is  ob- 
structed or  the  passage  of  the  tears  through  the  nasal  duct  prevented. 

Constitutional  Asthenopia. — Painful  use  of  the  eyes  may  result  from 
any  physical  or  mental  condition  which  tends  to  lessen  the  physical 
powers  of  resistance  or  to  the  induction  of  irritability  or  undue  im- 
pressionability of  the  nervous  system.  Among  the  former  causes  are 
convalescence  from  illness,  toxaemia,  gout,  rheumatism,  exhaustive 
hemorrhages  (as  in  childbirth),  anaemia,  diseases  of  the  blood,  sedentary 
life,  and  impure  air.  Among  the  latter  are  functional  and  organic 
derangements  of  the  cerebrospinal  system,  resulting  from  disappointed 
ambition  and  desires,  anxieties,  and  a  want  of  proper  relation  between 
financial  income  and  outgo.  The  ocular  defect  in  constitutional  asthe- 
nopia may  be  small  or  entirely  absent.  The  symptoms  depend  less  on  the 
state  of  refraction  or  of  the  muscles,  and  more  upon  a  deficient  muscular 
and  nerve  force.  The  ciliary  and  extraocular  muscles,  like  other 
muscles  of  the  body,  are  unable  to  respond  normally  to  the  demand 
made  upon  them,  and  the  nerve  stimulation  to  the  muscles,  originating 
in  the  consciousness,  is  weaker  than  the  will  which  governs  it.  The  so- 
called  "  retinal"  asthenopia  may  be  regarded  as  another  name  for  con- 
stitutional asthenopia.  The  limit  of  endurance  of  the  retina  to  the 
impression  of  light  rays  and  that  of  the  optic  nerve  to  conveying  the 
retinal  impulse  to  the  cortical  centres  is  reached  in  a  comparatively 

•^  Transactions  of  the  American  Ophthalmological  Society,  vol.  vii. 


81G  THE    EYE    A^^D    :N^ERV0US    SYSTEM 

short  time.  The  retina  and  nerve  become  easily  fatigued  and  soon 
exliausted,  giving  rise  to  confusion  of  images  and  impaired  mental 
conceptions.  The  exhaustion  is  a  symptom  of  the  combination  of  opti- 
cal errors  and  of  constitutional  weakness,  and  is  common  in  nervous 
affections,  independent  of  ocular  error,  such  as  neurasthenia  and  hys- 
teria. "  The  subjects  break  down  easily  and  are  unable  to  continue 
work,  and  have  but  little  initiative."     (Dand.) 

Symptoms. — Etymologically,  asthenopia  means  weak  sight.  Oph- 
thalmologically,  the  Avord  has  come  to  signify  painful  sight.  The  symp- 
toms are  local  and  reflex.  Of  the  former,  pain  constitutes  the  principal 
phenomenon.  It  is  felt  in  the  eye,  in  the  orbit,  through  the  radiations 
of  the  first  and  second  branches  of  the  fifth  nerve,  and  the  first  and 
second  cervical.  It  follows  immediately,  or  not  for  some  minutes  or 
hours  after  using  the  eyes,  either  for  distance  or  in  reading.  It  is  slight 
or  severe,  sharp,  neuralgic,  or  dull  and  boring,  intermittent  or  constant, 
acute  or  chronic.  It  may  have,  apparently,  no  connection  whatever 
with  the  use  of  the  eyes  or  be  originated  or  increased  by  reading  or 
other  near  work.  Occasionally  ciliary  tenderness  is  present,  but  in 
most  cases  the  pain  is  temporarily  lessened  or  wholly  relieved  by  ex- 
ternal pressure  upon  the  eye-balls.  Blurring  of  letters  in  reading  or 
the  needle  in  sewing,  or  confusion  of  points  and  lines  in  any  near  work 
is  due  to  transiently  relaxed  accommodation  to  momentary  suspension 
of  convergence  or  to  exhaustion  of  retinal  energy.  The  time  of  the 
oncoming  of  asthenopia  after  commencement  of  the  near  use  of  the 
eyes  is,  like  other  forms  of  asthenopia,  dependent  upon  the  power  of 
accommodation,  the  relative  strengths  of  the  extrinsic  muscles,  and  the 
tone  of  the  nervous  system.  Ilypersemia  of  the  conjunctiva  and  lach- 
rymation  may  be  associated  with  pain  or  occur  independently  when 
the  eyes  are  subjected  to  strain  in  the  effort  for  the  accurate  definition 
of  images,  particularly  if  long  continued. 

Reflex  Symptoms. — Headache  is  one  of  the  most  constant  and  aggra- 
vated symptoms  of  ocular  strain  in  individuals  under  40  years  of  age. 
It  is  a  reflex  pain  of  muscular  effort  and  arises  from  excessive  or  pro- 
longed contraction  of  the  ciliary  muscle,  from  a  disturbance  of  the 
normal  relation  of  accommodation  to  convergence,  or  from  imbalance 
of  the  external  ocular  muscles,  intrinsic  in  the  muscles  themselves,  or 
in  their  innervation  and  possibly  independent  of  the  refraction  of  the 
eye. 

Location. — Some  authors  ^^  assign  definite  portions  of  the  head  as 


Marlow,  C.  A.  Wood. 


NEUROSES  OCCASrOXED  BY  EYE-STRAIX         817 

the  seat  of  ocular  headache — frontal  or  temporal,  for  instance — in  con- 
tradistinction to  the  location  of  headache  from  other  causes.  Wood 
has  stated  the  order  of  f requenby  to  be  supraorbital,  deep  orbital,  fronto- 
occipital,  and  temporal. 

The  exceptions  to  definite  localization  are  so  numerous  that  but 
little  diagnostic  value  attaches  to  the  location  designated  by  the  patient. 
In  one  hundred  successive  cases  of  accommodative  or  muscular  strain 
under  my  care  sixty-eight  patients  complained  of  headache,  classified 
under  the  descriptive  term  "  general,"  in  twenty  cases  the  pain  was 
frontal,  two  temporal,  and  in  ten  occipital.  The  disproportionately 
large  number  of  general  is  partly  the  result  of  inaccuracy  of  locali- 
zation, observation,  and  expression  on  the  part  of  the  patient,  and  to 
the  dissemination  of  the  pain  throughout  the  head,  from  its  origin 
within  the  orbit.  The  distribution  of  pain  must  be  along  the  sensitive 
nerves  of  the  head,  which  in  their  course  or  termination  are  subjected 
to  irritation.  Hence  the  pain  felt  outside  of  the  orbit  is  reflex.  Orbital 
pain,  that  arising  from  muscular  tension  or  irritation  of  orbital  nerves, 
might  readily  be  referred  to  the  temporal  and  frontal  regions  of  the 
head  through  the  distribution  of  the  fifth  pair  of  nerves.  The  exhausted 
ciliary  muscle,  through  sympathetic  fibres  or  directly,  causes  an  irregu- 
lar discharge  in  the  region  of  the  third  nerve  nucleus  or  an  irritation 
of  the  necleus  itself.  Occipital  pain,  often  a  symptom  of  muscular 
anomaly  and  of  uncorrected  astigmatism,  is  referred,  not  to  the  scalp, 
but  to  underlying  tissues,  and  is  possibly  explained  by  the  presence  of 
localized  congestion  of  the  vessels  and  perivascular  tissues  at  the  base 
of  the  brain,  induced  by  the  effort  to  maintain  balance  of  the  extra- 
ocular muscles  or  by  irritation  of  the  third  nerve  communicated  to  the 
sensitive  nerves  of  the  meninges.  * 

Wilder  ^^  believes  the  vessels  of  the  brain  and  meninges  are  dilated. 
Ocular  headaches  are  due,  according  to  W^ilder  (1)  to  exposure  to  bright 
light  in  cases  of  hypersensitiveness  or  irritation  of  the  retina,  which 
may  be  a  precursor  of  retinitis,  and  to  the  frequent  presence  of  venous 
dilatation;  (2)  to  strain  of  accommodation  in  near  use  and  in  pro- 
longed distant  vision,  giving  rise  to  "  academy  headache,"  as  it  is 
termed  in  London ;    (  3  )  to  muscular  insufficiency. 

Frequency. — To  compute  the  relation  and  frequency  of  ocular  head- 
aches to  headaches  due  to  other  causes  would  be  an  interesting  but  an 
almost  impossible  task.^'*      To  learn  the  frequency  of  headache  as  a 


•«  Ocular  Headaches.  J.  A.  M.  A.,  November  19,  1898. 

•*  C.  A.  Wood  states  that  40  per  cent,  of  all   headaches,  and  80  per  cent,  of 
frontal  headaches,  are  due  to  eye-strain. 
52 


818  THE    EYE    A^D    NERVOUS    SYSTEM 

symptom  of  ocular  disorder  is  only  to  review  the  case-book  of  any 
oculist  in  active  practice.  My  own  records  show  85  per  cent  in  per- 
sons under  45  years  of  age  who  are  fi*ee  from  recognizable  organic 
disease  of  every  description,  ocular  or  general.  Hospital  records 
would  of  course  show  a  less  percentage  since  hospital  cases  include 
more  diseased  conditions.  Zimmerman's  statistics  of  all  classes  of 
headaches  caused  by  ocular  defects  ^^  show  67.8  per  cent,  in  private 
practice  and  74.9  in  hospital  cases.  Zimmerman  explains  the  higher 
ratio  by  the  large  number  among  hospital  cases  who  must  use  their  eyes 
for  close  work  many  hours  in  the  day,  such  as  weavers,  woollen-mill 
workers,  etc.  Ninety-six  of  his  patients  had  monocular  vision  only 
(strabismus  or  enucleation  of  one  eye),  and  of  these  forty  had  headaches. 
Diagnosis. — The  headache  is  usually  induced  by  the  use  of  the 
eyes,  both  in  near  work  and  in  prolonged  effort  to  see  clearly  in  the 
distance,  and  consequently  the  connection  of  the  use  of  the  eyes  with 
the  development  of  the  exacerbation  of  the  pain  is  to  be  determined. 
It  must  not  be  forgotten,  however,  that  some  individuals,  particularly 
with  chronic  headache,  trace  no  connection  between  reading  or  other 
close  work  and  the  headache.  In  such  cases  it  is  practically  impossible 
to  ascribe  the  cause  to  the  eyes  until  after  the  therapeutic  or  optical 
test  is  applied.  The  former  consists  of  the  instillation  of  a  cycloplegic 
for  a  few  days  or  the  abolition  of  the  accommodation.  The  latter,  of 
the  continuous  wearing  of  the  correction  of  any  error  of  refraction  that 
may  have  been  found  under  paralysis  of  accommodation.  Yet  it  must 
be  remembered  that  the  presence  of  muscular  anomalies  of  sufficient 
degree  to  become  a  factor  in  the  causation  of  headache  vitiates  these 
tests,  for  it  is  not  uncommon  to  find  that  headache  persists  or  is  in- 
creased because  of  the  participation  of  the  extraocular  muscles  in  the 
visual  act.  This  is  shown  in  cases  of  functional  internal  hyperopic 
squint.  For  some  days  after  the  initiation  of  the  use  of  a  cycloplegic 
the  degree  of  squint  may  be  higher  than  before  its  use.  The  innerva- 
tion of  the  muscles  of  convergence  is  not  relaxed  because  of  the  tem- 
porary failure  of  the  ciliary  muscle  to  respond  to  its  innervation.  The 
diagnosis  is  simplified  by  the  discovery  of  ocular  strain  induced  by 
hyperopia,  myopia,  or  astigmatism,  as  shown  by  lowered  acuity  of 
vision,  by  the  ophthalmoscope,  ophthalmometer,  retinoscope,  and  test 
lenses,  or  by  imbalance  of  the  ocular  muscles,  as  revealed  by  the  Maddox 
rod,  cobalt  glass  and  diplopia  test,  and  the  proportionately  increased 
tension  of  the  muscles  of  adduction  and  abduction  or  sursumduction. 

"  New  York  Medical  Journal  and  Philadelphia  Medical  Journal,  November  21, 
1903. 


NEUEOSES  OCCASIONED  BY  EYE-STRAIN         819 

In  every  case  knowledge  of  the  functional  activity  of  other  organs  of 
the  body  contributes  materially  to  the  accuracy  of  the  diagnosis.  Or- 
ganic disease  of  the  nervous  system,  abdominal  organs,  digestive  tract, 
syphilis,  gout,  rheumatism,  and  other  general  affections  should  be 
excluded. 

Simulating  ocular  headaches  more  closely  than  the  other  varieties 
are  those  caused  by  nasal  disease.  Here  the  pain  is  frontal,  more  often 
dull  than  sharp.  Disease  of  the  nose  is,  in  many  cases,  excluded  by 
examination  of  its  cavities.  But  Ewing  ^^  describes  a  form  of  head- 
ache of  nasal  origin,  characterized  by  neuralgia  of  the  nasal  and  eth- 
moid nerves  and  tenderness  on  pressure  on  the  inner  upper  angle  of 
the  orbit  of  one  side,  rarely  both,  in  which  tlie  inflammation  is  not 
confined  to  the  frontal  sinus.  Rhinoscopic  examination  revealed  in  his 
case  no  signs  of  the  trouble.  The  headache  is  dull,  persistent,  located 
in  the  frontal  region  and  generally  worse  in  the  morning.  The  cause 
of  the  headache  is  referred  to  the  eyes,  because  the  pain  is  greatly 
aggravated  by  attempts  at  reading,  and  attacks  of  conjunctivitis  and 
blepharitis  are  frequent.  The  diagnostic  features  are  tenderness,  lim- 
ited to  one  orbit  or  one  nasal  bone,  and  asthenopia. 

The  near  work  includes  all  the  ordinary  occupations — reading, 
writing,  sewing,  weaving,  reading  music,  and  amusements,  such  as 
card-playing.  Ocular  headache  is  quite  common  among  card-players 
who  have  entered  the  presbyopic  age,  as  the  games  are  usually  played 
by  artificial  light  and  for  several  consecutive  hours.  The  asthenopia 
is  readily  explained  by  the  illumination  of  the  room,  the  glare  of  the 
light  reflected  from  the  cards,  and  the  accommodative  strain  induced 
by  demand  for  accurate  vision  at  distances  varying  from  one-third  of  a 
metre  to  one  and  one-half  metres,  and,  in  some  cases,  by  the  necessity 
for  constant  and  vigorous  mental  action. 

Presbyopia  is  frequently  a  cause  of  headache.  The  crystalline  lens 
has  become  hard  and  inelastic,  its  layers  of  fibres  gliding  on  one  another 
to  an  exceedingly  limited  degree  while  the  innervation  to  the  ciliary 
muscle  remains  practically  unchanged.  The  contraction  of  the  muscle 
in  response  to  the  innervation  is  also  almost  as  strong  as  early  in  life. 

Ocular  strain  in  the  continuous  effort  to  see  clearly  in  the  distance, 
such  as  the  preacher  in  the  pulpit  and  the  actor  on  the  stage,  is  a  fre- 
quent cause  of  headache  and  other  forms  of  asthenopia.  The  peculi- 
arity of  this  headache  is  its  occurrence  some  hours  after  the  strain  is 
over.  "  Panorama"  headache,  or  car-sickness,  is  less  to  be  attributed 
to  idiosyncrasy  of  individuals  than  the  reflex  of  disordered  ocular  func- 

«*Trans_.  Amer.  Ophthal.  Soc,  1901. 


820  thp:  eye  axd  xervous  system 

tion,  brought  about  by  the  effort  to  fix  distant  and  rapidly  passing 
objects.  The  constant  change  of  focus  and  line  of  direction  of  tlie 
visual  axes  and  also  the  rapidly  changing  retinal  image  entail  too  much 
action  of  the  accommodation,  of  the  extraocular  muscles,  and  of  the 
retina  and  afferent  visual  impulses. 

Character. — The  pain  is  sharp  and  neuralgic  or  dull  and  heavy,  vary- 
ing according  to  the  temperament  of  the  individual,  the  degree  of  the 
error  of  refraction,  the  length  of  time  during  which  the  near  use  of 
the  eyes  is  demanded,  age,  and  the  presence  of  muscular  anomaly.  The 
first  exceeds  in  importance,  as  a  cause  of  intensity  of  pain,  all  other 
causes,  and  demonstrates  that  the  cause  of  the  trouble  is  not  only  ocular 
anomaly,  but  the  combination  of  an  ocular  anomaly  with  a  susceptible 
impressionable  disposition.  Low  defects  in  subjects  enfeebled  by  dis- 
ease or  whose  power  of  resistance  is  weakened  by  suffering  or  mental 
distress  may  give  rise  to  intense  and  constant  headache,  while  in  healthy 
and  strong  individuals  equal  defects,  whether  of  refraction  or  muscular 
imbalance,  are  overcome  or  held  in  abeyance  without  any  response  what- 
ever of  the  nervous  system.  In  H.  and  HAs.  of  between  1  and  2  D. 
the  pain  is  apt  to  be  constant  during  the  working  hours,  and  is  aggra- 
vated by  close  work.  In  mixed  astigmatism  of  moderate  degree,  the 
headache  may  be  constant  and  associated  with  nausea  and  vomiting 
and  not  infrequently  with  symptoms  of  some  nervous  disorder.  Xear 
work  is  either  impossible  or  accomplished  only  at  the  expense  of  intense 
suffering,  and,  if  persisted  in,  results  in  chronic  invalidism.  The  so- 
called  neuralgia  and  migraine  attributed  by  medical  men  and  laymen 
to  malaria,  rheumatism,  anamia,  and  other  general  and  vague  diseases, 
is,  in  the  majority  of  cases,  only  an  expression  of  a  disordered  ocular 
function.  For  example:  G.  L.  Walton,  Boston  Medical  and  Surgical 
Journal,  has  made  an  interesting  study  of  migraine  among  individuals 
blind  from  infancy  and  others  who  later  in  life  became  partly  blind. 

He  found  among  41  persons  blind  from  infancy  80  per  cent,  had 
never  had  headache. 

Among  18  cases  becoming  completely  blind  in  life  only  5  were 
free  from  headache. 

Among  100  persons  in  good  health  only  31  per  cent,  had  never  suf- 
fered from  headache.  Seventeen  had  typical  migraine  (about  one  in 
six  individuals),  a  marked  contrast  to  the  absence  of  typical  migraine  in 
the  41  cases  of  congenital  and  complete  blindness.  He  believes  that  the 
explanation  is  to  be  sought  in  the  frequency  of  eye-strain  among  those 
who  have  their  sight,  particularly  among  the  partially  blind,  and  its 
absence  in  the  totally  blind. 


NEUROSES  0CCASI0:N^ED  by  EYE-STRAIX         821 

The  neuralgia  may  be  limited  to  one  side  of  the  face  or  may  be 
bilateral.  The  supraorbital  nerves  are  tender  on  pressure,  or  even 
painful  spots  located  in  the  scalp  simulating  supraorbital  neuritis  may 
be  present,  and  yet  the  cause  be  an  ocular  anomaly. 

The  ocular  headache  assumes  many  phases.  It  cannot  be  certainly 
diagnosticated  without  an  ocular  examination  and  proper  correction, 
but  it  is  safe  to  predict  in  any  given  case  that  the  combination  of  H. 
and  HAs.,  or  esophoria  with  an  average  nervous  susceptibility,  will  be 
the  cause  of  headache,  which  will  be  temporarily  relieved  by  rest  and 
permanently  relieved  by  well  adjusted  correction,  worn  constantly. 

WTiile  headache  is  the  most  common  form  of  asthenopia,  other  re- 
flexes not  so  directly  referable  to  the  use  of  the  eyes  are  worthy  of 
mention, — for  instance,  migraine,  neurasthenia,  insomnia,  restlessness, 
nervousness,  enuresis,  urticaria,  depression  of  spirits  or  mental  apathy. 
Examples  of  each  of  these  affections  and  others  are  found  in  medical 
literature.  Their  diagnosis  and  treatment  is  identical  with  that  of 
ocular  headache,  indigestion,  and  other  reflexes  of  eye-strain. 

Treatment. — The  indications  are  twofold :  first,  the  correction  of 
the  error  of  refraction  by  glasses  and  the  muscular  anomaly  by  appro- 
priate remedies ;  second,  hygienic  and  medical  measures  that  will 
reduce  the  susceptibility  of  the  nervous  system,  regulate  the  habits  and 
modes  of  life,  both  as  they  pertain  to  the  general  health  and  more 
particularly  to  lihe  ocular  function.  Ametropia  may  be  corrected  in 
individuals  under  45  years  of  age  who  have  hyperopic  astigmatism 
only  by  learning  the  static  refraction  under  paralysis  of  accommo- 
dation. The  cycloplegic  used  is  immaterial,  provided  it  abolishes  tem- 
porarily the  contractility  of  the  ciliary  muscle.  In  myopes  of  more 
than  4  D.  or  5  D.  of  myopia,  cycloplegia  is  seldom  necessary  since  such 
persons  rarely  suffer  from  accommodative  headache  and  the  ciliary 
muscle  cannot  conceal  the  optical  defect.  After  the  age  of  45,  cyclo- 
plegics  are  advisable  only  exceptionally,  since  the  true  refraction  of  the 
eye  is  manifest. 

The  eyes  are  not  exclusive  factors  in  the  production  of  headache, 
but  no  diagnosis  is  complete  which  ignores  them. 

SPASMODIC   MOVEMENTS   OF  THE   HEAD 
OTHER  THAN   CHOREIFORM. 

This  affection  may  be  due  to  organic  disease  of  the  cerebrum  or 
of  the  meninges,  to  unnatural  positions  of  the  head  in  consequence  of 
paralytic  or  functional  imbalance  of  the  ocular  muscles,  to  traumatism 
of  the  neck  or  trunk,  posture  incident  to  some  forms  of  chronic  invalid- 


822  THE    EYE    AND    NERVOUS    SYSTEM 

ism,  rheumatism,  and,  possibly,  to  other  deformities  by  which  the 
physical  equilibrium  is  disturbed.  In  the  adult,  head  shaking  is  a 
symptom  of  paralysis  agitans,  disseminated  sclerosis,  general  paralysis 
of  the  insane,  aneurism  and  abscess  of  the  brain.  Head  tilting  is  asso- 
ciated with  functional  and  organic  ocular  muscle  defects,  oblique  non- 
compensating  bilateral  astigmatism,  traumatic  or  diseased  contraction 
and  paralysis  of  the  neck  muscles  on  one  side,  and  to  disease  of  the 
cerebrospinal  system.  In  infants,  involuntary  rapid  oscillation  or  other 
movements  of  the  head,  variously  designated  as  head  shaking,  eclampsia 
nutans,  head  banging,  salaam,  convulsions  of  infancy,  head  bowing, 
and  nodding  convulsions,  is  accredited  to  arrested  development  of  the 
nervous  system,  and  is  found  in  association  with  rickets,  epilepsy,  im- 
paired intellect,  and  physical  deformities.  "  Sometimes  head  shaking 
in  infants  is  a  form  of  half-involuntary  movements,  twitchings,  antics, 
and  grimaces  to  which  children  are  subject.  In  this  case  there  is  no 
fear  of  epilepsy,  and  recovery  is  the  rule."  ^^ 

The  position  of  the  head  assumed  in  compensation  of  heterophoria, 
heterotropia,  and  errors  of  refraction,  is  independent  of  central  lesions 
and  is  purely  voluntary.  It  is  not  in  any  sense  a  symptom  of  disease 
or  a  disease  itself.  It  resembles  the  habit  spasm  or  contractions  of  the 
facial  muscles  common  in  individuals  in  whom  the  visual  act  is  accom- 
plished only  by  undue  muscular  strain.  The  direction  of  the  tilting 
of  tlie  head  characterizes  to  a  certain  extent  the  variety  "of  defect.  Thus, 
in  paralysis  of  the  externus  of  the  right  eye,  the  eye  is  deviated  to  the 
left  and  the  face  is  rotated  to  the  right  in  order  to  bring  the  visual 
line  of  the  right  eye  parallel  to  that  of  the  left.  In  paralysis  of  the 
motor  oculi  of  the  right  eye  the  right  cornea  is  deviated  towards  the 
temple  and  the  face  is  turned  to  the  left ;  in  paralysis  of  the  elevators 
of  the  cornea  the  chin  is  raised  in  order  to  bring  the  depressed  axis 
of  the  paralyzed  eye  in  line  with  that  of  the  sound  eye ;  in  paralysis 
of  the  depressors  the  head  is  tilted  forward  with  the  chin  receding. 
Bard  ^^  states  that  ocular  deviation  and  rotation  of  the  head  after 
hemiplegia  is  due  to  active  movements  of  the  muscular  groups  of  the 
unaffected  side  automatically  governed  by  the  unilateral  action  of  cen- 
tral perceptions,  or  simply  from  the  reflex  power  of  the  sensorimotor 
centres.  Sight  predominates,  but  other  senses  take  part,  particularly 
hearing  and  those  of  locality  and  equilibrium.  But  slightly  influenced 
by  the  seat  of  the  lesion,  it  is  affected  by  the  gravity,  extent,  sudden- 
ness and  intensity  of  the  shock  and  by  the  degree  of  coma.    Both  ocular 

"  Samuel  Gee,  St.  Barthol.  Hosp.  Rep.,  1886. 
•*Semaine  M§dicale,  January  13,  1904. 


Is^EUROSES  OCCASIONED  BY  EYE-STRAIN         823 

deviation  and  head  rotation  disappear  either  on  recovery  of  the  affected 
hemisphere  or  extension  of  the  lesion  to  the  other  side  of  the  brain. 
In  heterophoria — latent  squint — tilting  of  the  head  or  face  is  less  notice- 
able than  in  heterotropia  (manifest  squint),  since  in  the  former  there 
is  no  actual  deviation  of  the  visual  axes.  The  tension  of  the  weaker 
set  of  muscles  is  partly  substituted  by  contraction  of  the  occipito- 
frontalis,  corrugator  supercilii,  or  the  orbicularis  palpebrarum.  The 
undue  stimulation  of  the  facial  muscles  may  be  extended  to  include 
adjacent  muscles  of  the  neck  or  back,  by  the  contraction  of  which  the 
erect  posture  of  the  head  or  trunk  is  modified.  Lewis  ^^  reports  a  case 
of  pseudotorticollis  in  a  girl  eight  years  old.  She  complained  of  frequent 
headache  during  the  winter  school  session.  Previously  a  good  scholar, 
she  became  dull,  inattentive,  irritable,  and  thin.  During  the  summer 
vacation  she  gained  in  weight  and  the  headaches  were  less,  but  improve- 
ment was  cut  short  by  resumption  of  school  studies.  The  symptoms 
returned,  aggravated  by  marginal  blepharitis  and  lachrymation.  She 
held  her  head  in  a  depressed  position  downwards  to  the  right  side,  so 
that  the  eyes  when  fixing  an  object  directly  in  front  were  directed  up- 
wards and  to  the  left,  the  upper  lids  covering  the  irides  almost  down 
to  the  pupillary  margins.  Slow  rhythmic  swaying  of  the  head  when 
looking  at  the  test  letters  was  a  constant  symptom.  There  was  no  stiff- 
ness of  the  cervical  muscles.  The  oblique  position  of  the  head  was 
maintained  in  the  endeavor  to  overcome  the  imperfect  vision  due  to 
the  need  of  mixed  cylinders  (R.  — 75  =  -{-2°  ax.  100)  right  hyper- 
phoria of  3^°  and  esophoria  2°.  In  testing  the  near  vision  the  same 
rhythmic  swaying  was  noticed.  Gould  '^°  relates  an  interesting  and 
instructive  case  of  torticollis  and  spinal  curvature  due  to  eye-strain 
(from  astigmatism).  He  says  the  case  should  be  interesting  to  the 
general  physician,  orthopedic  surgeon,  and  oculist,  because  the  patient 
was  of  tuberculous  parentage,  and  it  was  feared  that  she  would  develop 
tuberculosis.  The  symptoms  simulating  tuberculosis  were  soon  found 
to  be  caused  by  the  false  position  of  the  head,  that  in  turn  by  wry- 
neck which  had  caused  spinal  curvature.  The  patient,  a  girl  of  nine- 
teen, complained  of  severe  and  protracted  headache,  sore  eyes,  and 
pain  in  the  eyeballs.  The  erect  position  was  resumed  and  all  the  symp- 
toms both  of  eye-strain  and  tuberculosis  disappeared  after  correction 
of  compound  hyperopic  astigmatism.  Without  her  glasses  the  head 
takes  its  former  abnormal  position,  the  right  shoulder  drops,  the  back 
becomes  more  humped,  etc.     The  regained  health  is  primarily  due  to 

~  Oph.  Rec,  1903,  No.  12. 
^"American  Medicine,  March  25,  1904. 


824  THE    EYE    AND    NERVOUS    SYSTEM 

the  glasses  and  secondarily  to  the  proper  position  of  the  body,  increased 
lung  capacity,  and  better  oxygenation  of  the  blood.  A  second  case  of 
torticollis  was  a  woman  of  25,  with  high  degree  of  astigmatism  in 
both  eyes,  axes  170°,  a  defect  which  would  not  allow  binocular  vision 
with  the  head  in  its  normal  position.  Gould  alludes  briefly  to  other 
cases,  whose  histories  are  confirmative  of  the  causation  of  torticollis 
and  spinal  curvature  by  ocular  defects  and  the  cure  of  tliose  affections 
by  relief  of  the  e^^e-strain. 

Wadsworth  '^  reports  a  case  of  spastic  torticollis  in  a  boy  of  four- 
teen which  had  persisted  since  infancy.  The  boy  was  healthy  and 
had  good  muscular  development.  He  sat  or  stood  with  his  head  tipped 
to  the  right  and  inclined  considerably  forward,  his  right  shoulder  lower 
than  the  left  and  the  spine  laterally  curved,  with  convexity  to  the  left. 
The  ocular  excursions  were  good  in  all  directions.  In  ordinary  posi- 
tions, with  fixations  for  near  or  distance,  the  left  eye  stood  out  about 
one  degree  higher  than  the  right.  Only  when  the  eyes  were  turned 
strongly  down  and  to  the  left,  while  at  the  same  time  tlie  head  was 
inclined  forward  and  to  the  right,  did  he  apparently  get  binocular  fixa- 
tion. The  superior  rectus  w'as  divided,  with  the  result  that  the  body 
has  been  much  straighter  ever  since,  and  is  still  improving. 

Walton  "^  reports  the  case  of  a  boy  12  years  of  age  who  had  a 
general  chorea,  head  jerking  being  the  most  marked  symptom.  The 
child  had  an  esophoria  of  6°.  His  internal  recti  were  tenotomized,  and, 
when  seen  two  weeks  after  the  operation,  the  twitchings  had  almost 
entirely  ceased. 

Recurrence  of  head  shaking,  head  tilting,  and  torticollis  may  be 
expected  upon  the  return  of  the  muscular  insufficiencies.  Should  they 
relapse  following  a  change  in  the  refraction  of  the  eyes,  especially  in 
the  axis  of  the  astigmatism,  they  may  be  again  relieved  by  restoration 
of  normal  ocular  conditions. 

Posey  '^  classifies  the  head  movements  into  (1)  physiologic,  or  "  the 
many  and  varied  movements  which  the  head  makes  through  the  medium 
of  the  neck  muscles;"  (2)  abnormal  independent,  but  simultaneous, 
or  the  accidentally  simultaneous,  occurrence  of  nystagmus  and  head 
tremor;  (3)  compensatory,  or  a  vicarious  position  of  the  head  assumed 
to  compensate  for  some  imperfect  action  of  one  or  more  of  the  extra- 
ocular muscles,  and  (4)  related,  but  not  compensatory,  or  those  anoma- 

"'  Trans.  Amer.  Ophthl.  Soe.,  vol.  v. 
"Boston  Medical  and  Surgical  Journal,  1892. 

"  Associated  Movements  of  the  Head  and  Eyes,  Journal  of  the  American  Medical 
Association,  November  29,  1902, 


NEUROSES  OCCASTON^ED  BY  EYE-STRAIN         825 

lous  movements  of  both  head  and  eyes,  the  result  of  central  lesion. 
Under  the  third  heading  he  refers  to  cases  reported  by  Wadsworth, 
Risley,  and  Wilder,  and  adds  one  case  of  his  own.  Under  the  fourth 
class  he  describes  a  new  case. 

Head  Bowing  of  Infants. — This  affection  is  essentially  different 
in  its  pathology  and  etiologj'  from  the  head  movings  or  tiltings  of  adults, 
symptomatic  or  ocular  disturbances.  In  infants  the  affection  is  attribu- 
table to  organic  changes  in  the  brain  or  its  membranes,  the  nature  of 
which  is  unknoMTi.  Another  and  entirely  distinct  form  of  eye,  head, 
and  hand  movements,  which  should  not  be  confounded  with  the  true 
eclampsia  nutans,  is  that  sometimes  seen  in  children  whose  vision  is 
reduced  to  the  perception  of  light  from  ophthalmia  neonatorum,  or 
congenital  cataract.  In  the  latter  affection,  nystagmus  is  invariably 
present.  The  head  is  moved  slowly,  horizontally,  and  vertically  and 
one  or  both  hands  are  held  between  the  eyes  and  the  light  and  rapidly 
moved  within  a  narrow  area.  In  the  former  the  infants  are  rhachitic, 
hydrocephalic,  strumous,  or  tubercular.  William  Xestham,^^  who  seems 
to  have  studied  this  affection  with  great  thoroughness,  says,  "  There 
is  evidently  in  this  malady  a  family  alliance  with  epilepsy,  and  hence, 
as  has  been  demonstrated  by  the  pregoing  cases,  it  often  passes  into 
epilepsy  or  other  form  of  infantile  convulsions.  Tetanoid  symptoms 
also  sometimes  occur  during  its  progress."  The  disease  has  been  at- 
tributed to  dentition,  irritation  of  spinal  nerves  from  the  presence  of 
worms  or  other  irritating  matter  in  the  body  and  also  to  atmospheric 
changes.  His  conception  of  the  morbid  action  is  that  the  essential  char- 
acter of  this  malady  is  inflammatory  action  in  the  weak  or  strumous, 
which  in  all  probability  commences  in  the  membranes  investing  the 
medulla  oblongata.  It  is  early  extended  to  the  membranes  covering 
the  base  of  the  brain,  and  is  succeeded  by  disease  of  the  vessels  supply- 
ing and  nourishing  the  organ  itself.  This  condition  is  then  followed 
by  exudations  of  lymph  or  serum,  the  locality  of  these  effusions  being 
determined  by  the  constitution  and  local  circumstances,  and,  as  a  con- 
sequence of  these  exudations,  paralysis  is  produced  from  pressure.  The 
regular  nutrition  of  the  brain  is  interrupted,  and,  in  the  more  aggra- 
vated cases,  the  organ  becomes  so  deteriorated  as  to  lose  all  power  of 
carrying  on  the  intellectual  functions. 

The  rapidity  and  frequency  of  the  head  movements  differ  in  differ- 
ent children,  and  in  the  same  child  at  different  times.  The  movements 
may  recur  at  intervals  of  months,  each  spasm  continuing  a  few  min- 


'*  London  Medical  Record,  May  14,  vol.  xi.   1881. 


826  THE    EYE    AND    NERVOUS    SYSTEM 

utes,  or  they  may  be  almost  incessant  for  several  hours.  The  head 
is  slowly  allowed  to  fall  backward,  possibly  because  the  child  is  too 
feeble  to  hold  it  erect,  and,  by  a  jerk,  restored  to  its  upright  position, 
or  it  is  thrown  violently  and  rapidly  from  side  to  side,  "  banged"  for- 
wards and  backwards  into  an  unresisting  substance,  as  a  pillow.  The 
spasms  may  be  associated  with  fainting  or  unconsciousness,  or  may 
occur  during  sleep  or  immediately  upon  awakening.  Dr.  Stephen  Mac- 
kenzie "^^  reported  two  cases,  each  presenting  a  series  of  more  or  less 
rhythmical  movements  of  the  head,  partly  consisting  of  a  nodding  of 
the  head  forwards  and  partly  of  a  rotary  movement  In  each  there 
was  nystagmus,  which  in  one  case  was  almost  entirely  confined  to  one 
eye.  The  nystagmus  was  increased,  or  evoked  if  absent,  by  restraining 
the  movements  of  the  head.     The  nodding  ceased  during  sleep. 

Thomson  "^^  says  that  more  than  tliree-fourths  of  the  published  cases 
of  spasmas  nutans,  or  head  jerking,  in  infants  begin  between  four  and 
twelve  months  of  age.  This  age  corresponds  to  the  period  during  which 
the  child  is  slowly  learning  to  co-ordinate  the  movements  of  the  eyes 
with  those  of  the  head.  Up  to  the  fourth  month  of  age  the  infant  has 
great  difficulty  in  moving  the  two  sets  of  ocular  muscles  together,  and 
even  towards  the  end  of  the  first  year  it  has  very  little  skill  in  following 
the  object  moving  with  his  head  and  eyes.  Cases  have  been  described 
beginning  as  early  as  one  month  "^"^  and  six  weeks  old,'*  but  no  details 
have  been  published  of  any  commencing  later  than  the  twentieth  month. 
In  the  case  of  second  attacks,  however,  the  symptoms  may  still  be 
present  in  the  third  year. 

The  prognosis  is  not  necessarily  unfavorable.  When  the  affection 
is  due  to  the  reflex  irritation  of  dentition,  intestinal  worms,  phimosis, 
and  other  curable  causes,  complete  recovery  ensues  upon  removal  of 
the  irritation,  provided  the  infant's  health  has  not  been  too  seriously 
undermined. 

Abt '®  saw  a  case  of  spasmus  nutans  with  rotary  nystagmus  follow- 
ing injuryto  the  head.  Examination  of  the  eyes  showed  that  the  pupils 
were  regular  and  reacted  normally.  The  media  and  fundi  were  healthy 
and  there  was  no  strabismus  present.  The  head-nodding  was  almost 
continuous.  Bromide  of  sodium,  either  alone  or  in  combination  with 
belladonna,  was  administered,  and  in  six  weeks  the  patient  recovered. 

"Lancet,  vol.  i.   1886. 

"  Scottish  Medical  and  Surgical  Journal,  1900. 

"  Schonberg,  Norsk,  Magaz.  for  Laegevidenskaben,  1891. 

"Gee,  St.  Barthol.  Hosp.  Reports  for  1886. 

"Journal  of  the  American  Medical  Association,  February,  1900. 


LEUKOSES  OCCASIONED  BY  EYE-STRAIN         827 

The  infants  who  survive  the  disease  of  the  cerebrospinal  system  of 
which  the  head  nodding  is  a  symptom,  are  usually  permanently  en- 
feebled, either  physically  or  mentally.  The  ophthalmoscopic  findings 
are,  as  a  rule,  negative,  but  should  optic  neuritis  or  optic  nerve  atrophy 
be  a  complication  the  prognosis  is  unfavorably  affected. 

Treatment. — The  principle  upon  which  the  treatment  is  based  varies 
according  to  the  most  probable  cause  of  the  affection.  Should  spinal 
irritation  be  suspected,  calomel  to  keep  the  secretions  in  order  and 
tonics  to  support  the  strength  are  indicated.  If  dental  irritation  be 
present,  thorough  and  repeated  lancing  of  the  gums  is  called  for.  The 
avoidance  of  everything  that  tends  to  increase  cerebral  action  should 
be  a  part  of  the  treatment.  The  bromides  are  useful.  Nourishing 
diet  at  regular  and  frequent  intervals,  abundance  of  fresh  air  and  sun- 
light, well  ventilated  sleeping-rooms,  suitable  clothing,  and  other 
hygienic  measures,  constitute  an  essential  part  of  the  treatment. 


CHAPTER     XIX. 

EXOPHTHALMIC  GOITRE. 
By  WILLIAM  CAMPBELL  POSEY,  M.D. 

Exophthalmic  goitre,  Graves's  disease,  or,  as  it  is  designated  in 
Europe,  Basedow's  disease,  is  an  affection  characterized  in  most  cases 
by  a  group  of  symptoms  which  consist  of  increased  frequency  of  the 
pulse,  enlargement  of  the  thyroid  gland,  the  presence  of  certain  charac- 
teristic ocular  changes,  a  fine  tremor,  and  nervous  irritability.  The 
disease  is  essentially  chronic  in  its  course  and  is  of  unknown  origin, 
though  probably  dependent  upon  some  disturbance  of  the  central  nervous 
system  induced  primarily  by  changes  in  the  thyroid  gland. 

Although  others  had  practically  described  the  disease,  in  reports  of 
cases  which  were  undoubtedly  true  instances  of  exophthalmic  goitre,  the 
honor  of  recognition  of  the  symptoms  as  belonging  to  a  distinct  and 
separate  morbid  process  should  be  accorded  to  Graves  (1835),  though 
it  was  not  until  five  years  later  (1840)  that  Basedow,  ignorant  of 
Graves's  discovery,  fully  described  the  disease  with  its  cardinal  symp- 
toms. 

SYMPTOMATOLOGY. 

All  the  five  primary  symptoms  which  have  just  been  mentioned 
are  not  always  present  at  the  same  time  in  every  case,  and  one  or  more 
may  be  absent  during  the  entire  course  of  the  disease ;  the  tachycardia 
and  the  nervous  irritability  appear,  however,  to  be  present  in  all  cases. 
In  addition  to  these  cardinal  symptoms,  there  are  other  secondary  symp- 
toms which  are  quite  frequently  present;  these  include  changes  in  the 
alimentary  canal,  in  the  respiratory  and  genito-urinary  organs,  and  in 
the  skin. 

Vascular  System. — Increased  Frequency  of  the  Pulse;  Tachycardia. — 
The  most  constant  and  usually  the  first  symptom  of  exophthalmic  goitre 
is  a  marked  increase  in  the  force  and  frequency  of  the  contraction  of 
the  heart;  the  pulse  may  beat  as  rapidly  as  120  to  160  times  in  the 
minute,  and  even  200  beats  have  l)ecn  recorded ;  it  rarely  sinks  to 
less  than  100.  The  action  of  the  heart  is  often  irregular  and  may  l)e 
at  times  intermittent;  it  is  usually  perceptible  to  the  patient,  espe- 
cially if  it  be  even  slightly  increased  by  exertion  or  excitement.  The 
828 


EXOPHTHALMIC  GOITRE  829 

cardiac  impulse  is  strong  and  extends  over  a  larger  area  than  is  normal. 
In  the  early  stages  the  sounds  of  the  heart  are  usually  unaffected,  but 
later,  as  a  consequence  of  the  prolonged  increased  activity  in  the  cardiac 
action,  the  sounds  become  louder  than  normal,  and  Gowers  ^  is  authority 
for  the  statement  that  they  may  be  so  intense  at  times  as  to  be  heard 
as  far  as  four  feet  from  the  patient. 

Murmurs  present  in  the  early  stages  of  the  disease  are  usually  of 
anaemic  origin,  but  later  actual  murmurs  from  faulty  valvular  action, 
as  a  consequence  of  hypertrophy  and  dilatation  of  the  heart,  may  arise. 
Pulsation  in  the  arteries  is  common  and  can  be  readily  seen  and  felt  in 
the  large  arteries  at  the  root  of  the  neck  and  even  in  the  peripheral 
vessels ;  in  thin  subjects  pulsation  can  be  demonstrated  in  the  abdominal 
aorta. 

Nervous  System. — Tremor  and  Nervous  Irritability. — Tremor  is  prob- 
ably present  in  all  cases  at  some  time  or  other ;  though  usually  an 
early  s^onptom,  it  may  not  manifest  itself  until  after  other  symp- 
toms have  appeared.  It  is  most  marked  in  the  extremities,  par- 
ticularly the  upper,  though  it  may,  however,  extend  over  the  entire 
body.  The  character  of  the  tremor  varies,  but  is  usually  fine  and 
regular.  In  cases  where  the  exophthalmus  and  goitre  have  been  limited 
to  one  side  of  the  body,  the  tremor  may  be  unilateral  also.  It  is  best 
elicited  by  requesting  the  patient  to  stretch  out  the  hands  with  the  palms 
directed  downward,  when,  if  too  slight  to  be  seen,  by  laying  the  palm  of 
the  hand  lightly  upon  that  of  the  patient,  the  observer  will  note  that 
the  trembling  is  not  in  the  fingers  alone,  but  that  the  entire  hand  moves, 
this  being  occasioned  by  a  spasmodic  action  of  the  flexor  and  extensor 
muscles  of  the  wrist.  The  tremor  is  increased  by  excitement  or  by 
attempting  to  perform  actions  with  extreme  care,  and  resembles  the 
tremor  of  fatigue  and  weakness  in  being  a  little  more  frequent  in  time 
than  that  of  paralysis  agitans  or  of  disseminated  sclerosis  (page  908). 

In  nearly  all  cases  of  Graves's  disease  there  is  a  slight  mental  change 
which  usually  manifests  itself  in  an  unwonted  irritability  and  rest- 
lessness. There  may  be  times  when  the  patient  regains  his  usual  spirits, 
but  he  soon  lapses  again  into  a  peevish  and  depressed  state.  In  other 
cases  the  mental  derangement  takes  the  form  of  a  nervous  expectancy, 
and  the  patient  is  in  a  flutter  of  excitement  about  trifles.  Nervous 
symptoms,  as  a  rule,  develop  in  individuals  who  are  predisposed  by  a 
neurotic  ancestry,  but  they  may  appear  in  subjects  who  were  quiet  and 
unexcitable  or  even  phlegmatic  before  the  disease  manifests  itself. 

In  addition  to  this  general  nervousness,  there  are  not  infrequently 
other  nervous  symptoms  which  are  of  a  functional  rather  than  of  an 


830  THE    EYE    AXD    KERVOUS    SYSTEM 

organic  nature.  Hysteria  is  often  present  and  may  occur  under  various 
forms,  though  some  variety  of  anaesthesia  or  paralysis  is  the  most 
common.  Many  of  the  convulsions  which  occur  in  the  course  of  exoph- 
thalmic goitre  are  of  an  hysterical  origin.  Headache  and  migraine 
in  its  typical  form,  neuralgia,  dizziness,  ringing  in  the  ears,  and  other 
similar  neuroses  have  also  heen  met  with. 

Actual  insanity  may  supervene,  taking  the  form  of  melancholia  and 
mania  or,  more  rarely,  that  of  recurrent  mania  and  general  paralysis. 
The  prognosis  is  always  rendered  grave  by  this  complication,  as  death 
ensues  in  some  cases. 

Paralyses  of  cranial  nerves  are  not  very  unusual,  probably  from 
nuclear  involvement.  Symptoms  of  tabes  have  been  observed  in  a 
number  of  cases ;  paraplegia  may  manifest  itself,  either  with  flaccidity 
or  rigidity  of  the  legs,  and  Charcot  ^  and  Maude  ^  have  observed  an 
inability  to  stand  or  walk,  while  the  legs  can  be  freely  moved  when 
lying  down  (astasia-abasia),  as  an  early  symptom. 


Fig.  1.— a  case  of  exophthalmic  goitre.    (From  Wilbrand  and  Saenger.) 

Rarer  complications  include  chorea,  tetany,  chronic  muscular  atro- 
phy, paralysis  agitans,  Thomsen's  disease,  rheumatoid  arthritis,  and 
osteomalacia. 

The  Enlargement  of  the  Thyroid  Gland. — This  usually  occurs 
simultaneously  with  the  exophthalmus.  It  may,  however,  exist  for 
some  years  before  any  of  the  other  symptoms  of  exophthalmic  goitre 


EXOPHTHALMIC  GOITRE  831 

appear.  Of  eighty-seven  cases  seen  by  Murray,  in  which  either  one 
or  two  symptoms  appeared  simultaneously  before  the  others,  an  enlarge- 
ment of  the  thyroid  gland  was  the  first  symptom  of  the  disease  in 
forty-three.  Though  not  so  constant  a  symptom  as  the  tremor,  it  is 
present  in  most  of  the  cases,  Murray  finding  enlargement  of  the  gland 
absent  during  some  period  of  the  disease  in  but  three  out  of  one  hundred 
and  twenty  cases  recorded  by  him  (21/4  per  cent.).  Griffith^  failed 
to  find  an  enlargement  at  the  time  of  examination  in  thirteen  out  of 
thirty  cases.  The  gland,  as  a  rule,  is  but  moderately  enlarged,  and 
rarely  attains  the  size  of  parenchymatous  goitres.  The  enlargement 
is  usually  symmetrical  upon  both  sides,  though  it  frequently  happens, 
especially  when  the  exophthalmus  is  unilateral,  that  one  lobe  is  more 
enlarged  than  the  other;  when  this  is  the  case  the  right  lobe  is  usually 
the  larger. 

The  vessels  of  the  growth  are  usually  much  dilated,  and  pulsation 
of  the  entire  gland  is  generally  palpable  and  visible  as  well,  especially 
when  the  heart's  action  has  been  increased  by  excitement.  A  thrill  can 
generally  be  felt  on  the  gland,  and  the  ear  will  detect  a  loud  systolic 
murmur  or  often  a  bruit  de  diahle.  Guttman  considers  a  double  mur- 
mur to  be  pathognomonic. 

THE   OCULAR   SYMPTOMS. 

These  constitute  a  very  striking  feature  of  the  disease,  and  though 
none  of  them,  when  existing  alone,  can  be  considered  to  be  pathog- 
nomonic of  exophthalmic  goitre,  the  association  of  one  or  more  of  the 
most  significant  with  but  one  of  the  other  cardinal  symptoms  of  the 
disease  is  sufficient  to  establish  the  diagnosis  of  exophthalmic  goitre. 
They  may  best  be  studied  in  the  following  order : 

The  Exophthalmus. — ^Actual  protrusion  is  not  always  easy  to  deter- 
mine, especially  as  the  development  of  the  exophthalmus  is  usually 
gradual,  and  the  clinician  will  frequently  be  compelled  to  rely  upon 
the  observation  of  the  patient  or  his  family  and  friends  or  to  have 
recourse  to  photographs  taken  earlier  in  life  before  it  can  be  definitely 
asserted  that  an  actual  protrusion  of  the  globe  has  occurred.  Care 
must  also  be  taken  to  differentiate  unusual  prominence  of  the  eyeball 
due  to  increased  length  in  the  axis  of  the  globe  (myopia  or  anterior 
staphyloma)  or  to  shallow  orbits,  from  true  exophthalmua  The  degree 
of  the  exophthalmus  varies  greatly  in  different  cases,  in  some  being  so 
slight  that  it  occasions  a  mere  staring  expression,  while  in  others  it  is  so 
pronounced  that  the  lids  cannot  be  completely  closed  over  the  globe ; 
in  extreme  cases  the  eyeball  may  even  be  dislocated  in  advance  of  the 
lids  (Pain.).-^ 


832 


THE    EYE    AND    NERVOUS    SYSTEM 


The  degree  of  the  exophthahiius  may  change  from  day  to  day,  the 
protrusion  being  made  greater  by  whatever  increases  the  heart's  action. 
Its  progress  also  is  not  always  uniform,  for  there  are  frequent  periods 
when  the  globe  appears  to  remain  stationary.  In  some  cases  the  exoph- 
thalmus  has  appeared  only  under  special  circumstances ;  thus  De  Mussy 
found  it  in  a  case  only  during  choreic  attacks  and  Savage  in  another 
case  at  the  time  of  menstruation  alone,  when  there  were  symptoms  of 
mental  disturbance. 

The  eyeballs  can  be  pushed  easily  back  into  the  orbits,  though  they 
come  rapidly  forward  as  soon  as  the  pressure  is  relieved.  The  protru- 
sion is  generally  directly  forwards,  and  both  eyes  are,  as  a  rule,  simi- 
larly affected,  though  the  exophthalmus  may  be  limited  to  one  eye. 

A  case  of  this  nature  was  presented  by  Dr.  Swindells  and  the  writer 
before  the  College  of  Physicians  of  Philadelphia.  In  this  patient,  a 
female,  aged  22,  the  left  eye  became  suddenly  proptosed  two  years  pre- 
viously following  a  fright.     She  had  been  nervous  for  some  years  and 


FiQ.  2.— Unilateral  exophthalmus. 


had  suffered  from  irregular  menstruation.  Her  mother  was  neurotic 
and  had  goitre,  but  no  exophthalmus.  A  maternal  grandmother  had 
died  of  cancer  of  the  throat.  The  exophthalmus  in  the  left  eye  was  of 
moderate  degree  and  the  right  eye  was  normal.  All  lid  signs  were 
absent  except  infrequent  winking.  The  gland  was  bilaterally  enlarged, 
though  the  right  side  was  the  larger.  Marked  improvement  in  the  gen- 
eral and  local  condition  followed  a  strict  rest  cure,  galvanism,  and  syrup 
of  hydriodic  acid. 

A  comprehensive  monograph  upon  the  subject  of  unilateral  exoph- 
thalmus was  contributed  by  Fridenburg  in  1895.  This  author  reported 
one  case  in  his  own  practice  and  one  from  Jacoby's,  and  gave  ®  in  addi- 
tion the  notes  of  ten  additional  cases  which  he  had  collected  from  the 
literature.     Since  the  publication  of  this  article  additional  cases  have 


EXOPHTHALMIC  GOITKE 


833 


been  reported  by  Sichels,^  Volkels,*  Prael,^  Mauthner,^*^  Mooren,^^ 
Rosner/^  Emmert/^  De  Giovanni/*  Barella,^"  Hitscham,^®  Mendel,^ '^ 
Trousseau/^  and  Bistis.^^ 

A  number  of  observers  have  remarked  that  in  many  of  these  cases 
the  enlargement  of  the  thyroid  was  limited  to  one  side  of  the  gland,  and 
that  upon  the  side  on  which  the  eye  was  proptosed.  The  majority  of 
the  cases,  however,  show  no  such  association,  and  no  special  significance 
can  be  attached  to  the  occurrence.  Sattler  ^o  explains  unilateral  exoph- 
thalmus  by  a  disturbance  of  the  vasomotor  centres  of  the  orbit  of  the 
affected  side.  -  ' 

The  Various  Lid  Signs. — The  Graefe  Sign. — This  sign,  which  con- 
sists in  the  impairment  of  the  consensual  downward  movement  of  the 
upper  lid  with  that  of  the  globe  (see  page  351),  was  described  by  Von 
Graefe  ^^  in  1864.  Though  present  in  many  cases  of  exophthalmic 
goitre,  it  cannot  be  considered  to  be  pathognomonic  of  this  disease,  for 
it  may  not  infrequently  be  demonstrated  upon  the  eyes  of  healthy  indi- 


FiG.  3.— Graefe's  lid  sign  in  exophthalmic  goitre.    (From  Wilhrand  and  Saenger.) 


viduals.  Thus,  Sharkey  ^^  observed  it  twelve  times  among  six  hundred 
and  thirteen  cases  of  all  kinds  of  disease,  exophthalmic  goitre  excluded. 
The  same  author  found,  too,  that  a  large  proportion  of  healthy  people 
Can  voluntarily  produce  this  sign  and  the  one  next  to  be  described — the 
Dalrymple — by  staring  at  an  object  in  front  of  them.  The  writer  has 
frequently  noticed  that  when  patients  are  first  made  to  fix  a  near  object 
in  testing  the  balance  of  the  extraocular  muscles,  the  palpebral  fissure 

53 


834  THE    EYE    AND    NERVOUS    SYSTEM 

frequently  widens  and  the  Graefe  sign  can  be  demonstrated.  In  such 
cases,  however,  in  a  few  moments,  especially  after  several  trials  have 
been  made,  the  fissures  assume  their  normal  width,  and  the  normal 
relationship  between  the  movement  of  the  lids  and  the  globe  is  regained, 
showing,  in  the  opinion  of  the  writer,  that  the  anomalous  behavior  of 
the  lids  and  globe  was  due  to  a  temporary  incoordination  produced  by 
a  confusion  in  the  mind  of  the  patient  as  to  what  particular  demand 
was  being  made  upon  him.  Hughlings  Jackson,^^  Wilbrand  and  Saen- 
ger,^*  and  Flatan  ^^  also  have  observed  the  symptom  in  healthy  indi- 
viduals. Raymond  ^^  noted  it  in  two  cases  of  Thomsen's  disease. 
Kocher  ^^  and  Jessup  have  shown  that  similar  phenomena,  but  to  a 
lesser  degree,  may  be  produced  by  instilling  cocaine  into  the  conjunctiva, 
and  deemed  the  sign  to  be  occasioned  in  these  cases  by  a  direct  stimu- 
lation of  the  sympathetic  nerve-endings,  and  a  consequent  contraction 
of  the  orbital  muscles  supplied  by  these  nerves.  As  further  evidence 
that  the  Graefe  sign  is  not  pathognomonic  of  exophthalmic  goitre,  it 


Fiu.  4.— Dalrymple  lid  sign  in  a  healthy  subject. 

may  be  remarked  that  there  are  many  cases  of  this  disease  in  which 
it  does  not  occur  at  all ;  tlius,  Mannheim  ^®  observed  it  but  eighteen 
times  in  forty-one  cases,  and  Russell  Reynolds  ^®  in  but  four  out  of 
forty-nine.  Passler  ^^  saw  it  but  nine  times  among  fifty-eight  cases ; 
Bruns^^  ten  times  out  of  forty-two  cases;  Flatan^'  sixteen  times 
among  thirty  cases,  and  Murray  ^^  thirty-six  times  among  ninety-one 
cases. 

An  impaired  movement  similar  to  that  observed  in  the  upper  lids  has 


EXOPHTHALMIC  GOITRE  835 

"been  noted  upon  several  occasions  in  the  ascent  of  the  lower  lids  also,  but 
as  Maude  has  pointed  out,  this  is  never  so  marked  as  in  the  upper  lid,  on 
account  of  the  greater  range  in  the  downward  rotation  of  the  globes. 
The  Graefe  sign  occurs  quite  independently  of  the  degree  of  the  exoph- 
thalmus,  and  may  disappear  during  the  course  of  the  disease  and  re- 
appear again  without  the  exophthalmus  having  changed  its  degree. 

The  Dalrymple  Sign.* — This  refers  to  the  peculiar  staring  expression 
which  patients  with  exophthalmic  goitre  present.  It  is  occasioned  by 
an  abnormal  widening  of  the  palpebral  fissure,  due  to  retraction  of  the 
eyelids,  and  gives  rise  to  one  of  the  most  striking  symptoms  of  the 
disease  (Fig.  4).      (Compare  Page  350). 

The  Stellwag  Sign. — This  is  the  name  applied  to  the  infrequency  and 
incompleteness  with  which  the  act  of  winking  is  performed.  It  is  a 
very  marked  symptom  in  some  cases  and  may  lead  to  corneal  ulceration. 

Too  frequent  nictitation  has  been  observed  in  some  cases;  thus, 
Hitschmann  ^^  has  reported  a  case  where  the  act  of  winking  was  per- 
formed about  thirty  times  a  minute. 

Both  the  Dalrymple  and  Stellwag  signs  may  be  confined  to  one  eye, 
though  they  usually  occur  in  both.  They  are  more  marked  at  times 
than  at  others,  being  increased  by  fatigue,  excitement,  or  anxiety.  They 
may  each  exist  independently  or  in  combination  with  one  another  or 
with  exophthalmus  and  the  Graefe  sign.  The  figures  of  Wilbrand  and 
Saenger  ^*  are  of  interest  in  this  connection.  Among  thirty-nine  cases 
of  exophthalmic  goitre  seen  by  these  authors,  exophthalmus  was  present 
in  twenty-seven  cases,  in  twenty-three  of  which  the  exophthalmus  was 
bilateral  and  in  four  unilateral.  Among  these  twenty-three  cases,  ex- 
ophthalmus, Graefe  sign  and  Stellwag  sign  occurred  simultaneously  in 
five  cases ;  exophthalmus,  Graefe  sign,  and  Stellwag  sign  occurred  simul- 
taneously in  four  cases  in  the  same  eye ;  exophthalmus,  Graefe  sign, 
and  Stellwag  sign  in  fourteen  cases ;  exophthalmus,  Graefe  sign,  and 
Stellwag  sign  in  four  cases ;  exophthalmus,  Graefe  sign,  and  Stellwag 
sign  were  absent  in  six  cases ;  Graefe  and  Stellwag  signs  in  one  case ; 
Stellwag  sign  alone  in  two  cases ;   Graefe  sign  only  in  three  cases. 

Mannheim's  ^^  statistics  show  that  among  forty-one  cases  of  exoph- 
thalmic goitre  the  Graefe,  Dalrymple,  and  Stellwag  signs  existed  at  the 
same  time  in  ten  cases,  and  that  six  showed  the  Graefe  sign  without 
the  other  two,  while  the  Graefe  sign  was  absent  and  the  two  others 
present  in  four  cases. 

•  This  sign  is  frequently  included  as  part  of  the  Stellwag  sign,  but  the  credit 
of  its  discovery  should  be  given  to  Dalrymple,  who  first  pointed  it  out  twenty  years 
before. 


836  THE    EYE    AND    XERVOUS    SYSTEM 

Despite  these  figures,  however,  it  is  probable  that  if  carefully 
searched  for,  all  three  of  the  signs  will  be  found  at  some  stage  of  the 
disease,  and  while  their  presence  cannot  be  considered  to  be  pathog- 
nomonic of  exophthalmic  goitre,  it  is  of  great  value  in  confirming  other 
symptoms. 

The  Mbbius  Sign. — According  to  Mobius,^®  unilateral  convergence 
is  normal  in  exophthalmic  goitre,  but  the  force  of  binocular  convergence 
is  sensibly  diminished,  quite  independent  of  any  mechanical  obstruction 
offered  to  the  proper  convergence  of  the  globes  by  extreme  exophthalmus 
or  from  any  paralysis  of  the  ocular  muscles.  This  weakness  does  not 
occasion  diplopia,  but  evidences  itself  in  a  troublesome  asthenopia,  which 
frequently  leads  the  patient  to  consult  an  oculist  for  relief. 

Paralysis  of  Extraocular  Muscles. — In  cases  of  marked  exophthal- 
mus there  is  frequently  more  or  less  restriction  in  the  movements  of 
excursion  of  the  eyeballs,  although,  as  the  degree  of  this  limitation  is 
about  the  same  in  both  eyes,  the  patient  suffers  but  little  upon  that 
account.  There  may,  however,  be  actual  palsy  of  the  extraocular 
muscles. 

Interesting  monographs  upon  this  subject  have  been  written  by 
Ballet,^"  Liebrecht,'^®  Buschan,^^  Mannheim,*^  and  Mobius."*^  It  would 
appear,  from  the  cases  reported  and  cited  by  these  authors,  that  the 
paralysis  may  affect  a  single  muscle  or  group  of  muscles  of  one  eye, 
or  even  all  the  extrinsic  muscles  of  one  eye ;  and  that  occasionally  one 
or  more  muscles  of  both  eyes  are  simultaneously  affected.  Palsy  of  the 
ciliary  muscle  or  iris  is  not  recorded,  nor  of  any  of  the  associated  move- 
ments of  the  eyes,  with  the  exception  of  reports  by  Schmidt-Rimpler  *^ 
and  Vossius  *^  of  cases  of  paralysis  of  convergence.  Palsy  of  the  levator 
palpebral  superioris  in  conjunction  with  palsy  of  the  other  branches  of 
the  third  nerve  is  rare,  but  is  rather  more  common  without  such  asso- 
ciation. The  palsy  may  come  on  early  in  the  course  of  the  disease,  but 
in  most  cases  it  is  a  late  manifestation ;  though  usually  persistent,  it 
may  be  but  a  transient  condition,  the  muscles  regaining  their  full 
strength. 

The  palsy  may  occur  without  paralysis  of  other  cranial  nerves,  or 
it  may  be  associated  with  a  similar  affection  of  one  or  more  of  them ; 
thus,  Bristowe  **  has  recorded  the  case  of  a  young  man  who  developed 
the  symptoms  of  ophthalmoplegia  externa  three  years  after  the  ordinary 
symptoms  of  Graves's  disease  had  manifested  themselves.  Tliere  was 
also  right  hemiana^sthesia,  with  color-blindness  and  loss  of  taste  and 
smell  on  the  same  side,  some  palpitation  and  dyspnoea  with  headache 
and  sickness.     After  a  period  of  some  months,  epileptic  fits  came  on. 


EXOPHTHALMIC  GOITRE  837 

and  later  on  hemorrhages  from  both  ears.  Death  finally  occurred  from 
bronchitis.     Autopsy  was  negative. 

Similarly,  in  a  case  reported  by  Warner  *^  there  was  binocular  exter- 
nal ophthalmoplegia  with  palsy  of  the  facial  and  trigeminal  nerves. 
Jendrassik  ^^  saw  paresis  of  the  muscles  of  mastication,  in  addition  to 
palsy  of  the  rotary  muscles  of  both  globes,  as  well  as  most  of  the  mus- 
cles of  the  face  and  palate.  Of  interest  in  this  connection  also  are  four 
cases  reported  by  Ballet ;  ^^  the  first,  the  subject  of  severe  hysteria,  in 
addition  to  ophthalmoplegia  externa,  presented  a  number  of  bulbar 
symptoms, — i.e.,  palsy  of  both  facials  and  paresis  of  the  hypoglossus. 
In  the  second,  also  the  subject  of  severe  hysteria,  there  was  right-sided 
hemianesthesia  and  loss  of  smell  and  taste  on  the  same  side.  Autopsy 
showed  no  visible  change  in  the  central  nervous  system.*  In  a  third 
case,  in  addition  to  palsy  of  both  external  rectus  muscles,  there  was 
right-sided  hemiplegia  and  hemiansesthesia,  with  epileptic  attacks.  The 
fourth  case  was  an  instance  of  ophthalmoplegia  externa  with  paresis 
of  the  facial  and  hypoglossus.  Chevalier  ^^  saw  palsy  of  the  externi 
and  oblique  muscles  associated  with  disturbance  in  the  supply  of  the 
fifth  and  seventh  nerves  and  of  speech. 

In  a  case  of  Maude's  ^^  the  lesion  seemed  to  spread  over  the  entire 
region  of  the  nuclei  of  the  extraocular  muscles  and  those  neighboring 
upon  them ;  paresis  of  the  external  rectus,  and  probably  of  the  superior 
oblique,  being  preceded  by  palsy  of  the  facial,  and  quickly  followed  by 
general  ophthalmoplegia. 

Stellwag's  *^  case  was  one  of  paralysis  of  the  lateral  rotators  (the 
ocular  axes  being  parallel  and  convergence  undisturbed),  and  later  palsy 
of  both  externi  with  convergent  strabismus  and  diplopia  developed  with 
transient  anaesthesia  and  restricted  movement  of  the  left  half  of  the 
upper  legs. 

In  a  case  reported  by  Fereol,^"  of  a  man  41  years  old,  six  months 
after  the  appearance  of  the  initial  symptoms  of  exophthalmic  goitre 
there  were  headache,  vomiting  and  dizziness,  tremor,  with  disturbed 
gait,  and  a  disposition  to  face  to  the  right ;  then  diplopia,  the  result  of 
right-sided  trochlearis  palsy.  There  was  also  right-sided  diminution 
of  motion  and  hyperalgesia,  while  on  the  left  side  there  was  a  diminu- 
tion in  sensation  of  pain. 

Quite  recently  Voss,^^  of  St.  Petersburg,  has  reported  two  additional 
cases  of  palsy  of  the  extraocular  muscles  in  exophthalmic  goitre.  '  The 
palsy  in  the- first  case  had  affected  the  right  superior  and  external  rectus 
muscles  of  the  right  eye  and  the  inferior  and  external  rectus  muscles 
of  the  left  eye.  There  was  also  a  partial  palsy  of  the  right  facial  nerve, 
with  some  cerebral  symptoms. 


838  THE    EYE    AND    NERVOUS    SYSTEM 

The  second  case  exhibited  multiple  lesions  in  the  muscles  of  both 
eyes,  the  superior  rectus  muscle  of  the  right  eye  being  particularly 
affected. 

Voss  refers  to  a  case  of  Bartholow's  where  there  was  ptosis  in  asso- 
ciation with  gangrene  and  neuritis  of  the  lower  extremities. 

Dyson  ^^  reported  the  case  of  a  man  with  the  symptoms  of  incipient 
Graves's  disease,  in  which,  in  addition  to  slight  exophthalmus  and  the 
Graefe  lid  sign,  there  was  slight  internal  convergent  strabismus  and  dila- 
tation of  the  pupil  of  the  left  eye.    The  patient  was  a  chronic  alcoholic. 

Bruns  ^'  saw  ptosis  on  the  left  side,  with  palsy  of  the  superior  recti 
in  both  eyes,  with  the  cardinal  symptoms  of  exophthalmic  goitre,  but 
as  the  patient  was  the  subject  of  a  chronic  nephritis,  it  was  thought 
that  the  nuclear  lesion  might  be  referable  to  this  source  and  not  to 
Graves's  disease. 

Wilbrand  and  Saenger  ^*  report  a  case  with  slight  ptosis  on  the  left 
side,  with  retraction  of  the  upper  lid,  with  Graefe  lid  sign,  upon  the 
right  side.     This  was  explained  by  a  bilateral  affection  of  the  nuclei  of 


Fio.  5.— Paralysis  of  eye  muscles  in  ezopbtbalmic  goitre. 

the  levator, — i.e.,  an  irritation  of  the  levator  on  the  right  and  a  palsy 
on  the  left  side. 

De  Giovanni  '^  has  reported  a  case  of  imilateral  exophthalmus  as  a 
consequence  of  palsy  of  the  extraocular  muscles,  especially  of  the  rectus 
superior,  in  which  goitre  and  other  classical  symptoms  of  Graves's  dis- 
ease were  present. 

The  accompanying  figure  is  that  of  a  patient  presented  by  the  writer 
before  the  College  of  Physicians  of  Philadelphia,  in  whom  there  were 


EXOPHTHALMIC  GOITRE  839 

all  the  cardinal  signs  of  exophthalmic  goitre,  and,  in  addition,  paralysis 
of  all  the  extraocular  muscles  of  the  left  eye,  with  the  exception  of  the 
superior  oblique. 

In  addition  to  these  cases  of  more  or  less  complete  ophthalmoplegia 
externa,  others,  though  more  uncommon,  of  palsy  of  a  single  eye-muscle 
have  been  reported.    These  have  been  grouped  by  Buschan,^^  as  follows: 

(1)  Cases  in  which  diplopia  luas  complained  of,  hut  the  muscles  at 
fault  not  ascertained:  Trousseau  Smith,  Chvostek,  Stellwag,  Impac- 
cianti,  Strlimpell,  F.  Miiller,  Lang-Pringle.  (2)  Cases  of  palsy  of  the 
rectus  internus:  West,  Ilomen,  Cohen,  Westedt,  and  Romberg  (rectus 
■interni  of  both  eyes)  ;  Schoch  and  Rothmann  (rectus  internus  and  ex- 
ternus).  (3)  Cases  of  palsy  of  the  rectus  externus:  Kurella,  Mac- 
kenzie (several  cases),  Eulenburg,  Stellwag,  Makeig  Jones,  Liebrecht, 
Dyson,  Ballet,  Cohen.  (4)  Cases  of  palsy  of  the  rectus  superior:  Sol- 
lier,  Fischer-Degranges,  Kahler,  Roth,  Schlesmger,  Chvostek.  (5) 
Cases  of  palsy  of  the  trochlearis :  Fereol.  (6)  Cases  of  palsy  of  the 
levator  palpebrce  of  both  eyes:   Maude,  West. 

Changes  in  the  Cornea.. — Notwithstanding  the  exposure  to  which 
the  eyeball  is  subjected  by  the  widening  of  the  palpebral  fissure  and 
by  the  extreme  degree  of  exophthalmus  in  many  instances,  and  to  the 
lessening  in  its  moisture  by  the  infrequent  winking,  and,  furthermore, 
by  the  areas  of  anaesthesia,  which  may  be  demonstrated  in  the  cornea 
in  some  cases  of  exophthalmic  goitre,  ulceration  of  that  membrane 
occurs  but  rarely,  appearing  to  depend  more  upon  individual  predis- 
position than  upon  any  of  the  foregoing  factors,  those  who  are  feeble  and 
cachectic  as  well  as  those  in  advanced  years,  being  particularly  liable 
to  severe  corneal  ulceration.  Corneal  complications  show  a  tendency 
to  occur  more  frequently  among  men  than  women,  which  is  the  more 
striking,  in  view  of  the  fact  that  exophthalmic  goitre  is  far  more  com- 
mon among  females.  This  may  possibly  be  explained  by  the  greater 
severity  with  which  the  disease  attacks  men  and  also  by  the  advanced 
age  at  which  they  are  affected. 

In  cases  where  the  cornea  is  but  slightly  diseased,  its  epithelium 
becomes  thickened,  giving  a  dull  and  lustreless  appearance  to  that  mem- 
brane. The  conjunctival  vessels  are  usually  dilated,  as  a  consequence 
of  which  the  patient  experiences  sensations  of  more  or  less  irritation 
and  dryness  of  the  eyes,  especially  after  reading.  In  more  severe  cases 
one  or  more  small  yellowish -gray  areas  of  infiltration  appear  under 
the  epithelium.  The  epithelium  over  these  areas  is  thrown  into  folds 
and  finally  breaks  down  into  shallow  ulcers.  In  other  cases,  where  the 
exophthalmus  is  of  extreme  degree  and  the  lids  do  not  close  and  properly 


840  THE    EYE    AND    NERVOUS    SYSTEM 

protect  the  cornea  at  night,  the  lower  third  of  the  cornea  becomes  cov- 
ered with  thick  yellow  crusts,  beneath  which  ulcers  form,  and  not  infre- 
quently necrosis  of  the  cornea  and  perforation  of  the  eyeball  ensues. 
It  should  be  remarked,  however,  that  extensive  ulceration  may  occur 
in  individuals  where  there  is  but  slight  exophthalmus,  and  in  cases  even 
where  there  is  a  plentiful  supply  of  tears,  the  process  in  such  instances 
being  similar  to  that  observed  in  keratitis  of  a  neuroparalytic  origin, 
the  so-called  keratitis  xerotica. 

Anomalies  of  Lachrymation. — Berger  °'  called  attention  to  a  num- 
ber of  cases  in  which  he  claims  that  epiphora  was  the  initial  symptom 
of  exophthalmic  goitre,  and  Vemean  and  Diaman  Herger  have  reported 
similar  cases.  The  first  author  regards  the  epiphora  as  a  secretory 
neurosis  of  the  lachrymal  gland,  being  due  to  a  reflex  condition  of  sen- 
sory excitation  of  the  sympathetic  vasomotor  fibres.  Most  authors, 
however,  agree  with  Sattler  in  ascribing  the  epiphora  to  two  factors: 
(1)  The  increased  width  of  the  palpebral  fissure,  whereby  a  larger 
portion  of  the  conjunctiva  is  exposed  to  the  action  of  the  air  than  is 
usual,  and  thus,  being  irritated,  excites  reflexly  an  increased  flow  of 
tears,  and  (2)  the  interference  with  the  proper  sucking  up  of  the  tears 
by  tl?e  lachrymal  apparatus,  as  a  consequence  of  the  imperfect  and 
infrequent  winking  and  closing  of  the  lids. 

In  cases  where  the  disease  is  of  long  standing,  there  is  frequently 
a  diminution  in  the  amount  of  tears,  and  an  actual  dryness  of  the  eye 
ensues;  this  may  be  accredited  to  a  lessening  in  the  sensibility  of  the 
sensory  corneal  and  conjunctival  nerves,  as  the  result  of  exposure  and 
irritation,  and  to  a  corresponding  lessening  in  the  reflex  secretion  of 
tears.      (Compare  Page  344.) 

Unusual  Ocular  Manifestations. — Obliteration  of  the  Sulcus  Orbito- 
palpebralis  Superior. — In  spite  of  the  emaciation  which  is  often  seen  in 
subjects  of  exophthalmus,  the  sulcus,  which  is  usually  deep  under  such 
circumstances,  may  be  obliterated,  and  in  its  place  a  sac-like  fulness  of 
the  lid  may  appear,  which  is  occasioned  by  the  increase  in  the  contents 
of  the  orbit  due  to  the  fulness  and  stretching  of  the  blood-vessels. 

CEdema  of  the  Lids. — CEdema  of  the  lids  is  sometimes  present ;  it 
may  be  an  early  symptom,  or  may  not  come  on  until  after  some  years. 
Such  swelling  is  often  very  persistent,  and  may  remain  after  all  the 
other  symptoms  have  disappeared.  Mackenzie  '*  considered  it  to  be 
somewhat  akin  to  the  swelling  of  myxcedema  and  to  be  quite  independent 
of  the  exophthalmus. 

A  bag-like  swelling  of  the  under  lid,  with  dilatation  of  the  veins 
at  the  ciliary  border,  associated  with  severe  pain  in  the  forehead  and 


EXOPHTHALMIC  GOITRE  841 

over  the  vertex,  has  been  described  by  Stellwag.^^  Vigouroux  ^°  found 
a  false  oedema  of  the  lids  to  be  of  frequent  occurrence.  He  considered 
this  to  be  dependent  upon  paresis  of  the  orbicularis,  as  he  observed  that 
when  this  muscle  was  made  to  contract  by  electric  excitation,  the  swell- 
ing disappeared. 

Tremor  of  the  Lids, — Homen  ^^  has  noticed  a  tremor  in  the  upper 
lids  when  the  eyes  were  closed,  w^hich  differed  from  the  tremor  seen  in 
the  lids  in  other  diseases  of  the  nervous  system  and  occasionally  in 
health  by  the  rapidity  with  which  it  manifested  itself  after  the  lids 
were  closed,  by  its  regularity  and  rhythm,  and  its  extremely  fine  char- 
acter. A  similar  tremor  was  noted  by  Liebrecht.^^  In  this  case  the 
motion  in  the  lid  could  be  stopped  after  a  few  moments  by  pressure 
upon  the  supraorbital  and  facial  nerves. 

Nystagmus. — Cases  of  nystagmus  have  been  reported  by  Bramwell  ®^ 
and  Freund.^^  The  latter  author  believes  the  movements  in  the  eye- 
balls, although  differing  in  rhythm,  to  be  analogous  to  the  tremor  in 
the  extremities. 

Changes  in  the  Fundus  Oculi. — With  the  exception  of  a  tortuosity  and 
dilatation  of  the  retinal  veins  which  are  present  in  some  cases,  changes 
in  the  retinal  vessels  are  very  uncommon.  O,  Becker  ^^  saw  a  dilata- 
tion of  their  walls  and  the  presence  of  a  pulse  isochronous  with  the 
radial  pulse  in  the  retinal  arteries  in  ten  out  of  thirteen  cases,  but  most 
observers  have  failed  to  substantiate  these  findings. 

In  like  manner,  changes  in  the  optic  nerves  are  uncommon,  and  the 
cases  of  double  optic  neuritis  which  have  been  recorded  by  Story  ^^ 
and  Rieger  and  Eorster  ^"^  and  the  two  cases  of  optic  atrophy  seen  by 
Emmet  ^^  should  be  regarded  as  complications  rather  than  as  symptoms 
of  the  disease. 

Refraction. — In  thirty  cases  examined  by  him,  Griffith  ^^  found  the 
refraction  to  be  normal  in  twenty-two  instances.  Of  the  remaining 
eight,  four  were  myopic,  one  highly  hypermetropic,  and  three  astig- 
matic. 

Field  of  Vision.  — Notwithstanding  the  observations  of  Kast  and  Wil- 
brand,"^"  who  found  concentric  narrowing  of  the  field  of  vision  with  or 
without  impairment  of  the  central  vision  or  the  color  sense  in  subjects 
free  from  hysteria,  but  exhibiting  more  or  less  signs  of  cardiac  palpi- 
tation, the  visual  fields  in  exophthalmic  goitre,  except  in  so  far  as  they 
may  be  influenced  by  an  accompanying  hysterical  condition,  are  normal 
(see  page  638). 

The  Cutaneous  System. — The  changes  in  the  integument  may  affect 
(a)  the  vasomotor  system,  occasioning  at  times  the  so-called  "  tache  cere- 


842  THE    EYE    AND    NERVOUS    SYSTEM     • 

bral,"  oedema,  and  peripheral  changes  in  temperature.  Of  these  the 
"  tache  cerebral,"  the  transient  redness  which  follows  even  the  slightest 
touch  of  the  skin,  is  a  not  infrequent  symptom  of  exophthalmic  goitre, 
and  was  attributed  by  Trousseau  '^  to  a  marked  asthenia  of  the  vaso- 
motor apparatus.  CEdema  may  be  either  general  or  local.  It  occurs 
for  the  most  part  in  debilitated  subjects  and  is  influenced  by  gravity, 
though  there  are  other  forms  of  circumscribed  oedema  probably  akin 
to  those  observed  in  myxoedema.  Increase  in  the  bodily  temperature 
has  been  noted  not  rarely,  and  may  occur  in  all  the  stages  of  the  disease 
without  apparent  cause.  This  actual  increase  in  temperature  is  to  be 
differentiated  from  the  sensation  of  excessive  heat,  without  increase  in 
temperature,  which  is  at  times  complained  of  and  to  which  Charcot 
has  assigned  the  name  of  thermophobia. 

(b)  The  glands, — excessive  sweating.  Excessive  sweating  upon 
slight  cause  is  one  of  the  most  frequent  symptoms  of  the  disease,  and 
may  be  local,  as,  for  example,  when  it  is  confined  to  the  hands,  or,  what 
is  more  often  the  case,  it  may  involve  the  entire  surface  of  the  body. 
It  may  be  confined  to  one-half  of  the  body.  Diarrhoea  is  sometimes  most 
troublesome,  and  is  thought  to  be  a  kind  of  intestinal  sweating. 

(c)  The  skin — pigmentation.  Pigmentation  of  the  skin  has  been 
observed  by  a  number  of  authors.  In  most  cases  it  assumes  a  bronze 
tint,  comparable  to  that  in  Addison's  disease,  though  never  so  pro- 
nounced as  in  advanced  cases  of  this  affection.  Vitiligo  and  urticaria 
eruption,  and  a  double  form  of  rash  consisting  of  dusky  papules  like 
the  eruption  of  measles  in  its  later  stages,  have  also  been  observed.  An 
abnormal  dryness  of  the  skin  has  been  noted  in  severe  cases. 

It  was  noted  by  Vigouroux  '^  that  in  many  cases  of  exophthalmic 
goitre  there  was  a  decided  decrease  in  the  normal  resistance  offered  by 
the  skin  to  the  passage  of  the  galvanic  current.  This  he  thought  was 
due  to  hyperidrosis.  As  this  symptom  does  not  occur  in  other  diseases 
where  the  perspiration  is  abnormal,  and  may  even  occur  in  health,  it 
cannot  be  considered  to  be  pathognomonic. 

The  hair.  — Loss  of  hair  frequently  occurs  from  the  scalp,  axilla?, 
and  pubes.  Thus,  Mannheim  ^^  observed  this  symptom  in  fifteen  out 
of  forty-one  cases,  and  Yeo  ^*  reports  an  interesting  case  where  the 
hair  fell  out  of  the  eyebrows  and  eyelids  coincidently  with  the  protru- 
sion of  the  eyeballs.  It  is  of  interest  to  note  that  the  loss  of  hair  is 
often  unilateral. 

Respiratory  System. — Disorders  of  respiration  are  not  uncommon 
and  are  occasioned  either  by  irritation  or  pressure.  Among  the  former 
may  be  included  the  attacks  of  coughing  which  resemble  laryngeal  crises. 


EXOPHTHALMIC  GOITRE  843 

In  many  of  these  cases  the  dyspnoea  may  be  very  great  and  death  from 
asphyxia  may  ensue.  There  may  be  but  little  secretion,  but  at  times 
there  is  a  profuse  bronchorea,  which  is  conceded  to  be  due  to  vasomotor 
paralysis. 

Digestive  System. — Disturbance  in  the  gastro-intestinal  tract  occurs 
in  at  least  half  of  the  cases,  and  usually  takes  the  form  of  a  serous  diar- 
rhoea, which  appears  in  paroxysms  and  without  colicky  pains.  These 
attacks  may  be  most  persistent,  and  may  even  end  fatally. 

Generative  System. — Although  many  observers  are  of  the  opinion 
that  menstrual  disorders  are  a  frequent  cause  of  exophthalmic  goitre, 
there  is  no  evidence  to  prove  this  or  to  indicate  that  the  quite  frequent 
disturbances  in  menstruation  which  occur  in  conjunction  with  this  dis- 
ease are  more  than  functional  derangement,  the  result  of  anaemia  and 
chlorosis. 

Urinary  System. — Polyuria  is  of  common  occurrence,  and  is  de- 
pendent upon  vasomotor  activity,  being  analogous  to  the  excessive  sweat- 
ing and  the  diarrhoea.  Transitory  albuminuria  has  been  reported  in  a 
number  of  cases.  Glycosuria  is  a  frequent  symptom,  depending  in 
some  cases  upon  true  diabetes,  in  others  upon  simple  so-called  alimen- 
tary glycosuria. 

Anaemia,  chlorosis,  and  any  condition  which  lowers  the  vitality,  such 
as  prolonged  illness,  loss  of  blood,  amenorrhoea,  sexual  excess,  the  abuse 
of  alcohol,  and  syphilis,  may  predispose  to  the  development  of  the  dis- 
ease ;  cases  have  been  reported  also  where  diseases  of  the  nose  and  pelvic 
organs  were  thought  to  have  originated  the  symptoms.  Among  the 
exciting  causes  are  sudden  fright,  great  grief,  prolonged  fatigue,  and 
violent  physical  strain. 

COURSE,  PROGNOSIS,  AND  FREQUENCY. 

The  disease  begins,  as  a  rule,  very  suddenly,  symptoms  of  palpita- 
tion and  tachycardia  rapidly  developing  after  a  physical  shock.  These 
either  persist,  or,  what  is  more  common,  subside,  to  be  re-excited  again 
by  similar  causes  until  the  symptoms  remain  permanent.  The  future 
course  of  the  disease  is  usually  a  chronic  one,  and  it  may  persist  for 
years  with  occasional  variations  in  severity.  Complete  recovery  occurs 
in  some  cases,  but,  as  a  rule,  some  of  the  symptoms  remain  until  death 
ensues  from  some  intercurrent  disease. 

Prognosis. — The  prognosis,  as  far  as  life  is  concerned  and  also  for 
a  comparatively  short  duration  of  the  disease,  is  good,  provided  the 
cases  are  submitted  early  in  their  course  for  treatment  and  that  the 
subjects  are  young.     The  prognosis  is  more  favorable  in  women  than 


844  THE    EYE    AND    NERVOUS    SYSTEM 

in  men.  Though  rapid  onset  may  be  followed  by  rapid  recovery,  re- 
covery is  usually  more  rapid  and  permanent  in  cases  where  the  symp- 
toms develop  more  slowly.  The  appearance  of  grave  complications, 
such  as  vomiting,  diarrhoea,  and  the  signs  of  cardiac  failure,  are  always 
of  serious  import,  as  they  may  occasion  a  fatal  termination.  Exoph- 
thalmic goitre  of  itself  is  seldom  fatal. 

Frequency. — Exophthalmic  goitre  is  a  rare  disease;  it  occurs  in 
all  lands  and  shows  a  preference  to  affect  individuals  between  20  and 
30  years  of  age.  Thus,  of  four  hundred  and  ninety-five  cases  collected 
by  Buschan  (four  hundred  and  seven  females  and  eighty-eight  males), 
the  statistics  were  as  follows : 

Females.  Males. 

From  two  and  a  half  to  ten  years  of  age 12  3 

From  eleven  to  fifteen  years  of  age  17  11 

From  sixteen  to  twenty  years  of  age 83  10 

From  twenty-one  to  thirty  years  of  age 144  19 

From  thirty-one  to  forty  years  of  age 79  17 

From  forty-one  to  fifty  years  of  age 51  18 

From  fifty-one  to  sixty  years  of  age 21  10 

Sex. — Buschan  collected  nine  hundred  and  eighty  cases  in  the  litera- 
ture, and  found  of  this  number  eight  hundred  and  five  to  be  women 
and  one  hundred  and  seventy-five  men  =  1 : 4,  6. 

PATHOLOGY. 

Since  the  recognition  of  Graves's  disease  as  a  distinct  morbid  entity 
numerous  theories  have  been  advanced  to  account  for  its  causation,  and 
it  has  variously  been  imputed  to  a  dyscrasia,  to  intestinal  intoxication,  to 
disease  of  the  nervous  system,  and,  finally,  to  a  disturbance  in  the  action 
of  the  thyroid  gland  itself.  Of  these  theories  the  most  tenable  are  those 
which  are  based  upon  either  a  disease  of  the  nervous  system  or  of  the 
thyroid  gland.  Regarding  the  former,  it  may  be  said  that  although 
observers  have  designated  nearly  every  portion  of  the  nervous  system 
as  the  primary  seat  of  the  disease,  our  present  knowledge  of  the  subject 
permits  us  to  eliminate  all  parts  other  than  the  medulla  oblongata  and 
the  sympathetic  nervous  system. 

While  it  may  be  stated  that  it  has  been  demonstrated  that  symptoms 
of  exophthalmic  goitre  may  be  occasioned  by  interference  with  the  nerve- 
centres  in  the  medulla  oblongata,  and  though  definite  lesions  have  been 
found  in  this  part  of  the  brain  in  some  autopsies  which  have  been  made 
upon  fatal  cases  of  this  disease,  there  is  not  yet  sufficient  evidence  to 
prove  that  an  organic  lesion  of  the  medulla  is  -necessary  to  produce 
exophthalmic  goitre.  Nor  is  there  ground  to  warrant  the  assertion  that 
the  sympathetic  nervous  system  is  primarily  at  fault,  though  many  of 


EXOPHTHALMIC  GOITRE  845 

the  chief  symptoms  of  the  disease  may  be  explained  by  an  affection  of 
that  part  of  the  nervous  system,  and  though  lesions  have  been  found 
there  in  cases  of  Graves's  disease. 

As  regards  the  gland  itself,  while  it  is  evident  from  microscopical 
studies  that  there  is  a  great  increase  in  the  amount  of  the  secreting  gland 
tissue  in  exoj)hthalmic  goitre,  and  from  other  observations  that  it  is  not 
improbable  that  there  is  an  alteration  in  the  character  of  the  secretion 
as  well  as  its  quantity,  and  that  the  symptoms  of  the  disease  may  be 
due  to  an  action  of  an  excess  of  this  altered  secretion  of  the  gland  upon 
the  tissues,  and  especially  upon  the  nerve  nuclei  of  the  medulla,  the 
entire  question  of  the  essential  factor  or  factors  in  the  production  of 
exophthalmic  goitre  must  still  remain  sub  judice. 

TREATMENT. 

The  treatment  of  Graves's  disease  resolves  itself  into  a  general 
hygienic,  medical,  electrical,  and  surgical. 

General  Hygiene. — The  chief  indication  in  the  treatment  of  Graves's 
disease  is  rest.  Most  patients  do  best  upon  a  prolonged  course  of  strictly 
enforced  rest  cure,  the  treatment  being  protracted  over  a  period  of  one 
or  more  months  of  absolute  rest  in  bed.  Where  this  is  impracticable 
or  seems  undesirable,  bodily  and  mental  rest,  as  far  as  is  possible,  should 
be  installed,  with  the  avoidance  of  all  but  the  slightest  amount  of  physi- 
cal exercise  and  the  withdrawal  of  the  patient  from  all  contact  with 
that  which  might  w^orry  or  excite  the  mind.  Xo  specific  regimen  can 
be  prescribed  as  suitable  for  all  cases,  the  physician  being  compelled 
to  adjust  the  amount  of  exercise  and  rest  for  each  individual.  All 
violent  exercise  should  be  proscribed,  and  gymnastic  exercises,  except 
perhaps  the  passive  mechanical  exercise  employed  by  Bryson,  are  to  be 
avoided.  Patients  do  well  by  the  sea,  and  a  bracing  air  is  usually  of 
advantage.  High  altitudes  should  be  avoided.  Long  sea-voyages  are 
often  beneficial,  provided  the  patient  is  not  subject  to  sea-sickness. 
Alcoholic  stimulants  are  only  to  be  prescribed  when  the  heart  is  very 
weak  or  assimilation  very  poor.  Tea,  coffee,  and  tobacco  should  be  pro- 
hibited. The  free  consumption  of  milk  should  be  encouraged,  and 
simple  and  readily  digested  foods  selected ;  but  the  diet  should  always 
be  prescribed  with  reference  to  the  conditions  revealed  after  an  analysis 
has  been  made  of  the  gastric  contents.  If  massage  be  administered,  it 
should  be  given  gently  and  not  too  frequently.  Cold  baths  are  harmful ; 
warm  baths  and  occasional  shower-baths  at  100°  F.  are  of  benefit.  The 
application  of  Leiter's  ice-coil  over  the  thyroid  gland  and  occasionally 
over  the  heart  will  often  diminish  the  exophthalmus,  as  well  as  the 
vascular  excitement. 


846  THE    EYE    AND    NERVOUS    SYSTEM 

Medicinal  Treatment. — The  indications  to  be  met  in  the  adminis- 
tration of  drugs  are  to  lessen  the  excessive  and  abnormal  secretion  of 
the  thyroid  gland,  to  quiet  the  general  nervousness,  to  build  up  the 
general  health  and  overcome  ana?mia.  A  most  efficient  means  to  reduce 
the  size  of  the  gland  consists  in  rubbing  a  small  piece  of  red  iodide  of 
mercury  ointment  over  the  gland  each  night  until  the  skin  becomes  too 
tender  for  the  patient  to  bear  its  further  application.  After  a  few  days 
of  interruption,  this  treatment  may  be  resumed  and  continued  with 
similar  interruptions  for  several  weeks  or  months,  or  the  skin  over  the 
gland  may  be  painted  with  tincture  of  iodine  or  anointed  with  a  com- 
pound consisting  of  half  a  drachm  of  extract  of  belladonna  and  one 
ounce  of  iodine  ointment. 

Belladonna  may  also  be  administered  internally,  beginning  with  ten 
minims  of  the  tincture  three  times  daily  and  continued  until  secretion 
has  been  appreciably  affected.  Iodine  and  the  iodides  have  also  been 
of  advantage  when  given  by  the  mouth  in  some  cases,  though  in  others 
they  have  been  harmful.  Bromides  are  of  service  in  controlling  the 
nervous  irritability,  and  sulphonal,  in  addition  to  its  action  as  an 
hj^'pnotic,  has  been  found  to  have  a  favorable  effect  upon  the  cardiac 
and  general  nervous  systems.  The  valerianates,  sumbul,  and  asafoetida 
are  also  of  service.  Musser  relies  as  standards  upon  iron,  cod-liver  oil, 
and  opium.  Powdered  opium  is  administered  in  doses  of  a  quarter  to 
a  half  of  a  grain  to  quiet  the  erythresium,  control  the  diarrhoea,  and 
stinmlate  the  heart.  Strophanthus  is  often  of  service,  but  contrary  to 
what  might  be  expected,  drugs  that  slow  the  pulse,  like  digitalis  and 
strophanthus,  have  but  little  influence  in  controlling  the  disease,  though 
they  should  be  administered  when  the  heart  is  weak.  Ergot  is  often 
of  decided  value,  and  iron  and  quinine  are  useful  if  there  is  anaemia. 

An  extract  of  the  thymus  gland  has  done  good  in  some  cases,  but 
the  results  from  its  use  have  been  disappointing.  Thyroid  extract  should 
always  be  used  with  caution,  on  account  of  its  liability  to  increase  the 
symptoms.  Powdered  oxide  of  zinc  may  be  employed  to  check  the 
sweating  and  control  the  diarrhoea. 

Electrical  Treatment. — Galvanism  is  of  decided  value  in  many 
cases,  and  is  best  applied  by  placing  the  cathode  over  the  angle  of  the 
jaw,  first  upon  one  side  and  then  on  the  other,  while  the  anode  is  held 
upon  the  back  of  the  neck  over  the  seventh  cervical  vertebrae.  The  cur- 
rent should  be  applied  for  five  minutes  at  a  time,  three  times  daily,  in 
the  strength  of  from  two  to  three  milliamperes.  Vigouroux  employs 
the  induced  current,  the  positive  electrode  being  placed  upon  the  neck 
and  the  smaller  cathode  over  the  carotids ;  this  current  should  be  applied 
once  daily,  ten  to  fifteen  minutes  at  a  time. 


EXOPHTHALMIC  GOITRE 


847 


Surgical  Treatment. — On  account  of  the  uncertainty  attending 
operative  procedures  for  tlie  relief  of  exophthalmic  goitre  and  the  dan- 
ger by  which  they  are  surrounded,  none  of  the  various  forms  of  opera- 
tions which  are  about  to  be  mentioned  should  be  tried  until  all  medicinal 
and  other  therapeutic  methods  have  failed,  and  should  only  be  resorted 
to  then  when  the  disease  is  progressing  and  the  symptoms  distressing 
or  dangerous. 


A.— The  patient  before  operation.  B.— The  same  patient  shortly  after. 

Fig.  6.— Showing  the  Effect  of  Bilateral  Resection  of  the  Cervical  Sympathetic.      (Jonesco  in  Inter- 
national Clinics,  Vol.  I.,  13th  Series.) 


Degeneration  of  the  heart  or  serious  involvement  of  the  nervous 
system  are  contraindications,  except  in  the  rare  cases  where  the  enlarge- 
ment of  the  thyroid  is  so  great  that  the  trachea  is  compressed,  when  a 
part  of  the  gland  should  be  at  once  removed.  According  to  Buschan, 
the  most  suitable  cases  for  operation  appear  to  be  those  in  which  the 
enlargement  of  the  thyroid  gland  has  existed  for  some  time  previous 
to  the  development  of  the  other  symptoms. 


848  THE    EYE    AND    NERVOUS    SYSTEM 

From  the  statistics  which  have  been  compiled  by  Kocher  and  others, 
it  would  appear  that  operative  measures  hold  out  a  fair  chance  of 
improvement  or  even  of  cure  of  cases  of  exophthalmic  goitre,  even  after 
all  medicinal  measures  have  failed,  and  that  tlie  ligature  of  the  thyroid 
arteries  is  equally  efficacious  and  less  serious  than  thyroidectomy. 

In  1896  Jonesco  resected  the  superior  and  middle  cervical  ganglia 
for  exophthalmic  goitre,  and  the  operation  has  been  performed  a  num- 
ber of  times  since  by  others.  Though  the  number  of  cases  which  have 
been  operated  on  is  small,  no  deaths  have  occurred  thus  far,  and  while 
the  results  obtained  by  this  procedure  are  inferior  to  those  observed 
after  ligature  or  partial  thyroidectomy,  the  operation  is  of  value  and 
may  be  u^ed  to  supplement  the  action  of  either  of  these  operations  in 
the  event  of  the  persistence  of  symptoms,  such  as  tachycardia,  and  ex- 
ophthalmus,  after  their  performance. 

Subjects  of  Graves's  disease  bear  general  anaesthetics  badly,  so  that 
whatever  the  operation,  it  is  essential  that  it  should  be  performed  under 
local  anaesthesia. 

Treatment  of  the  Exophthalmus. — In  the  very  early  stages  a  light 
pressure  bandage  often  decreases  the  exophthalmus,  especially  where 
the  perfect  closure  of  the  lids  is  still  possible ;  when  the  exophthalmus 
is  pronounced,  tarsorrhaphy,  or  union  of  the  edges  of  the  lids,  h  indi- 
cated, not  only  for  cosmetic  reasons,  but  also  as  a  prophylactic  measure 
against  corneal  ulceration.  Lateral  tarsorrhaphy  consists  in  shortening 
the  palpebral  fissure  by  uniting  the  edges  of  the  lids  at  the  canthi. 
This  is  accomplished  by  an  incision  into  the  margin  of  the  upper 
and  lower  lids,  which  separates  the  skin  from  the  cartilage,  thus  per- 
mitting the  anterior  layer  containing  the  cilia  to  be  everted  and  the 
groove  between  the  two  layers  to  gap ;  the  raw  surfaces  of  the  lids  are 
then  approximated  by  sutures.  To  insure"  a  good  hold  on  the  edge  of 
the  lid  and  to  prevent  the  sutures  tearing  out  by  the  pressure  of  the 
globe,  it  is  advisable  to  introduce  the  needles  several  millimetres  beyond 
the  border  of  the  lid,  and  not  including  the  latter.  At  times  it  is  neces- 
sary to  combine  a  median  tarsorrhaphy  with  the  lateral,  in  which  event 
a  narrow  strip  of  skin  is  excised  from  the  ciliary  border  of  the  upper 
and  lower  lids  at  their  middle  third,  and  the  lids  then  united  in  the 
manner  just  described. 

Treatment  of  Corneal  Ulceration. — Even  in  cases  where  corneal  ulcera- 
tion is  not  present,  the  precaution  should  be  taken  to  flush  the  conjunc- 
tival cul-de-sacs  two  or  three  times  daily  with  a  solution  of  boracic  acid 
(gr.  X  ad  f  5  i).  When  corneal  ulceration  is  present,  greater  care  should 
be  taken  in  cleansing  the  eye,  and  two  or  three  drops  of  a  solution  of 


EXOPHTHALMIC  GOITRE  849 

atropine  (gr.  iv  ad  f  §  i)  should  be  instilled  night  and  morning.  Hot 
compresses  are  of  service  and  should  be  employed  for  one-half  hour 
at  a  time  three  or  four  times  daily.  The  ulcers  should  be  carefully 
cleansed  with  peroxide  of  hydrogen  (50  per  cent.)  and  a  firm  compress 
bandage  should  be  carefully  applied.  After  the  inflammation  has  sub- 
sided, if  the  central  part  of  the  cornea  is  occupied  by  an  opacity,  it  may 
be  possible  to  improve  vision  by  an  iridectomy.  If  perforation  occurs 
abscission  of  the  anterior  segment  of  the  globe  or  even  enucleation  may 
have  to  be  resorted  to. 

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54 


850  THE    EYE    AND    NERVOUS    SYSTEM 

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*"  Passler,  Deutsche  Zeitschrift  fiir  Nervenheilkunde,  vi.  p.  210. 

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EXOPHTHALMIC  GOITKE  851 

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CHAPTER     XX. 

THE  PSYCHOLOGICAL  EFFECTS  OF  OPERATIONS 
UPON  THE  EYES. 

By  WILLIAM  CAMPBELL  POSEY,  M.D. 

This  subject  may  be  studied  from  two  stand-points, — first,  from  that 
wliich  deals  with  the  effect  upon  the  mind  of  an  operation  which  restores 
sight  to  individuals  who  were  born  blind  or  who  became  so  by  accident 
or  disease,  and,  second,  from  that  which  considers  the  impression  such 
operations  produce  upon  the  mentality  in  the  nature  of  a  shock,  quite 
independent  of  any  influence  which  might  be  exerted  by  the  restoration 
of  sight.  Before  considering  either  of  these  phases,  it  is  necessary  to 
inquire  whether  the  mental  state  of  the  congenitally  blind  differs  from 
that  of  those  born  with  all  their  faculties,  and  to  what  extent  loss  of 
sight  affects  the  mind  of  individuals  who  have  had  both  mind  and  sight 
developed.  In  answer  to  these  questions  it  may  be  stated  that  in  general, 
blindness  in  no  way  affects  the  mind  in  its  development,  and  the  con- 
genitally blind  possess  as  good  a  mentality  as  their  fellows.  It  is  a 
matter  of  experience  also  that  subjects  with  healthy  minds,  who  have 
lost  their  sight  after  their  mental  powers  have  been  developed,  suffer 
no  impairment  of  reason,  apart  from  the  general  depression  consequent 
upon  the  realization  of  how  much  they  have  been  deprived,  normal 
cerebration  being  continued  without  interruption.  In  individuals,  how- 
ever, who  are  intellectually  weak,  it  can  be  readily  understood  how  the 
loss  of  so  important  a  sense  as  sight  might  be  of  serious  moment,  even 
sufficient,  perhaps,  to  break  the  links  in  the  fragile  chain  of  cerebration 
and  cause  insanity.  Notwithstanding  this,  instances  of  serious  mental 
lesions  from  visual  defects  are  very  rare  and  blindness  as  a  causal  factor 
in  the  production  of  insanity  is  mentioned  but  seldom  in  alienist  litera- 
ture. It  is  probable,  however,  that  were  a  more  careful  etiological  study 
made  of  the  blind  insane  from  an  ophthalmological  stand-point,  that  a 
connection  between  the  mental  and  ocular  conditions  would  be  traced 
more  often.  The  cases  which  have  been  reported,  however,  are  espe- 
cially significant,  for  they  demonstrate  that  not  only  can  insanity  be 
traced  to  loss  of  sight,  but  that  the  relief  of  the  blindness  by  operation 
can  restore  the  mental  condition. 

8... 


MEXTAL  EFFECT  OF  EYE  OPERATIOXS  853 


RELIEF  OF  INSANITY  BY  EYE  OPERATIONS. 

The  literature  contains  but  few  cases  where  it  could  be  definitely 
ascertained  that  an  improvement  in  the  mental  state  was  noted  after 
such  operations,  for,  although  cataracts  have  been  Removed  from  the 
insane  many  times,  either  because  the  subjects  who  were  operated  upon 
had  become  chronically  insane  by  reason  of  the  long  continuance  of  the 
blindness,  or  because  their  blindness  was  of  accidental  occurrence  and 
in  no  way  responsible  for  their  mental  deficiency,  the  mind  was  un- 
changed by  the  operation  in  nearly  all  instances.  A  few  cases,  how- 
ever, where  a  connection  could  be  traced  between  loss  of  sight  and  loss  of 
mentality  have  been  reported,  in  which  restoration  of  sight  by  operation 
was  followed  by  complete  recovery  of  mind.  Thus,  the  writer  would 
refer  to  two  cases  which  he  rej)orted  in  1900,  before  the  County  Medical 
Society  of  Philadelphia.  The  first  case,  which  was  seen  in  the  Avards 
of  the  State  Hospital  for  the  Insane  at  Xorristown,  a  male,  45  years 
old,  had  been  practically  blind  for  3  years  with  cataracts.  This  patient 
had  inherited  a  disposition  towards  insanity  from  his  father,  who  had 
had  acute  mania,  but  his  mind  had  been  apparently  unaffected  until  his 
vision  failed,  when  he  became  greatly  depressed  and  irritable  and  de- 
veloped homicidal  and  suicidal  tendencies.  Rest  and  tonic  treatment 
were  prescribed  without  avail,  the  mental  condition  showing  no  improve- 
ment. The  connection  between  the  loss  of  sight  and  the  insanity  being 
so  obvious,  the  cataract  was  removed  by  operation  and  practically  nor- 
mal vision  regained.  The  improvement  in  the  mental  condition  was  im- 
mediate and  convalescence  was  so  rapid  that  in  a  month  the  patient  was 
discharged  from  the  hospital  apparently  well.  Shortly  after,  a  clerical 
position  was  obtained,  in  which  he  has  continued  without  relapse  from 
sanity  during  the  past  six  years. 

The  second  case  occurred  in  a  woman,  60  years  of  age,  who  had  de- 
veloped melancholia,  with  failure  of  sight  consequent  upon  cataracts 
forming  upon  both  eyes,  five  years  previous  to  the  writer's  first  observa- 
tion of  her.  The  mental  condition  was  extremely  bad :  the  patient  being 
excessively  violent  at  times  and  never  entirely  rational.  Vision  was 
restored  by  operation  and  was  followed  by  a  rapid  and  permanent  return 
of  a  normal  state  of  mind.  Cases  analogous  to  these  have  been  reported 
by  Bouissin  and  Meiser.  That  of  the  former  author  referred  to  a  young 
man  who  had  lost  his  mind  with  the  development  of  cataract  and  re- 
covered it  when  the  cataract  was  removed.  In  Meiser's  ^  patient,  a 
woman,  68  years  old,  also  the  subject  of  cataracts,  hallucinations  similar 


854  THE    EYE    AND    NERVOUS    SYSTEM 

to  those  observed  after  operations  upon  the  eye  developed  with  the 
failure  of  her  vision  but  disappeared  after  the  removal  of  the  cataract. 

In  addition  to  these  cases  where  the  mental  disturbance  was  made  to 
disappear  by  the  restoration  of  sight,  cases  have  been  reported  in  which 
the  removal  of  peripheral  irritation  by  enucleation  of  the  globe  was  fol- 
lowed by  the  relief  of  severe  mental  symptoms.  Thus,  Moulton  reports 
a  case  in  which  progressive  melancholia,  which  had  developed  secondary 
to  an  irido-cyclitis  of  long  standing,  was  immediately  relieved  by  re- 
moval of  the  eyeball.  Of  a  similar  nature  was  a  case  recently  reported 
by  IIansell.2  This  patient,  a  female  25  years  of  age,  had  been  born 
blind,  but  during  her  youth  had  given  evidences  of  average  intelligence 
and  had  some  school  training  in  a  blind  asylum,  where  she  had  later 
become  a  teacher.  After  some  years  she  developed  pain  in  the  head 
and  delusional  insanity,  the  delusion  taking  the  form  of  the  envelop- 
ment of  all  objects  in  a  bright  red  glare.  The  eyes  were  injected, 
tender  to  the  touch,  sightless  and  atrophic,  and  as  it  was  thought  that 
they  might  be  the  possible  source  of  her  delusion,  both  were  enucleated. 
The  patient  gradually  became  more  quiet  and  in  a  few  days  after  the 
operation  her  mind  regained  its  former  clearness,  and  all  delusions 
vanished,  so  that  she  was  enabled  to  resume  her  occupation  of  teaching. 
The  relief  of  similar  symptoms  by  operation  has  been  noted  by  De 
Schweinitz^  and  Risley.* 

From  these  cases  it  would  appear  that  grave  mental  disturbances  may 
follow  affections  of  the  visual  apparatus  and  that  relief  may  be  ob- 
tained by  the  removal  of  the  cause,  and  although  the  writer  has  been  told 
by  alienists  that  many  forms  of  insanity  may  be  temporarily  improved 
if  not  entirely  relieved  by  any  form  of  operation,  or  even  by  shock,  or 
by  any  force  which  strongly  impresses  the  mind,  he  believes  that  the 
connection  between  cause  and  effect  in  the  cases  which  were  reported  by 
him  was  too  close  to  deny  the  direct  influence  of  the  restoration  of  sight 
upon  the  mind.  He  would  not  deduce  from  this  that  the  mind  can  be 
restored  to  all  blind  insane  by  similar  operations,  but  he  is  convinced 
that  every  effort  should  be  made  to  give  useful  vision  to  all  such  cases, 
for  although  in  many  instances  operations  may  be  unavailing,  yet  the 
return  of  even  a  few  such  subjects  to  society  will  amply  compensate  for 
many  failures. 

MENTAL  DISTURBANCES  AFTER  EYE  OPERATIONS. 

This  subject  refers  to  the  second  category  contained  in  the  opening 
paragraph  and  relates  to  the  impressions  made  upon  the  mind  by  the 
operation  itself,  independent  of  any  influence  which  might  be  exerted 


MENTAL  EFFECT  OF  EYE  OPEEATIONS  855 

upon  it  bj  the  restoration  of  sight.  Such  impressions  develop  as  a 
consequence  of  the  shock  to  which  the  patient  is  subjected  at  the  time  of 
the  operation  and  as  a  result  of  conditions  which  arise  afterwards,  and 
are  of  the  nature  described  by  neurologists  as  traumatic  neuroses. 

In  contradistinction  to  the  phase  of  the  subject  which  has  just  been 
considered,  where  it  was  shown  that  restoration  of  sight  rarely  exercises 
any  decided  action  upon  the  mind,  it  not  rarely  happens,  after  opera- 
tions upon  the  eye,  that  those  who  are  operated  upon  develop  more  or 
less  disturbance  of  mentality.  This  derangement,  as  a  rule,  takes  the 
form  of  a  delirium  which  appears  usually  upon  the  evening  of  the  third 
day  after  the  operation  and  continues  a  few  days  to  a  week,  according 
as  the  surgeon  is  prompt  in  combating  it,  and  fortunate  in  his  choice 
of  remedies.  Although  frequently  retarding  the  healing  process  by  the 
irritation  which  is  provoked  in  the  eye  at  the  height  of  the  delirium  by 
interference  with  the  dressings,  etc.,  the  ultimate  success  of  the  opera- 
tion is  not  as  a  rule  marred  by  its  occurrence.  It  is  rarely  seen  in  the 
young,  but  it  is  met  with  in  those  who  are  well  advanced  in  years,  and, 
according  to  the  majority  of  observers,  especially  in  those  who  are  pre- 
disposed by  heredity,  alcoholism,  or  some  other  cause,  to  mental 
aberration.. 

During  the  performance  of  the  operation  and  for  the  first  few  days 
after  it,  the  behavior  of  the  patients  is  usually  all  that  can  be  desired. 
At  the  end  of  the  second  or  third  day,  however,  towards  evening,  the 
patients  become  restless  and  wish  to  rise  out  of  bed,  and  if  unrestrained 
would  tear  the  dressings  from  the  eyes.  Rapid  and  violent  delirium 
may  supervene.  Such  patients  usually  fancy  the  room  on  fire  in  which 
they  are  confined,  and  would  make  their  escape  at  all  hazards.  I^ot 
rarely  they  imagine  they  are  subjects  of  persecution ;  that  they  are  being 
pursued  or  unjustly  confined,  and  they  demand  a  knife  with  which  to 
wreak  vengeance  upon  their  oppressors.  Although  the  symptoms 
usually  are  more  apparent  at  night,  the  patient  rarely  recovers  his  full 
senses  during  the  day.  Hallucinations  are  not  always  present,  but  there 
is  usually  some  fixed  idea  which  predominates.  Pain  in  the  eye  is  but 
rarely  complained  of,  and  rise  of  temperature  is  accidental  and  not 
constant.  After  a  few  days  the  delirium  subsides  and  the  mental  facul- 
ties are  slowly  regained  without  leaving  any  permanent  mental  aberrant 
processes. 

Until  Dupuytren,^  in  1819,  differentiated  a  purely  nervous  type  in 
non-alcoholics,  all  forms  of  delirium  occurring  after  operations  were 
thought  to  be  due  to  alcohol  and  were  considered  to  be  instances  of 
delirium  tremens.    This  distinguished  surgeon  saw  this  nervous  form  of 


856  THE    EYE    AND    NERVOUS    SYSTEM 

delirium  develop  after  a  luiniber  of  diverse  general  operations,  though 
it  is  of  interest  to  note  that  in  one  instance  it  was  recorded  that  the 
mental  symptoms  appeared  after  the  couching  of  a  cataract.  In  1863, 
Sichel  **  contributed  the  first  article  which  was  directed  exclusively  to 
the  consideration  of  mental  disturbances  after  operations  upon  the  eye 
and  reported  eight  cases  of  delirium  following  the  removal  of  cataract  in 
old  people;  later  in  the  same  year  monographs  appeared  upon  the  same 
subject  by  Lanne,"  Magne,*  and  Borelli.®  In  his  classic  article  upon 
oj^erations,  in  the  first  edition  of  tiie  Graefe-Saeniisch  Ilandbuch,  Arlt 
refers  briefly  to  the  subject,  stating  that  in  addition  to  delirium  tremens, 
a  peculiar  delirium  appears  during  the  first  few  days  after  the  operation, 
chiefly  in  old  persons  of  an  anxious  temperament,  who  are  broken  down 
in  health. 

The  next  observation  of  importance  was  by  Schmidt-Rimpler,^"  in 
1878,  who  asserted  that  mental  disturbance  could  originate  not  only 
after  operations  upon  the  eye,  but  could  develop  even  without  an  opera- 
tion in  individuals  whose  eyes  had  been  bandaged  or  who  had  been  con- 
fined in  dark  rooms.  In  1890  the  entire  subject  was  discussed  very 
thoroughly  before  several  French  ophthalmological  societies,  without 
any  definite  conclusion  being  arrived  at,  regarding  the  prophylaxis  or 
etiology  of  the  disturbances,  and  though  a  number  of  communications 
have  appeared  since  that  time,  those  phases  of  the  subject  still  remain 
obscure. 

From  a  comparison  of  the  mental  disturbances  which  occur  after 
operations  upon  the  eye  with  those  which  follow  other  operations  upon 
the  body,  it  appears  that  both  processes  are  similar,  and  just  as  the 
delirium  and  mental  disturbances  arising  after  operations  elsewhere 
may  asume  a  variety  of  forms,  so  the  psychoses  after  operations  upon 
the  eye  may  vary  in  their  manifestations,  and  while  the  picture  of  the 
delirium  which  has  been  given  above  describes  the  usual  symptoma- 
tology, the  mental  disturbance  may  assume  other  forms,  being  influenced 
largely  by  the  individuality  of  the  subject  affected.  Thus,  the  time  of 
the  appearance  of  the  symptoms  may  vary,  and  instead  of  arising  within 
the  first  few  days,  the  delirium  may  not  manifest  itself  until  the  6th 
or  Tth  day  and  may  be  retarded  even  for  two  or  three  weeks.  In  like 
manner  the  convalescence  may  be  protracted,  and  the  symptoms  instead 
of  disappearing  within  a  few  days  may  persist  for  weeks  or  even  months. 
While  a  mild  delirium  is  the  rule,  the  nervous  disturbance  may  take  the 
form  of  an  active  mania  and  though  relapses  are  very  rare  and  the  prog- 
nosis is  good  in  most  cases,  the  patient  regaining  his  normal  mind  at 
the  end  of  a  week,  in  other  instances  the  delirium  passes  into  the  deepest 


MEXTAL  EFFECT  OF  EYE  OPEEATIOXS  857 

melaucholia,  and  suicide  may  result.  Ou  account  of  these  variations 
in  the  symptoms  and  course  of  the  disease,  Frankl-Hochwart  ^^  has 
divided  these  psychoses  into  four  groups:  1.  Hallucinatory  mental  de- 
rangement in  non-alcoholics.  In  this  class  the  commencement  of  the 
affection  is  usually  on  the  first  day  after  the  operation  and  occurs  in 
individuals  ranging  from  30  to  90  years  of  age.  2.  Simple  confusion 
of  ideas  in  senile  individuals.  Hallucinations  are  not  present  in  this 
class,  and  dementia  not  rarely  follows.  3.  Delirium  in  alcoholism.  In 
this  class  the  delirium  appears  earlier  and  subsides  more  rapidly,  -i. 
Confusion  of  ideas  due  to  inanition,  occurring  in  extremely  marasraatic 
individuals  and  followed  by  death. 

Knapp^^  has  made  a  still  more  practical  division  of  the  cases  into 
three  clinical  varieties,  (a)  Light  cases,  exhibiting  but  slight  mental  dis- 
turbance, and  occurring  in  eccentric  and  odd  characters,  (h)  those  ex- 
hibiting violent  mania,  (c)  persons  of  a  mild  melancholic  disposition 
with  temporary  aggravations  and  suicidal  tendencies. 

In  attempting  to  classify  cases  of  mental  disturbance  after  opera- 
tions upon  the  eye,  Schnabel  ^^  holds  that  the  delirium  is  analogous  to 
the  acute  delirium  which  is  seen  in  the  aged,  though  its  presence  in  so 
many  young  subjects  suggests  important  differences.  Mendel  ^*  re- 
gards the  disturbance  as  a  form  of  delirium  hallucinatorium,  including 
under  this  heading,  in  addition  to  the  mental  symptoms  after  operations 
upon  the  eye,  other  traumatic  psychoses  as  well  as  senile  delirium.  He 
insists  upon  the  necessity  of  strongly  differentiating  other  types  of  de- 
lirium from  senile  debility,  for  he  believes  that  organic  changes  are 
rarely  absent  in  senile  debility  and  that  the  prognosis  is  consequently 
bad,  whereas  the  prognosis  in  other  forms,  which  he  considers  as  being 
purely  functional  psychoses,  he  regards  as  being  the  most  favorable 
among  mental  disorders. 

Lowy  ^^  holds  much  the  same  view  as  Mendel,  believing  that  the 
mental  disturbance  is  a  typical  psychosis  with  a  characteristic  set  of 
symptoms,  and  regards  senile  delirium  as  a  stage  of  senile  dementia. 
Schmidt-Rimpler  ^^  classifies  the  delirium  among  the  paranoia,  with  the 
understanding  that  primary  insane  conceptions  and  hallucinations 
should  be  regarded  as  the  chief  symptom  of  paranoia. 

As  might  be  expected  by  reason  of  the  greater  number  of  times  the 
operation  for  cataract  is  performed  upon  the  eyes  of  the  aged,  a  class  of 
individuals  who  are  especially  liable  to  be  affected  by  mental  symptoms 
after  operations  or  shock  of  any  kind,  the  number  of  times  this  com- 
plication is  met  \vith  after  the  removal  of  the  lens,  is  far  greater  than 
after  any  other  operation  upon  the  eye.     There  are  no  statistics  in  the 


858  THE    EYE    AXD    NERVOUS    SYSTEM 

literature  regarding  the  frequency  of  the  disease,  witli  the  exception  of 
those  drawn  from  a  summary  of  24  cases,  collected  by  the  writer.  Of 
these  24  cases  the  delirium  appeared  11  times  after  simple  extraction; 
8  times  after  the  combined  operation;  3  times  after  iridectomy  for 
glaucoma,  and  twice  in  traumatic  cases  where  no  operation  had  been 
performed  upon  the  eyes  or  adnexa.  Sixteen  of  these  cases  were  col- 
lected from  the  Wills  Eye  Hospital  records  and  extended  over  a  period 
in  which  770  cataract  extractions  had  been  performed  in  that  institution, 
making  a  percentage  of  '2-rj  per  cent.  It  was  felt  by  the  writer,*'  how- 
ever, that  these  figures  might  be  misleading  and  mental  disturbances 
might  be  much  more  common,  as  the  custom  which  prevails  in  that  hos- 
pital of  administering  hypnotics  at  the  first  symptom  of  mental  unrest 
might  have  prevented  the  development  of  what  would  have  been  a  true 
post-operation  psychosis.  Of  12  cases  observed  by  Kipp,  5  followed  cat- 
aract operation,  1  an  iridectomy  for  glaucoma,  3  traumatic  injuries  of 
the  eyeball,  and  3  corneal  ulceration.  Schnabel  *^  saw  12  cases  of  delir- 
ium among  183  cases  of  senile  cataract  in  his  own  clinic  at  Innsbruck, 
though  in  Jager's  clinic  in  Vienna  delirium  occurred  but  11  times 
among  1500  cases  of  cataract.  All  of  Schnabel's  cases  occurred  in  old 
subjects  and  several  times  after  iridectomy  without  removal  of  the  lens. 
The  psychoses  began  20  hours  to  6  days  after  the  operation  and  lasted 
from  1  to  4  days.  In  half  of  the  cases  the  mental  symptoms  occurred  at 
night  and  in  the  other  half  during  the  day.  Convalescence  was  rapid 
and  without  complications.  Lowy  explains  the  unusual  frequency  with 
which  Schnabel  saw  cases  in  Innsbruck  and  its  rarity  in  Vienna,  by 
the  fact  that  the  inmates  of  the  hospital  in  Innsbruck  were,  for  the 
most  part,  inhabitants  of  the  mountains  and  unaccustomed  to  restraint 
and  confinement  and  more  liable  to  be  affected  by  such  conditions  than 
the  city-bred  Viennese.  He  says  that  the  same  explanation  applies  to 
Christen  of  Pavia's  cases  also,  the  frequency  with  which  delirium 
occurred  in  his  clinic  in  that  city  being  accounted  for  by  the  unre- 
strained and  rather  wild  character  of  the  inhabitants  of  that  neighbor- 
hood. 

As  a  rule,  recovery  is  more  rapid,  the  symptoms  less  violent  and  the 
prognosis  generally  better  among  the  aged  than  the  young.  Webster*® 
saw  a  fatal  case,  as  the  result  of  meningitis,  secondary  to  panophthal- 
mitis, which  resulted  from  a  patient  with  delirium  tearing  off  the 
bandage.  Berry  ^^  recorded  epileptic  attacks  following  an  intraocular 
hemorrhage  complicating  cataract  extraction. 

Etiology. — As  would  be  expected  from  the  nature  of  the  affec- 
tion and  our  ignorance  regarding  the  pathogenesis  of  psychoses  in  gen- 


MEXTAL  EFFECT  OF  EYE  OPEEATIOXS  859 

eral,  many  theories  have  been  advanced  to  explain  the  origin  of  the 
affection.  Sichel  thought  the  delirium  was  due  to  a  change  in  the  dis- 
position of  the  individual,  which  resembled  nostalgia.  A  number  of 
authors,  however,  assume  a  special  tendency  towards  mental  derange- 
ment in  all  who  become  delirious  following  operations  upon  the  eye. 
Among  these  may  be  mentioned  Arlt,^^  Schmidt-Rimpler,^^  Knies,^^ 
Warloment,^-'  Ilirschberg,  ^^  and  Valude.^^  These  authors  believe  that 
mental  symptoms  arise  in  only  very  weak  and  nervous  people,  but  fail 
to  mention  further  characteristics  of  this  predisposition.  Lopez  thinks 
that  the  delirium  occurs  only  in  those  who  are  already  actually  mentally 
alienated,  although  usually  in  a  latent  form,  the  mental  weakness  having 
passed  unperceived  by  the  physician. 

Among  occasional  exciting  causes  may  be  mentioned  Parinaud's^" 
explanation  of  extreme  preoccupation  of  the  patient  for  several  days 
preceding  the  operation  itself,  and  the  withdrawal  of  all  external  im- 
pressions during  the  first  few  days  after  the  operation,  resulting  from 
the  absolute  rest  in  bed,  and  the  darkness  and  seclusion  enforced  by 
the  closed  eyes.  Schmidt-Rimpler  ^^  is  strongly  of  the  impression  that 
one  of  the  chief  factors  in  the  production  of  the  delirium  is  the  ban- 
daging of  both  eyes,  and  in  support  of  this  theory  cites  two  cases  in 
which  no  operation  was  performed,  but  who  developed  delirium,  as  he 
thought,  simply  by  reason  of  their  being  confined  in  a  darkened  room 
during  the  treatment  for  their  ocular  affection.  One  of  these  cases 
was  that  of  a  young  man,  19  years  old,  who  was  suffering  from  irido- 
chorioiditis ;  the  other  that  of  a  man,  59  years  old,  who  had  plastic  iritis. 
This  theory  is  supported  by  Sichel,^®  Swanzy,^**  Yalude,^^  Borelli,^^ 
and  Armaignac ;  ^^  the  last  three  authors  giving  the  notes  of  cases  where 
the  delirium  disappeared  with  the  removal  of  the  bandage. 

Another  set  of  authors,  including  Galezowski,^^  Salvator,  Angela 
Ledda,  and  Lemkiewicz,  attributes  the  delirium  to  the  action  of  atropine. 
Grandclement  ^^  subscribes  to  this  theory  also,  as  the  mental  condition 
of  a  case  observed  by  him  was  made  worse  after  each  instillation  of  this 
drug. 

As  just  mentioned,  Schnabel  was  of  the  opinion  that  the  psychosis 
was  merely  a  form  of  senile  delirium  and  he  explained  the  transitory 
nature  of  the  disturbance  in  mentality  as  well  as  its  sudden  appearance, 
to  circulatory  changes  in  an  atrophic  brain.  He  thought  that  the  hyper- 
semia  of  the  brain  is  favored  by  the  prolonged  rest  upon  tbe  back,  with 
the  stillness  and  darkness,  to  which  the  patients  are  subjected  while  in 
a  more  or  less  excited  condition.  He  does  not  believe  Schmidt-Rimpler's 
theory  of  the  exclusion  of  light,  as  the  sole  cause,  as  he  had  observed 


860  THE    EYE    AND    NERVOUS    SYSTEM 

the  condition  to  arise  after  the  bandage  had  been  removed,  and  calls 
attention  to  the  rarity  with  wliicli  delirium  occurs  in  tlie  large  number 
of  young  persons  who  are  treated  in  darkened  rooms  and  with  bandaged 
eyes.  Kraft-Ebbing  '*  is  of  the  opinion  that  fear  is  the  causal  factor, 
and  tliinks  that  the  symptoms  rarely  develop  in  those  who  have  normal 
minds,  but  rather  in  those  who  are  abnormally  excitable,  in  imbeciles, 
or  in  those  weakened  by  senility,  alcohol,  or  sickness.  Frankl-IIochwart 
called  attention  to  the  connection  between  affections  of  the  nerves  (espe- 
cially neuralgia)  and  psychoses  and  also  to  the  fact  tliat  mere  injury 
of  the  globe,  as  in  the  cases  reported  by  Griesinger,^^  Arlt,  and  Fiirstner, 
could  occasion  delirium  and  even  deatli,  and  ascribes  the  delirium  to  an 
affection  of  the  sensory  nerves  of  the  eye  conjoined  with  senility,  dark- 
ness, absolute  rest  in  bed  and  the  isolation  from  the  external  world. 

An  additional  theory  is  mentioned  by  Berger,^^  who  says  that  he 
remembers  that  Meynert,  of  Vienna,  ascribed  the  production  of  the 
delirium  to  the  excision  of  part  of  the  iris,  which  is  one  of  the  steps  in 
the  common  operation  for  cataract.  Berger,  however,  remarks  that  this 
theory  is  no  longer  tenable,  since  Parinaud  has  observed  the  delirium 
following  the  extraction  of  cataract  without  iridectomy. 

The  restriction  in  diet  and  lessening  of  the  amount  of  food  imme- 
diately before  and  after  the  operation  has  also  been  advanced  as  "a  cause. 
Dupuytren  was  the  first  advocate  of  this  theory,  as  he  found  tliat  after 
he  gave  his  patients  sufficient  nourishment,  mental  derangements  oc- 
curred mucli  more  rarely.  Parinaud  and  Gillett  de  Grandement  also 
support  it,  for  both  think  that  the  weakness  induced  by  the  restricted 
diet  is  an  important  factor  in  precipitating  the  delirium ;  the  latter 
author  citing  a  remarkable  instance  which  was  evidently  induced  by 
this  cause. 

^^^lile  it  is  evident  that  in  alcoholism  the  excitement  consequent 
upon  the  operation,  conjoined  with  the  depressing  circumstances  which 
are  attendant  upon  the  after  treatment,  may  easily  provoke  a  delirium 
of  persecution,  it  is  not  the  purpose  of  the  writer  to  consider  this  type 
of  cases  in  this  paper.  Tn  tliis  connection,  however,  reference  may  be 
made  to  a  case  reported  by  Lopez,^®  where  the  delirium  occurred  in  an 
alcf)holic,  in  whom  the  eyes  were  not  bandaged  and  where  the  wound 
healed  perfectly.  Lopez  thinks  that  it  is  a  circumstance  worthy  of 
consideration  that  he  has  always  observed  the  delirium  in  the  old  and 
atheromatous,  and  says  this  has  led  him  to  think  that  this  disease  of  the 
vascular  system  plays  an  important  role  in  the  production  of  cerebral 
troubles,  and  subsequently  of  maniacal  paroxysms.  He  adds  that  it  is 
not  strange  that  the  cause  of  the  delirium  in  question  should  be  charged 


MEXTAL  EFFECT  OF  EYE  OPERATIONS  861 

ty  some  to  alcoholism,  as  this  is  a  frequent  factor  in  the  production  of 
atheromatous  degeneration. 

Martin  ^^  also,  who  has  contributed  a  recent  and  comprehensive 
article  on  the  subject,  holds  that  the  delirium  is  due  to  inanition,  but 
thinks  that  this  is  induced  chiefly  by  the  withdrawal  of  alcohol.  He 
does  not  believe  that  the  mental  derangement  in  this  class  of  cases  is 
an  alcoholic  delirium  in  the  sense  that  the  subjects  are  ever  intoxicated 
by  alcohol,  but  that  it  is  delirium  occasioned  by  the  privation  of  this 
agent  in  individuals  who  are  accustomed  to  it  in  moderate  amounts ;  for 
he  thinks  that  the  brain,  especially  in  the  old,  which  has  been  habituated 
to  act  normally  under  the  influence  of  alcoholic  stimulation,  becomes 
deranged  if  this  agent  is  withdrawn. 

Fernandez  ^^  believes  the  abstinence  from  customary  alcoholic 
stimulation  is  the  determining  factor  in  producing  the  delirium.  He 
has  observed  the  conditions  six  times  in  a  very  large  experience,  and 
only  after  cataract  operations. 

In  a  paper  which  was  presented  at  the  Congress  of  Alienists  of  La 
Rochelle,  in  1893,  Regis  and  Chevalier-Lavaure  considered  the  de- 
lirium which  is  observed  after  operations  in  general  to  be  due  to  auto- 
infection  as  a  result  of  uraemia  and  toxins  originating  in  the  intestines, 
and  thought  that  the  mental  disturbance  in  these  cases  was  analogous  to 
those  occurring  in  typhoid  fever,  influenza,  and  erysipelas.  Fromaget 
subscribed  to  this  theory  as  being  operative  in  most  cases  of  delirium 
after  operations  upon  the  eyes  and  was  of  the  opinion  that  the  conditions 
favoring  autoinfection  were  found  in  the  disturbances  of  the  liver, 
kidney,  and  heart,  which  are  common  to  the  aged.  In  support  of  his 
views,  he  gave  the  notes  of  a  man  aged  92  years,  in  whom  an  anuria 
prevailed  two  days  after  the  removal  of  the  lens,  the  flow  of  urine  being 
increased  after  some  difficulty  by  injections  of  caffein.  This  theory  of 
autointoxication  has  also  been  subscribed  to  by  Finlay  ^^  from  con- 
' elusions  based  upon  the  study  of  a  case  where  there  were  signs  of 
uraemia. 

Kipp  ^^  in  a  recent  article,  in  which  he  reported  12  cases  of  delirium 
after  operation  and  injury  of  the  eye,  concluded  from  a  study  of  these 
cases  that  the  "  psychoses  were  the  result  of  a  change  in  the  patient's 
environment  and  to  an  increasing  longing  to  get  away  from  the  new 
surroundings."  He  refers  to  the  condition  as  a  homesickness,  or  nostal- 
gia, which  may  end  in  a  form  of  melancholia  Avith  homicidal  and 
suicidal  propensities. 

Such  are  the  theories  Avhich  have  been  advanced  to  account*  for  this 
strange  form  of  mental  disturbance,  and  while  in  the  present  state  of 


862  THE    EYE    AND    XERVOUS    SYSTEM 

uncertainty  regarding  the  nature  of  psychological  processes  in  general, 
it  may  be  impossible  to  determine  the  precise  etiological  factor  which 
occasions  the  delirium,  there  are  such  decided  objections  which  can  be 
offered  to  some  of  them  that  they  may  be  eliminated  from  the  list. 
Thus,  the  assertion  that  the  mental  aberration  is  due  to  a  predisposition 
to  mental  unsoundness  can  be  disproved  by  the  absence  of  symptoms  in- 
dicating insanity  in  the  previous  history  of  most  of  the  patients,  and  by 
the  transient  character  of  the  delirium.  In  the  few  cases  in  which  the 
mind  does  not  recover  its  normal  condition  after  one  or  two  weeks,  but 
lapses  into  mania  and  melancholia,  it  is  fair  to  suppose  that  some  such 
conditions  may  have  existed  previous  to  the  operation. 

Martin  could  satisfy  himself  of  a  state  of  mental  weakness  prior  to 
the  operation  in  but  two  cases  mentioned  in  the  literature, — i.e.,  that  of 
Warloment,  where  the  statement  is  made  that  his  case  seemed  mentally 
"  innocent,"  but  that  he  had  never  given  another  sign  of  delirium  or 
insanity;  and  that  of  Hirschberg's,  where  the  patient  was  said  to  have 
some  mental  unsoundness.  He  says  that  in  none  of  the  20  or  more 
other  cases  mentioned  is  there  any  reason  to  suspect  a  prior  predisposi- 
tion towards  insanity.  Lopez's  claims  of  a  mental  predisposition  should 
not  be  regarded,  as  two  of  his  cases  were  probably  quite  insane  before 
the  operation,  and  should  not  really  have  been  described  as  instances  of 
post-operative  psychoses ;  one  of  the  cases  occurring  in  a  family  of  eccen- 
tric members  and  the  patient  remaining  insane  for  two  months  after  the 
operation,  while  the  second  case  was  melancholic. 

Sweet's  ^*  case  belongs  to  the  same  category.  This  author  cites  an 
instance  of  delirium  of  persecution  following  a  cataract  extraction  which 
was  evidently  excited  by  the  fear  of  going  blind.  The  patient  was  very 
nervous,  and  although  there  was  no  former  history  of  insanity,  there 
was  probably  a  strong  disposition  toward  mental  weakness.  The  patient 
died  two  months  after  the  operation. 

Of  the  24  cases  reported  by  the  writer,  there  were  but  3  of  question- 
able healthy  mentality. 

In  opposition  to  the  bandage  theory  is  the  fact  that  even  after  it  is 
removed  from  the  unoperated  eye,  there  is  usually  no  improvement  in 
the  mental  condition.  Thus,  neither  in  Warloment's  and  Hirschberg's 
cases  nor  in  any  of  those  cited  by  the  writer  was  there  any  improvement 
after  the  bandages  were  removed.  Gorecki,  also,  and  Calderon  *'  saw 
delirium  after  cataract  operation  in  non-alcoholics,  when  the  eyes  were 
not  bandaged,  and  the  same  was  true  in  a  case  observed  by  the  author. 

This*  would  seem  to  be  in  exact  contradiction  to  a  case  reported  by 
Valude,  for,  in  this  instance,  as  soon  as  the  bandage  was  removed  from 


MEis^TAL  EFFECT  OF  EYE  OPEEATIOXS  863 

a  75-year-old  patient,  who  had  developed  delirium  as  a  result  of  double 
iridectomy  for  glaucoma,  the  delirium  ceased ;  and  likewise  a  case  of 
Borelli,  where  amelioration  was  almost  immediate  when  coquilles  were 
substituted  for  the  bandage.  Martin,  however,  disposes  of  both  of  these 
objections,  or  rather  exceptions,  as  follows :  Valude's  case  was  a  simple 
coincidence, — i.e.,  the  bandage  being  removed  about  the  time  that  the 
other  remedies  which  had  been  employed  became  efficacious,  while 
Borelli's  case  was  not  one  of  true  delirium,  but  simply  one  of  a  fixed 
idea  which  existed  before  the  operation. 

It  can  also  be  argued  against  the  bandage  and  exclusion  of  light 
theory,  that  if  the  delirium  be  due  to  this  cause,  insanity  should  be  much 
more  common  among  the  blind  than  is  actually  the  case. 

The  statistics  of  the  summary,  compiled  by  the  writer  in  his  series 
of  cases,  show  conclusively  that  atropine,  also,  could  not  be  considered 
as  a  causal  factor  in  the  production  of  the  delirium,  Cocain  can  also 
be  eliminated.  Indeed  it  would  seem  to  be  proven  beyond  question 
that  the  condition  of  the  eye  itself  has  nothing  to  do  with  the  develop- 
ment of  the  mental  alienation. 

Sichel's  and  Kipp's  theory  that  the  delirium  was  due  to  homesick- 
ness cannot  be  true  in  all  cases,  for  the  delirium  has  been  known  to 
originate  in  many  cases  when  the  patient  was  operated  upon  in  his  own 
home.  Many  of  Kipp's  cases  seemed  to  be  alcoholic,  and  the  improve- 
ment noted  in  their  condition  after  they  left  the  hospital  may  have  been 
due  to  the  stimulation  of  alcohol  which  they  then  had  an  opportunity  of 
acquiring ;  or,  tlue  restraint  of  the  hospital  being  removed,  to  the  stimu- 
lus given  their  mind  by  the  necessity  of  self-direction  and  responsibility. 

But  three  theories  remain — that  of  excessive  preoccupation  upon  the 
part  of  the  patient  prior  to  the  operation,  that  of  the  withdrawal  of 
food,  and  that  of  autointoxication.  Of  these,  the  latter  two  may  be 
readily  disposed  of,  at  least  in  so  far  as  the  cases  which  came  under  the 
observation  of  the  writer  are  concerned,  for  he  believes  that  there  can 
be  no  question  of  starvation  in  any  of  the  cases  which  he  reported,  as  it 
is  his  custom  to  make  no  change  in  the  diet  until  24  hours  before 
operating.  At  this  time  the  first  preparations  are  made  for  the  opera- 
tion ;  the  patient  is  bathed  and  the  eye  which  is  to  be  operated  upon  is 
protected  by  an  antiseptic  dressing  after  thorough  flushing  with  a 
boracic-acid  lotion.  A  light  supper  is  then  administered,  which  is  fol- 
lowed by  a  laxative  pill  at  bedtime.  The  following  morning  the  patient 
partakes  of  a  simple  breakfast,  and  as  the  usual  hour  of  operation  is  in 
the  afternoon,  a  glass  of  milk  and  a  cracker  are  given  at  luncheon. 
After  the  operation,  milk  is  again  repeated  at  frequent  intervals.     The 


8G4  THE    EYE    AXD    NERVOUS    SYSTEM 

diet  of  the  second  24  hours  also  is  largely  a  milk  one,  though  broths  or 
beef-tea  are  given  twice  to  prevent  extreme  constipation.  On  the  third 
day  the  patient  is  usually  allowed  to  get  out  of  bed  and  is  placed  on  a 
soft  diet,  and  if  the  bowels  have  not  been  opened,  a  laxative  pill  is 
administered.  On  the  fourth  day  liberal  and  supportive  meals  are 
prescribed. 

Similarly,  he  does  not  think  that  the  recent  theory  of  autointoxica- 
tion which  has  been  advanced  by  Fromaget  could  have  operated  in  any 
of  the  cases  whicli  he  has  cited,  for  care  is  always  exercised  to  avoid 
any  such  complications.  Thus  it  is  a  rule  that  the  urine  of  every 
cataract  patient  shall  be  examined  the  morning  of  the  day  of  the  opera- 
tion, and  care  is  exercised  to  avoid  retention  after  operation.  The 
bowels,  also,  if  nature  has  not  already  accomplished  this,  are  invariably 
opened  on  the  third  day  by  a  cathartic. 

After  a  critical  study  of  tlie  entire  subject  the  writer  has  finally 
arrived  at  the  opinion  that  while  any  of  the  above  factors,  or  others, 
such  as  the  constraint  of  the  supine  position  and  the  unusual  stillness 
of  the  surroundings,  may  operate  in  certain  cases  as  etiological  factors, 
the  chief  cause  of  the  delirium  after  operation  upon  the  eyes  is  largely 
psychic,  and  agrees  with  Parinaud  that  it  is  due  to  the  preoccupation 
upon  the  part  of  the  patients  prior  to  and  after  the  operation.  Wliile 
they  may  appear  calm  and  collected  and  submit  to  the  operation  most 
cheerfully,  if  one  scrutinizes  them  closely,  in  most  instances  he  will 
find  that  this  state  of  quietude  is  assumed  and  that  in  reality  there  is 
great  though  suppressed  excitement. 

The  very  thought  of  sight  after  the  weeks  and  months,  nay,  unfor- 
tunately, in  many  instances,  after  the  years  of  blindness,  is  enough  to 
upset  their  mental  equilibrium,  and  the  desire  to  assist  the  operator 
during  the  operation,  and  not  to  wince  at  pain  or  to  do  an^'thing 
that  miglit  mar  its  success,  must  also  be  a  great  tax  on  their  courage,  and 
calls  forth  all  of  their  power  of  self-control ;  and  then,  following  the 
removal  of  the  lens,  the  glimpse,  though  momentary,  which  is  obtained 
of  the  outside  world,  which  has  been  so  long  hidden  from  their  gaze, 
and  the  realization  that  the  suspense  is  over,  must  undoubtedly  occasion 
more  or  less  psycliical  disturbance. 

Treatment  consists  in  improving  the  state  of  the  cerebral  circula- 
tion by  nitroglycerin  and  strychnine,  and  in  controlling  the  delirium  by 
hypnotics,  in  a  sup])lcniontarv  diet,  and  in  the  administration  of  alcohol 
if  the  patient  has  been  habituated  to  the  use  of  intoxicants.  As  the 
statistics  do  not  show  any  improvement  in  the  symptoms  to  follow  the 
removal  of  the  bandage  from  the  unoperated  eye,  this  step  is  not  to  be 


MEXTAL  EFFECT  OF  EYE  OPERATION'S  865 

advised,  unless  the  wound  caused  by  the  operation  be  thoroughly  healed. 
There  is  also  no  reason  to  discontinue  the  employment  of  atropine. 

Constant  oversight  and  judicious  and  tactful  nursing  are  most  es- 
sential, and  amelioration  in  the  mental  condition  frequently  follows 
the  installation  of  a  proper  person  by  the  bedside. 

BIBLIOGRAPHY. 

^Meiseb:    Psychiat.  u.  Xeurolog.  Blatt.,  No.  1,  1897. 

'Han SELL:    Meeting  of  the  Section  in  Ophthalmology  of  the  College  of  Physicians 

of  Philadelphia,  January,   1900. 
'De  Schweinitz:    Ibid. 

*  RiSLEY :    Ibid. 

*  DuPUYTREN :    Legons  Orales  de  cliniq.  ehirurg.,   1819. 

•SiCHEL:    Union  Med.,  1863,  No.  1,  and  Annal.  d'Ocul.,  149,  p.  154. 

*  Lauxe  :     Du  delire  consecutif  a   I'operation   de  la   cataracte.     Gazette   des   HOp., 

No.  57. 
"Magne:    Du  delire  consecutif  a  I'operation  de  la  cataracte.     Bull.  gen.  de  therap., 

etc.,  Paris,  1863,  xiv.  463;  Du  delir  senile  suivant  a  la  suite  de  1' extraction  de  la 

cataracte.    Ibid.,  1865,  254-256. 
'BoRELLi:    Giorn.  d'ophth.  Ital.,  1863. 
'"  Schmidt-Rimplek:    Archiv  f.  Psych.,  1878-79,  p.  234. 
"  FuAXKL-HocHWABT:    Jahresber.  f.  Psych.,  Bd.  9,  1889. 
"  Knapp  :    Arch,  of  Ophthal.,  vol.  xxxii..  No.  4,  p.  375. 
"ScHNAHEL:    Berieht  des  Natur.  Medic-Vereins,   1883. 
"Mendel:    Berl.  klin.  Wochensch.,   1894,  No.  29. 
"LowY:    Allegem  Zeitschr.  f.  Psych.,  1896,  vol.  lii.,  p.  166. 

"  SCHSIlDT-RlMPLER  :      loc.    Cit. 

"Posey:    Philada.  Med.  Journ.,  Sept.  15,  1900. 

'*  SCHNABEL :      loc.    cit. 

"Webster:    Arch.  f.  Augenheilkunde,  xxi.  p.  191. 

=">  Berry:    Trans.  Ophth.  Soc.  U.  K.,  xi.  p.  152. 

"Arlt:    Graefe-Saemisch  Handbuch,  1st  edition. 

^  Schmidt-Rimpleb:    loc.  cit. 

=°  Knies  :    The  Eye  in  General  Diseases. 

^  Waeloment  :    Annal.  d'oculist.,  t.  liv.  p.  254. 

=°  Hibschbebg  :     Ein    Fall   von   Delerium   nach   Glaucoma-iridectomie.     Centralb.    f. 

Augenh.,  Leipsic,  1885,  ix.  1875. 
*'Valude:    Revue  g6n.  d'ophth.,  1890. 
"Parinaud:    De  la  Soc.  Franc,  d'ophth.,  1890,  p.  126. 
^  Schmidt-Rimpleb:    loc.  cit. 
"  Sichel:    loc.  cit. 

^'Swanzy:    N.  Y.  Med.  Journ.,  xii.  p.  146;    Brit.  Med.  Joum.,  1523,  1890. 
*^  Valude  :    loc.  cit. 
^  BoRELLi :    loc.  cit. 

^Armaignac:    Revue  clinique  d'oculist..  Mars,  1881. 
"Galezowski:     Rec.    d'ophth.,    September,    1879;     Soc.    Franc,    d'ophth.,   January, 

1885. 
"  Grandclement  :    Lyons  Med.  Journ.,  29,  1890. 
''Kraft-Ebbing:    Lehrbuch  f.  Psych.,  1888. 
^^  Griesinger  :    Pathol,  der  psych.  Krankh.,  1871. 
**  Berger  :    Les  Maladies  des  Yeux  dans  leur  rapport  avec  la  pathologie  g6n6ral, 

p.  450. 
55 


866  THE    EYE    AXD    NERVOUS    SYSTEM 

"Lopez:    Med.  Times,  August,  1894;    International  Med.  Mag.,  1894. 

"Martin:     Sui  de  delire  consecutif  a  I'operation  de  la  cataracte.     Jour,  de  Med. 

de  Bordeaux,  1894,  xxiv.  173,  187,  198,  206. 
"Fernandez:    Arch,  de  oft.  Hisp.-Amer.,  October,  1903. 
*^FlNLAY:    Arch,  of  Ophthal.,  January,  1904. 
"Kipp:    Arch,  of  Ophthal.,  1903,  vol.  xxxii..  No.  4,  375. 
"Sweet:     Delirium   and    Death   following   Cataract    Extraction.     Occidental    Med. 

Times,  Sacramento,  1894,  viii.  055. 
**  Calderon  :    Del  delerio  consecutivo  a  la  operacion  de  la  cataracta.     Rev.  clin.  de 

1.  gosp.,  Madrid,  1890,  ii.  241-243. 


CHAPTER  XXL 

THE  SURGICAL    TREATMENT    OF   INTRACRANIAL   LESIONS 
CAUSING  DISTURBANCES  OF  VISION. 

By  CHARLES  H.  FRAZIER,  M.D. 

LESIONS  OF  THE  CRANIAL  SINUSES  AS  AFFECTING 
THE  CONTENTS  OF  THE  ORBIT. 

Anatomical  Considerations. — The  venous  circulation  at  the  base  of 
the  brain  through  the  medium  of  the  cranial  sinuses  corresponds  in  one 
particular  especially  with  the  arterial  circulation  of  the  same  region,  in 
that  there  is  a  free  anastomosis  not  only  between  the  sinuses  on  the  same 
fcide,  but  also  between  the  sinuses  of  the  opposite  side  of  the  brain. 
Therefore,  a  thrombus  forming  in  one  sinus  may  extend  by  continuity 
to  another  on  the  same  or  the  opposite  side.  The  tissues  of  the  orbit 
are  not  disturbed  unless  there  is  a  lesion  of  the  cavernous  sinus ;  there- 
fore, it  is  the  conditions  that  affect  this  sinus  that  concern  us  especially. 

The  cavernous  sinuses  lie  on  either  side  upon  the  body  of  the 
sphenoid  bone,  and  are  in  direct  communication  with  one  another 
through  the  circular  or  intercavernous  sinus.  Into  the  anterior  portion 
of  the  cavernous  sinus  empty  the  ophthalmic  veins,  while  the  posterior 
portion  through  the  medium  of  the  inferior  and  superior  petrosal  sinuses 
is  in  communication  with  the  lateral  or  transverse  sinus.  In  addition 
to  these  the  cavernous  sinus  has  other  important  anastomotic  relations, 
which  bring  the  sinus  indirectly  into  relation  with  extracranial  struc- 
tures. Of  the  most  importance  is  the  so-called  vein  of  Trolard  which, 
arising  in  the  pterygoid  plexus  of  veins,  passes,  with  the  third  divi- 
sion of  the  fifth  nerve,  through  the  foramen  ovale  and  empties  into  the 
inferior  petrosal  sinus.  These  means  of  communication  explain  the 
relation  of  cause  and  effect  between  inflammatory  lesions  in  the  dis- 
tribution of  the  pterygoid  plexus  of  veins  (the  alveoli  of  the  inferior 
maxillary  bone,  the  tonsil,  pharynx,  etc.)  and  thrombosis  of  the  cavern- 
ous sinus.  By  way  of  the  temperosphenoidal  sinus  the  infectious  agents 
from  the  skull  may  be  carried  to  the  cavernous  sinus.  I^ot  to  be  for- 
gotten is  the  anastomosis  between  the  terminal  capillaries  of  the  facial 
and  ophthalmic  veins.  Thus  inflammatory  lesions  in  the  distribution  of 
the  facial  vein  by  a  retrograde  process  may  give  rise  to  sinus  thrombosis. 

867 


868  THE    EYE    AND    NERVOUS    SYSTEM 

Finally  the  intimate  relation  between  the  cavernous  sinus  and  the  third, 
the  fourth,  the  first  division  of  the  fifth,  and  the  sixth  cranial  nerves  is 
not  to  be  forgotten.  The  pressure  of  a  swollen  sinus  or  of  an  extrasinous 
exudate  will  account  for  many  of  the  symptoms  of  thrombosis.  As  the 
sinuses  have  no  valves,  the  blood  may  flow  in  either  direction.  To  the 
absence  of  valves,  as  well  as  to  the  freedom  with  which  sinuses  on  one 
side  anastomose  with  those  on  the  other,  must  be  attributed  the  non- 
appearance of  obstructive  symptoms  in  thrombosis  of  some  of  the  sinuses 
and  the  difficulties,  therefore,  attending  the  diagnosis. 

THROMBOSIS    OF  THE    CAVERNOUS    SINUS. 

Etiology. — The  structure  of  the  sinuses  is  said  to  favor  venous 
stasis.^  The  triangular  shape  of  the  canals  increases  the  frictional 
resistance,  the  walls  constructed  of  fibrous  tissue  are  rigid,  and  the  dense 
bands  of  fibrous  tissue  which  pass  from  one  wall  to  the  other  prevent  the 
collapse  of  the  sinus  and  in  themselves  offer  resistance  to  the  blood 
current.  Furthermore,  the  horizontal  decubitus  naturally  predisposes 
towards  venous  stasis,  so  that,  in  cases  of  long-standing  illness  associated 
with  depression  of  the  cardiac  action,  the  effect  of  this  position  in  co- 
operation with  the  diminished  force  of  the  blood  stream  is  an  important 
etiologic  factor. 

In  general  terms,  thrombosis  may  be  due  to  (1)  systemic  disturb- 
ances, (2)  to  traumatism,  and  (3)  to  a  septic  process.  Systemic  disturb- 
ances are  observed  in  the  very  young  and  the  very  old.  In  the  first  four 
years  of  life  one  observes  the  marasmic  thrombosis  of  tlie  old  writers,  as 
in  patients  suffering  from  grave  forms  of  gastro-enteritis,  cholera  in- 
fantum, bronchopneumonia,  more  rarely  tuberculosis  and  hereditary 
syphilis,  as  well  as  in  convalescents  from  the  acute  infectious  fevers, 
such  as  scarlet  fever,  typhoid  fever,  diphtheria,  and  influenza.  Marasmic 
thrombosis  almost  invariably  involves  the  longitudinal  sinus,  and  the 
treatment  is  purely  medical.  Both  glycosuria  and  albuminuria  are 
believed  to  exert  a  predisposing  influence  in  the  origin  of  thrombosis  in 
people  of  advanced  years,  but  it  is  more  than  likely  that  this  relation, 
with  glycosuria  especially,  may  be  accounted  for  by  the  fact  that  not 
uncommonly  carbuncles  develop  in  diabetic  individuals. 

Thrombosis  as  a  result  of  traumatism  is  uncommon.  The  sinus  has 
been  injured  frequently  in  operations  upon  the  Gasserian  ganglion,  but, 
so  far  as  I  know,  these  injuries  have  not  given  rise  to  any  signs  of  inter- 
ference with  the  circulation  of  the  orbit.     In  the  absence  of  infection, 


» Marfan,  Traits  Maladie  de  I'Enfance,  tome  iv. 


SURGICAL    TREATMENT  869 

gunshot  wounds  are  more  likely  to  determine  an  arteriovenous  aneurism 
than  thrombosis  of  the  sinus.  Fracture  of  the  base  of  the  skull,  blows 
upon  the  head,  and  punctured  wounds  through  tlie  orbit,  occasionally 
cause  thrombosis.  But  four  cases  have  been  reported  in  which  the  sinus 
has  been  injured  directly  without  involving  the  skull. 

Infective  thrombosis  is  by  far  the  most  common.  The  source  of 
infection  may  be  intracranial  or  extracranial,  and  the  thrombus  may  be 
either  primary  or  secondary.  The  intracranial  causes  include  basal 
meningitis,  osteitis  of  the  sphenoid  bone,  and  abscess  together  with 
thrombosis  of  the  lateral  or  other  sinuses.  According  to  Germain,^ 
secondary  tlirombosis — i.e.y  extension  from  other  sinuses — is  probably 
the  most  common  if  we  accept  all  cases.  Out  of  one  hundred  and  forty- 
nine  cases  in  which  an  autopsy  was  made,  in  forty-three  the  disease  orig- 
inated in  the  ear,  causing  thrombosis  of  all  or  most  of  the  basal  sinuses, 
and  it  is  fair  to  assume  that  the  lateral  sinus  was  the  one  first  affected. 

The  extracranial  sources  of  infection  are  as  follows:  (a)  affections 
of  the  orbit,  panophthalmitis,  chalazion,  blepharitis,  and  conjunctivitis ; 
(6)  affections  of  the  nasal  cavities, — e.g.,  tubercular  and  syphilitic 
ulcerations;  (c)  infection  of  maxillary,  ethmoidal,  sphenoidal,  and 
frontal  sinuses;  (d)  affections  of  the  mouth  and  phamyx,  including  the 
tonsils, — e.g.,  tonsillitis,  angina,  diphtheria,  and  alveolar  abscess; 
(e)  inflammatory  lesions  in  the  distribution  of  the  facial  vein,  furuncle, 
cellulitis,  etc. ;  (/)  inflammatory  lesions  of  the  scalp.  Of  one  hundred 
and  eighty -two  cases  collected  by  Dwight  and  Germain  the  source  is 
given  as  follows :  From  the  eye,  4 ;  nose,  9 ;  mouth,  14 ;  face,  34,  and 
lips,  2. 

Pathological  Anatomy. — The  thrombi  of  an  infective  thrombo- 
phlebitis are  at  first  red  and  friable,  but  later  become  grayish  or  yellow- 
ish and  of  firmer  consistency.  The  lumen  of  the  sinus  may  be  com- 
pletely or  incompletely  obliterated  and  the  clots  are  more  or  less  adherent 
to  the  sinus  wall.  The  latter  becomes  thickened  and  its  internal  coat 
becomes  lustreless  and  rugose.  Infective  thrombi  should  be  distinguished 
easily  from  the  cadaveric  thrombi,  which  are  blue,  friable,  and  not 
adherent  to  the  sinus  walls. 

Meningeal  or  cerebral  lesions  are  more  or  less  widely  distributed, 
according  to  the  extent  of  the  thrombus.  If  limited  to  the  cavernous 
sinus,  a  localized  meningitis  may  develop,  which  may  become  diffuse  or 
may  resolve  itself  into  an  abscess.  On  the  other  hand,  if  the  thrombus  is 
extensive  and  involves  a  number  of  sinuses,  hemorrhages,  usually  menin- 


'  Boston  Medical  and  Surgical  Journal,  vol.  cxlvi.,  No.  18. 


870  THE    EYE    AND    NERVOUS    SYSTEM 

geal,  intra-arachnoid  or  subarachnoid  congestion,  oedema  of  the  brain, 
and  intraventricular  hydrops  may  be  looked  for.  Large  cerebral  hemor- 
rhages are  seldom  seen,  and  softening  of  the  brain  when  it  occurs  is  due 
to  the  local  stasis  or  the  anaemia  which  the  hemorrhage  may  bring  about. 

S)miptoms. — The  symptoms  of  thrombosis  of  the  cavernous  sinus  for 
the  most  part  are  due  to  obstruction  of  the  ophthalmic  veins.  They  are 
due  also  to  pressure  of  the  sinus  upon  tlie  adjacent  structures,  to  menin- 
geal irritation,  and  to  general  systemic  infection.  Usually  within  a  few 
days  of  the  onset  of  the  disturbance  of  the  sinus,  although  in  some  cases 
the  interval  is  much  longer,  signs  of  obstruction  make  themselves 
apparent;  oedema  of  the  eyelid  and  conjunctivae  and  face,  haziness  of 
the  cornea,  choked  disk,  dilatation  and  tortuosity  of  the  retinal  veins, 
with  oedema  of  the  papillae  and  retinal  hemorrhages,  exophthalmos,  due 
to  the  infiltration  of  the  cellular  tissue  of  the  orbit,  ptosis,  which  may  be 
the  result  either  of  the  oedema  of  the  upper  lid  or  of  paralysis  of  the 
oculomotor  nerve,  dilatation  of  the  pupil ;  the  movements  of  the  bulb  are 
restricted  (Plate  VII).  Pressure  of  the  swollen  sinus  upon  the  adjacent 
structures  will  excite  pain  in  the  distribution  of  the  ophthalmic  division 
of  the  trifacial  nerve  and  paralysis  of  the  third,  fourth,  and  sixth  nerves. 
While  in  some  cases  but  one  of  the  cavernous  sinuses  is  involved,  in  most 
instances,  on  account  of  the  free  communication  between  the  right  and 
left  sinuses,  the  thrombus  extends  rapidly,  usually  within  forty-eight 
hours,  from  one  to  the  other,  and  the  same  clinical  picture  is  reproduced. 
It  has  been  observed  that  the  development  of  the  symptoms  on  the  oppo- 
site side  is  associated  with  an  equally  rapid  subsidence  of  the  symptoms 
in  the  side  primarily  affected. 

The  cerebral  symptoms  are  constant  and  form  a  very  conspicuous 
part  of  the  clinical  picture :  headache  which  is  often  most  intense  and  of 
early  onset;  cerebral  irritation,  which  may  be  due  to  meningitis  or 
to  circulatory  disturbance  and  is  manifested  by  the  irritable  and  appre- 
hensive state  of  the  patient;  vomiting,  delirium,  exaggerated  reflexes, 
somnolence  and  even  coma  preceding  death.  These  symptoms  may  be 
due  to  the  typhoidal  state  or  to  the  circulatory  changes,  but  when  the 
process  has  extended  to  the  meninges,  typical  symptoms  of  meningitis, 
such  as  convulsions  and  retraction  of  head,  may  be  present.  This  latter 
complication  is  rare,  however,  since  when  the  process  extends  it  is  more 
likely  to  extend  in  the  direction  of  least  resistance, — namely,  from  sinus 
to  sinus. 

To  complete  the  clinical  picture  should  be  added  the  symptoms  which 
are  the  result  of  the  general  systemic  infection,  fever,  chills,  rapid  pulse 
and  respirations,  and  so  on.     As  one  would  expect,  pyajmia  is  a  very 


PLATE   VII. 


Original. 


Showing  protrusion  of  the  eyeball,  oedema  of  the  conjunctiva,  and  injection  of  the  corneal 
vessels  attending  obstruction  of  the  cavernous  sinus. 


SUKGICAL    TREATMENT  871 

common  termination,  and  the  case  becomes  further  complicated  by 
metastatic  deposits  in  the  lung  and  other  organs. 

Diagnosis. — It  should  be  borne  in  mind  that  obstruction  of  the 
circulation  in  the  cavernous  sinus  and  ophthalmic  veins  is  not  necessarily 
due  to  thrombophlebitis.  The  pressure  of  a  tumor  in  the  orbit  or  sella 
turcica  or  aneurisms  of  the  carotid  artery  may  so  interfere  with  the 
venous  circulation  as  to  simulate  thrombosis.  Cellulitis  of  the  orbit  and 
phlebitis  of  the  ophthalmic  veins  may  produce  the  same  effect.  In  the 
case  of  tumors  the  diagnosis  is  usually  based  upon  the  gradual  onset  of 
the  symptoms,  upon  the  absence  of  constitutional  signs  of  infection,  and 
upon  the  absence  of  cerebral  phenomena.  In  the  absence  of  cerebral 
phenomena  it  would  be  almost  impossible  to  distinguish  between  a  true 
sinus  thrombosis  and  a  phlebitis  of  the  ophthalmic  veins  or  orbital  cellu- 
litis. In  the  latter  case  the  changes  in  the  fundus  and  other  signs  of 
obstruction  would  not  be  so  marked,  the  condition  would  not  spread  to 
the  opposite  side,  and  headache  would  be  less  intense  and  more  super- 
ficial. To  differentiate  a  thrombosis  of  the  cavernous  sinus  from  that  of 
the  longitudinal  or  transverse  sinus  it  should  be  borne  in  mind  that 
thrombosis  of  the  longitudinal  sinus  is  attended  with  cyanosis  of  the 
face,  with  dilatation  of  veins  leading  from  the  great  fontanelle  to  the 
temporal  and  facial  veins,  and  with  epistaxis.  Thrombosis  of  the  trans- 
verse or  lateral  sinus  would  be  preceded  in  almost  every  instance  by  in- 
fection of  the  auditory  canal  or  mastoid  cells,  and  would  be  associated 
with  oedema  of  the  mastoid  region,  cellulitis  of  the  neck,  phlebitis  of  in- 
ternal jugular  vein,  and  perhaps  paralysis  of  the  facial  and  acoustic 
nerves. 

Surgical  Aspects. — When  one  considers  that  barely  seven  in  every 
hundred  cases  of  infective  thrombosis  of  the  cavernous  sinus  recover,  it 
would  seem  that  surgeons  would  have  endeavored  to  enter  the  field  long 
ago  with  the  hope  of  reducing  the  mortality,  and  yet  until  recent  years 
no  attempt  has  been  made.  The  treatment  of  the  condition  has  been 
said  to  be  preventive  only,  and  no  further  consideration  has  been  given 
the  matter.  The  difficulties  that  arise  once  we  consider  seriously  the 
surgical  aspects  and  the  surgical  possibilities  are  manifest.  In  the  first 
place,  the  condition  itself  is  one  which  is  not  easy  of  diagnosis,  and  in 
many  cases  the  diagnosis  has  not  been  made  at  all,  or  not  until  metas- 
tasis has  occurred,  or  the  toxaemia  is  so  profound  and  the  throinbosis  so 
extensive  that  the  case  is  regarded  as  desperate,  and  all  hope  of  recovery 
is  abandoned.  Then,  again,  the  cavernous  sinus  was  supposed  to  be  so 
inaccessible  that,  even  were  operative  intervention  considered  justifiable, 
no  effort  would  be  made  to  reach  it. 


872  THE    EYE    AND    NERVOUS    SYSTEM 

The  principles  to  be  observed  in  the  treatment  of  this  condition  are 
recognized  fully  and  have  been  applied  frequently  for  the  relief  of 
thrombosis  of  the  lateral  sinus,  and  with  a  great  measure  of  success.  If 
the  cavernous  sinus  could  be  approached,  opened,  and  drained  with  as 
much  ease  and  safety  as  the  lateral  sinus,  the  results  of  such  intervention 
should  be  as  good  in  one  as  in  the  other.  It  remained  for  Dwight,^  in 
an  operation  which  he  performed  in  January,  1900,  to  demonstrate  the 
feasibility  of  opening  and  draining  the  cavernous  sinus  in  case  of 
thrombosis.  The  case  is  one  of  sufficient  moment  to  warrant  a  brief 
abstract  of  it.  The  patient,  a  man  forty  years  of  age,  was  admitted  to 
the  Boston  City  Hospital  suffering  from  a  small  abscess  on  the  right 
side  of  his  face  just  above  the  upper  lip.  He  had  in  addition  all  the 
symptoms  of  obstruction  to  both  ophthalmic  veins,  more  marked  in  the 
right  than  in  the  left  side.  lie  was  in  a  state  of  profound  toxaemia. 
Under  ether  anaesthesia  the  cavernous  sinus  was  approached  by  tlje  route 
recommended  by  Krause  for  resection  of  the  Gasserian  ganglion.  The 
dura  was  opened;  the  tip  of  the  temporal  lobe  was  lifted  iipward  and 
backward  until  the  cavernous  sinus  was  felt.  With  a  protected  bistoury 
the  sinus  was  opened,  and  a  quantity  of  dark  semiclotted  blood  gushed 
out.  A  strip  of  gauze  was  introduced  for  drainage  and  the  wound  closed. 
The  results  were  almost  immediate ;  within  half  an  hour  the  exophthal- 
mus  almost  entirely  disappeared,  and  to  a  great  extent  the  chemosis  and 
o'dema.  Ilis  general  condition  improved  somewhat,  but  he  died  six  and 
a  half  hours  after  the  operation.  The  lesson  to  be  learned  from  this 
operation  is  twofold:  first,  that  the  cavernous  sinus  is  not  without  the 
realm  of  the  surgeon's  knife,  and,  second,  that  an  incision  into  the 
sinus  on  one  side  will  suffice  to  drain  the  sinus  on  the  other  side.  The 
fact  that  both  sinuses  have  been  involved  is  not  necessarily  a  contrain- 
dication to  operative  intervention. 

While  the  first  recorded  operation  aimed  directly  at  the  cavernous 
sinus,  tliere  have  been  cases  reported  in  which  recovery  has  ensued  after 
indirect  drainage, — that  is  to  say,  when  the  cavernous  sinus  has  become 
the  seat  of  a  thrombus  which  has  extended  from  the  lateral  sinus,  the 
latter  has  been  opened  and  curetted  with  the  intention  of  draining  all 
the  basal  sinuses,  and  in  throe  instances  the  operation  was  successful.  In 
discussing  the  technique  of  this  operation  three  points  are  to  be  consid- 
ered,— the  method  of  approach,  the  incision  in  the  dura,  and  the  method 
of  drainage. 

The  sinus  will  be  found  most  accessible  if  approached  by  the  tem- 
poral route :   the  opening  in  the  skull  may  correspond  precisely  with  the 

•  Boston  Medical  and  Surgical  Journal,  vol.  xlvi,  No.  18. 


SUEGICAL    TREATMENT  873 

one  which  would  be  made  for  exposure  of  the  Gasserian  ganglion.  The 
difference  between  the  two  operations  would  consist  essentially  in  this^ — 
namely,  that  in  the  exposure  of  the  ganglion  the  operation  is  extradural, 
whereas  in  the  exposure  of  the  sinus  it  is  almost  imperative  to  make  a 
dural  incision  and  approach  the  sinus  in  the  space  between  the  base  of 
the  brain  and  the  dura.  The  incision  in  the  dura  should  be  made  on  a 
level  with  the  base  of  the  skull,  the  base  of  the  brain  elevated,  and,  once 
the  sinus  is  reache4j  it  should  be  laid  open  and  the  thrombus  thoroughly- 
removed  with  a  curette.  It  is  most  important  that  the  seat  of  the 
thrombus  should  be  effectually  drained,  and  to  this  end  a  small  cigarette 
drain  should  be  introduced. 

TUMORS  OF  THE  OCCIPITAL  LOBE. 

Tumors  in  this  region  may  be  considered  very  properly  in  the  cate- 
gory of  intracranial  lesions  giving  rise  to  visual  disturbances.  If  the 
tumor  involves  the  occipital  lobe,  hemianopsia  will  be  the  only  focal 
symptom ;  although,  if  the  visual  tract  be  involved  on  its  way  to  the  left 
occipital  lobe,  there  may  be  sensory  aphasia.  The  exposure  of  tumors  in 
this  region  may  be  of  exceptional  difficulty  if  the  tumor  is  not  situated 
on  the  surface  of  the  brain,  immediately  beneath  the  cranial  vault. 
Tumors  situated  on  the  mesial  surface  or  upon  the  surface  in  relation 
with  the  tentorium  are  very  much  more  difficult  of  access.  There  seems 
to  be  a  very  much  greater  tendency  for  the  brain  to  bulge  in  operations 
upon  the  occipital  than  upon  any  other  lobe,  and  this  of  itself  constitutes 
a  very  troublesome  and  serious  obstacle.  In  the  first  place,  it  makes  it 
harder  to  retract  the  brain  sufficiently  to  explore  the  mesial  and  tentorial 
surfaces,  and,  in  the  second  place,  it  may  prevent  proper  coaptation  of 
the  edges  of  the  dural  wound. 

The  osteoplastic  flap  for  the  exposure  of  this  region  is  quadrangular 
in  shape ;  the  anterior  margin  corresponds  to  the  line  of  the  occipito- 
parietal suture,  the  posterior  margin  should  come  as  near  as  one  can  go 
with  safety  to  the  lateral  sinus,  the  superior  margin  should  be  parallel  to 
and  not  more  than  one-half  inch  from  the  median  line,  and  the  base  of 
the  flap  connects  the  inferior  extremities  of  the  two  sides.  The  time 
required  to  reflect  a  flap  in  the  occipital  region  will  be  longer  than  that 
for  any  other  region,  because  the  occipital  bone  is  considerably  thicker 
than  either  the  parietal,  temporal,  or  frontal  bone.  Once  the  flap  is 
reflected,  the  surface  of  the  brain  thus  exposed  to  view  should  be  care- 
fully examined,  and,  if  the  results  are  negative,  a  suitable  retractor  is 
introduced,  first  on  the  mesial,  then  on  the  tentorial  surface,  and  a 
careful  exploration,  both  visual  and  digital,  is  made.     Because  of  the 


874  THE    EYE    AND    NERVOUS    SYSTEM 

tendency  of  the  brain  to  bulge  in  this  region,  once  the  dura  has  been 
reflected,  the  manipulations  necessary  for  exploration  should  be  con- 
ducted as  rapidly  and  as  delicately  as  possible.  Rough  handling  and 
frequent  sponging  will  rapidly  excite  such  oedema  and  congestion  of  the 
brain  tissue  as  to  cause  an  increase  in  volume  of  the  affected  tissue  and 
greater  or  less  protrusion  of  brain  substance  through  the  dural  opening. 

Chronic  hydrocephalus  sometimes  simulates  brain  tumor,  and,  if  the 
posterior  horn  of  the  lateral  ventricle  be  very  much  delated,  the  overlying 
layer  of  brain  tissue  may  be  so  thin  as  to  rupture  during  the  operation, 
and  thus  establish  a  direct  communication  with  the  ventricle.  In  a  case 
under  the  author's  observation  this  condition  was  found ;  at  first  it  was 
mistaken  for  a  cyst,  but  further  investigation  showed  that  the  cavity 
which  had  been  opened,  was  the  posterior  horn  of  the  dilated  ventricle. 

If,  when  the  exploration  has  been  completed  and  the  tumor  (if 
found)  removed,  there  be  any  difficulty, in  closing  the  dural  wound, 
because  of  undue  protrusion  of  the  brain,  it  will  be  necessary  to  fashion 
a  flap  from  the  pericranium  sufficiently  large  to  close  the  defect  left  by 
the  insufficient  size  of  the  dural  flap.  The  pericranial  flap  may  be 
dissected  from  a  portion  of  the  skull  adjacent  to  the  field  of  operation  or 
from  the  segment  of  bone  in  the  osteoplastic  flap.  When  the  tumor  has 
not  been  found,  or  if  found  cannot  be  removed,  it  is  better  to  remove  the 
pericranium  from  the  bone  in  the  flap,  since  for  the  permanent  relief  of 
pressure  the  osseous  flap  should  not  be  replaced.  The  wound  in  the  scalp 
should  be  closed  throughout  with  interrupted  silkworm-gut  sutures  and 
drainage  material,  preferably  rubber  tissue,  introduced  through  one  or 
two  openings  made  by  incising  the  scalp  obliquely  one  or  two  centimetres 
to  the  outer  side  of  the  margin  of  the  flap.  The  drainage  material  may 
be  removed  in  twenty-four  hours  and  the  sutures  one  day  later. 

PRESERVATION  OF  VISION  IN  CASES  OF  BRAIN  TUMOR. 

The  most  common  cause  of  disturbance  of  vision  in  cases  of  brain 
tumor  is  an  optic  neuritis,  leading  to  choked  disk  and  eventually  to  optic 
atrophy.  While  optic  neuritis  may  be  due  to  other  intracranial  diseases 
as  well  as  to  extracranial  lesions — as  albuminuria  and  chlorosis — the 
most  frequent  source  is  brain  tumor,  and,  according  to  Gowers,  a  con- 
siderable degree  of  neuritis  is  rarely  due  to  any  other  cause.  Optic 
neuritis  is  not,  as  was  hitherto  supposed,  due  to  increased  intracranial 
tension.  In  fact,  it  is  rarely  due  to  any  single  factor,  but  the  most 
potent  factor  is  the  descent  of  a  process  of  tissue  irritation,  which 
when  it  reaches  the  papilla,  sets  up  a  more  intense  inflammation.  But 
whatever  the  cause,  once  the  neuritis  begins  it  will  lead  eventually  to 


SURGICAL    TREATMENT 


875 


choked  disk  and  finally  to  optic  atrophy  and  total  and  irremediable 
blindness.  It  is  important  to  bear  in  mind  that  this  process  does  not 
develop  always  with  the  same  degree  of  rapidity,  and,  as  a  rule,  the  more 
rapidly  it  develops  the  more  intense  it  becomes.  In  some  cases  months 
may  elapse  and  the  neuritis  still  be  of  a  mild  grade,  while  in  others 
again  it  may  reach  a  stage  of  great  intensity  within  a  fortnight.  The 
essential  points  to  be  remembered  with  relation  to  the  development  of  an 
optic  neuritis  due  to  brain  tumors  are  these:  first,  that,  if  it  is  not 
relieved  before  the  optic  nerve  becomes  atrophied,  permanent  blindness 
will  be  inevitable ;  and,  second,  that  in  its  early  stages,  by  the  perform- 
ance of  an  operation  easy  of  execution  and  free  from  danger,  the  neuritis 
•will  subside  and  vision  be  restored.    In  addition  to  disturbance  of  vision, 


Fig.  2.— Original.  Hernia  cerebri,  showing  tlie  very  marked  protrusion  which  follows  the  removal 
of  a  considerable  portion  of  the  occipital  bone  In  palliative  operations  for  the  relief  of  increased 
intracranial  tension. 

patients  with  brain  tumors  also  suffer  from  headache  and  vomiting,  and 
not  only  the  former  but  the  latter  as  well  may  be  relieved  by  the  same 
operation.  The  operation  which  should  be  performed  in  these  cases  is 
the  trepanation  decompressive  of  the  French,  and  consists  essentially  in 
the  removal  of  a  not  inconsiderable  section  of  bone  from  the  cranium. 
It  matters  not  from  what  portion  of  the  cranium  the  bone  is  removed  in 
so  far  as  concerns  the  effectiveness  of  the  procedure,  although  preference 
should  be  given  to  the  parietal  or  temporal  region  rather  than  the  occip- 
ital or  frontal.  The  frontal  region  is  objectionable  for  cosmetic 
reasons,   and  the  occipital  region  because  here  there  seems  to  be   a 


876  THE    EYE    AND    NERVOUS    SYSTEM 

tendency  to  the  more  rai)i(l  development  of  a  hernia  cerebri,  and  in  this 
position  the  protrusion  would  be  conspicuous  and  unsightly  (Fig.  2).  A 
section  of  bone  six  to  eight  centimetres  in  its  various  dimensions  should 
be  removed  from  the  parietal  or  temporal  region,  first  on  one  side,  and 
later  on  the  other,  if  after  the  first  operation  there  are  any  signs  of  recur- 
rence. It  is  not  only  unnecessary  but  inadvisable  to  remove  the  dura, 
because  of  the  possibility  of  a  fungus  cerebri  developing  should  the 
wound  become  infected.  The  dura  may,  however,  be  cut  in  a  stellate 
fashion,  as  recommended  by  Kocher,  beginning  in  the  centre  of  tlie 
opening  and  making  several  radiating  incisions  towards  the  periphery. 
When  vision  is  beginning  to  fail,  delay  or  postponement  of  the  operation 
for  any  length  of  time  is  absolutely  unwarrantable.  In  some  cases  it 
may  be  impossible  at  first  to  localize  the  tumor  accurately,  but  this  should 
not  constitute  a  contraindication,  and  the  surgeon  should  proceed  to 
operate,  if  only  to  save  the  patient's  vision.  Needless  to  say,  the  indi- 
cations for  operation  are  quite  as  positive  in  inoperable  growths.  There 
is  no  treatment,  whether  medicinal  or  surgical,  which  gives  a  greater 
measure  of  relief  to  patients  suffering  from  an  incurable  lesion  than  the 
so-called  trepanation  decompressive.  The  relief  from  headache  and 
vomiting  is  often  immediate  and  permanent,  and  if  the  optic  neuritia 
is  not  of  too  long  duration  the  patient's  sight  will  be  restored. 

"^  TUMORS  OF  THE  CHIASM. 

In  the  great  majority  of  instances  the  optic  chiasm  is  injured  by 
tumors  taking  their  origin  from  the  pituitary  body,  and  of  these  a  con- 
siderable proportion  occur  in  cases  of  acromegaly.  No  attempt  has  been 
made  to  expose  or  remove  these  growths,  because  they  are  believed  to  be 
inaccessible.  Were  it  not  for  their  inaccessibility  many  of  the  tumors 
could  be  removed,  because  a  large  proportion  of  them  are  benign.  The 
malignant  tumors,  probably  under  any  circumstances,  should  be  re- 
garded as  inoperable.  Owing  to  the  firm  attachments  of  the  dura  to  the 
various  processes  at  the  base  of  the  skull,  an  extradural  operation  would 
be  impossible.  Even  though,  by  the  temporal  route,  one  should  succeed 
in  reaching  the  foramen  lacerum  anterior,  which  in  itself  is  not  so  easy 
of  access,  the  sella  turcica  and  the  processus  clynoideus  posterior  would 
still  have  to  be  passed.  Kiliani  ^  made  some  observations  upon  the 
cadaver  for  the  purpose  of  elaborating  a  practical  metliod  of  reaching  the 
chiasm,  and,  after  trying  various  means  of  approach,  he  concluded  that 
the  frontal  route  was  the  only  rational  one.     11  is  operation,  as  practised 


*  Annals  of  Surgery,  July,  1904. 


CEKEBELLAK  OPEEATIOA^S  877 

on  the  cadaver,  consisted  in  reflecting  an  osteoplastic  flap  of  omega 
shape ;  the  base  of  the  flap  was  about  one-half  inch  behind  the  coronary 
suture  and  three  and  a  half  inches  broad,  its  width  seven  inches,  and 
depth  five  inches.  A  similarly  shaped  flap  of  the  dura  was  reflected 
after  the  longitudinal  sinus,  which  at  this  point  is  no  larger  than  a  small 
vein,  had  been  ligated.  Elevation  of  the  frontal  lobe  exposed  the  optic 
nerve. 

This  operation,  though  necessitating  a  very  liberal  exposure,  need 
not  be  regarded  as  one  of  unusual  gravity.  The  rapid  and  efficient 
methods  of  making  an  osteoplastic  flap  now  in  practice  eliminate  as  a 
possible  objection  to  this  operation  the  magnitude  of  the  flap.  The  suc- 
cess or  failure  of  the  operation  would  depend  upon  the  ease  with  which 
on  the  living  subject,  not  on  the  cadaver,  the  brain  could  be  elevated 
sufficiently  to  expose  the  affected  region.  Though  as  yet  I  have  had  no 
experience,  I  would  not  hesitate  in  a  suitable  case  at  least  to  make  the 
attempt  to  expose  the  tumor. 

OPERATIONS  UPON  THE  CEREBELLUM  FOR 
RELIEF  OF  CHOKED  DISKS. 

One  of  the  most  constant  symptoms  of  tumors  of  the  cerebellum  is 
choked  disk.  The  appearance  of  certain  subjective  symptoms,  such  as 
headache  and  vertigo,  may  prompt  the  patient  to  consult  a  physician 
before  he  has  noticed  any  disturbance  of  vision,  but  in  most  cases  by  the 
time  the  physician  is  consulted  an  examination  of  the  eye-grounds  will 
reveal  choked  disks  of  greater  or  less  intensity. 

Indications  for  Operation. — In  general  it  may  be  said  that  indica- 
tions for  operation  in  cases  of  suspected  tumor  of  the  cerebellum  do  not 
differ  materially  from  those  that  apply  in  tumors  of  the  cerebrum.  In 
both  classes  of  cases,  once  the  diagnosis  has  been  made,  operation  should 
not  be  delayed  for  any  length  of  time.  Too  much  stress  cannot  be  laid 
upon  the  importance  of  presenting  the  case  to  the  surgeon  at  the  earliest 
possible  moment.  Months  have  been  spent  in  the  fruitless  administra- 
tion of  antisyphilitics  before  operation  has  been  considered,  and  in  many 
cases  an  unwarranted  amount  of  time  has  been  spent  in  the  efforts  to 
establish  the  diagnosis  beyond  the  peradventure  of  doubt  or  to  localize 
the  tumor  with  mathematical  accuracy.  As  Kocher  has  said,  there  is  no 
more  excuse  to-day  for  delaying  operation  because  the  tumor  has  not 
been  localized  exactly  than  there  would  be  for  declining  to  operate  upon 
a  case  of  intracranial  hemorrhage  because  one  was  unable  to  determine 
the  exact  seat  of  the  clot.  Postponement  of  operation  should  be  dis- 
countenanced for  several  reasons.     In  the  first  place,  in  cases  of  long 


878  THE    EYE    AND    NERVOUS    SYSTEM 

duration  the  patients  make  very  poor  subjects  for  operative  intervention ; 
the  operation  is  itself  one  of  considerable  gravity,  and  the  condition  of 
the  patient  should  be  such  as  to  enable  him  to  withstand  its  depressing 
effect.  The  longer  the  delay  the  larger  the  tumor  and  the  less  the  possi- 
bility of  its  complete  removal.  Wliat  might  have  been  a  radical  opera- 
tion becomes  now  only  palliative  in  its  effect.  These  arguments  against 
delay  apply  to  the  treatment  of  tumors  in  general,  but  the  presence  of 
choked  disks  in  cases  of  cerebellar  tumors  constitutes  in  itself  a  plea 
for  early  operation  which  cannot  be  disregarded.  If  too  much  time  is 
allowed  to  elapse  after  the  beginning  of  the  choked  disk,  an  optic  neuritis 
will  develop,  and  the  time  for  the  partial  or  complete  restoration  of 
vision  will  have  passed.  When  we  consider  that  in  practically  every 
case  early  operation,  whether  it  be  palliative  or  radical,  in  addition  to 
relieving  many  other  distressing  conditions,  such  as  headache  and  vomit- 
ing, will  restore  vision  also,  it  is  plainly  our  duty  to  consider  but  one 
plan  of  treatment — namely,  the  operative — and  to  put  it  into  effect 
without  delay. 

Anatomical  Considerations — When  one  takes  into  consideration  the 
position  of  the  lateral  and  occipital  sinuses  with  relation  to  the  only 
means  of  access  to  the  cerebellum,  and  the  plane  of  the  tentorium  cere- 
belli,  he  realizes  at  once  that  there  are  special  technical  difficulties  in 
surgical  attacks  upon  the  cerebellum  (Fig.  3).  Even  when  one  has 
removed  a  considerable  portion  of  the  skull  below  the  superior  curved 
line,  there  will  be  exposed  to  view  but  a  relatively  small  portion  of  its 
gross  surface  area.  Neither  the  upper  surface  nor  the  under  surface, 
but  only  a  portion  of  the  posterior  aspect  is  exposed  to  view  by  this  pro- 
cedure; whereas,  in  the  case  of  the  cerebrum  the  entire  cortex,  the 
mesial  surface  and  the  base,  can  be  exposed  without  any  difficulty.  In 
addition  to  the  larger  sinuses,  certain  tributaries  of  sufficient  size  to 
cause  when  injured  profuse  and  sometimes  alarming  hemorrhage,  pene- 
trate this  portion  of  tlie  occipital  bone  which  must  be  removed.  The 
most  constant  of  these,  a  branch  of  the  lateral  sinus,  will  be  found  about 
two  centimetres  to  the  inner  side  of  the  mastoid  process.  Occasionally 
one  or  more  will  be  found  just  below  the  superior  curved  line,  external 
to  the  occipital  protuberance. 

The  proximity  of  the  pons,  and  especially  the  medulla  oblongata,  to 
the  field  of  operation  should  be  borne  in  mind.  If  in  an  attempt  to 
sufficient  exposure  of  the  suspected  area  too  much  traction  or  pressure  is 
made  upon  the  medulla,  the  results  might  be  fatal. 

Furthermore,  the  cavity  of  the  cerebellum  is  very  much  smaller  than 
that  of  the  cerebrum,  so  that  there  is  correspondingly  less  space  in  which 


CEREBELLAR   OPERATIONS 


879 


to  conduct  the  manipulations  necessary  for  exposure  or  removal  of  the 
tumor.  In  the  adult  skull  one  hemisphere  of  tlie  cerebellum  is  contained 
in  a  cavity  whose  greatest  dimension  is  only  10.5  cubic  centimetres.  In 
addition  to  the  difficulties  that  are  associated  with  operations  in  a  space 
so  small  and  difficult  of  approach,  one  is  hampered  further  by  the  fact 
that  even  under  normal  conditions  the  cerebellar  hemispheres  are  com- 


FiG.  3.— Photograph  of  a  horizontal  section  of  the  head  cut  on  a  level  with  the  external  auditory 
meatus  ;  a,  representing  a  point  at  the  cerebellopontile  angle ;  b,  the  auditory  nerve  entering  the  in- 
ternal auditory  meatus  ;  c,  d,  e,  three  points  on  the  skull.  Note  the  distance  between  point  a  and  the 
points  c,  d,  and  e,  as  illustrating  the  shortest  route  to  the  cerebellopontile  angle  respectively.  The 
shortest  distance  from  the  skull  to  the  angle  is  measured  along  a  line  drawn  between  a  and  c.  The 
farther  away  from  c  or  the  nearer  to  e  the  greater  will  be  this  distance. 

pressed  in  a  relatively  smaller  space  than  the  hemispheres  of  the  cere- 
brum, and  are  so  compressed  that,  when  tension  is  relieved  by  opening  of 
a  dural  flap,  cerebellar  tissue  always  portrudes  through  the  opening; 
the  tissues  cannot  be  displaced  or  retracted,  therefore,  either  to  the  same 
degree  or  with  as  much  ease  as  can  the  cerebral  lobes. 

Operative  Technic. — Position  of  the  Patient. — The  head  and  shoul- 
ders should  be  considerably  elevated,  as  this  will  to  a  certain  extent 


880  THE    EYE    AND    NERVOUS    SYSTEM 

diminish  the  amount  of  hemorrhage.  To  afford  greater  freedom  for  the 
necessary  steps  of  the  operation,  a  suitable  head-rest  should  be  attached 
to  the  operating  table  (Fig.  4).  Schede  places  his  patients  in  the  sitting 
posture  leaning  far  forward.  This  posture,  he  says,  diminishes  hemor- 
rhage to  a  considerable  degree,  but  the  position  is  an  awkward  and 
difficult  one  in  which  to  retain  the  fully  anaesthetized  patient. 

Incision. — The  incision  begins  at  the  tip  of  the  mastoid  process  on  the 
affected  side  and  runs  parallel  to  and  one  centimetre  above  the  superior 
curved  line.  From  this  point  a  vertical  incision  is  made  sufficiently 
long  to  expose  the  field  of  operation.  The  flap  should  contain  the  skin, 
muscle,  and  pericranium.  If  the  pericranium  and  muscles  are  reflected 
together,  there  will  be  considerably  less  hemorrhage. 

Removal  of  Bone. — For  various  reasons  it  is  not  feasible  or  desirable 
to  make  an  osteoplastic  flap.  The  muscles  and  aponeurosis  are  thick 
enough  in  this  region  to  offer  adequate  protection  to  the  underlying 
structures  and  to  make  bony  closure  of  the  opening  unnecessary.  A 
small  opening  is  chiselled  out  at  a  point  midway  between  the  occipital 
protuberance  and  the  mastoid  process.  Here  the  bone  is  comparatively 
thin  and,  as  Poirier  says,  this  is  the  point  of  greatest  safety.  When 
completed,  the  opening  in  the  skull  should  extend  upward  one  centimetre 
above  the  superior  curved  line,  outward  as  far  as  one  can  go  without 
opening  the  mastoid  cells,  inward  to  within  one  centimetre  of  the  median 
line,  and  downward  to  a  point  at  least  one  centimetre  distant  from  the 
foramen  magnum. 

Exploration. — After  the  dural  flap  is  reflected,  one  proceeds  at  once 
to  search  for  a  tumor,  unless  it  has  been  decided  to  resort  to  the  two- 
stage  operation.  The  principles  to  be  applied  in  deciding  this  question 
are  precisely  those  which  have  been  adopted  in  our  operations  for  tumors 
of  the  cerebrum.^  Protrusion  of  the  cerebellum  through  the  opening, 
once  the  dural  flap  is  reflected,  is  indicative'  of  increased  intracranial 
tension  and  suggests  the  presence  of  a  tumor,  abscess,  cyst,  or  internal 
hydrocephalus.  Under  normal  conditions  the  cerebellum  is  under  greater 
tension  than  the  cerebrum,  and,  when  the  dura  is  incised,  a  normal  cere- 
bellum will  protrude  in  many  cases,  at  least  to  a  slight  degree.  The 
surface  of  the  cerebellum  should  be  inspected  and  palpated  on  all  sides, 
to  discover,  if  possible,  any  abnormality  in  appearance  or  consistency. 
If  there  is  reason  to  believe  that  the  tumor  is  subcortical,  an  exploratory 
incision  is  made,  and,  if  the  neoplasm  is  found,  it  is  removed.  On  the 
other  hand,  if  the  symptoms  point  t/>  a  tumor  at  the  cerebellopontile 

*  American  Journal  of  the  Medical  Sciences,  February,  1904. 


CEREBELLAR   OPERATIONS 


881 


angle,  the  subsequent  steps  of  the  operation  will  be  attended  with  some 

difficulty. 

Exposure  of  the  Cerebellopontile  Angle. — To  enable  one  to  expose  a 
tumor  in  this  region  it  may  be  necessary  to  resort  to  one  of  two  pro- 


5v. 


l^^^Bt        ,  ^4 


^^!:h^*  ■  } 


\\  \ 


Fig.  5.— The  larger  figure  to  the  left  illustrates  the  operation  for  the  combined  exposure  of  one 
cerebellar  hemisphere  and  the  occipital  lobe  of  the  cerebrum.  The  smaller  figure,  above  and  to  the 
right,  illustrates  the  structures  in  relation  to  the  anterior  aspect  of  the  cerebellum  and  the  petrous 
portion  of  the  temporal  bone.  Attention  is  called  especially  to  the  jwsition  of  the  fifth,  seventh  and 
eighth  cranial  nerves.  This  drawing  was  made  by  viewing  the  structures  from  the  lateral  aspect, 
such  an  exposure  as  would  be  made  in  exploring  for  tumors  of  the  cerebellopontile  angle.  1.  Osteo- 
plastic flap  reflected  in  an  operation  for  the  combined  exposure  of  occipital  lobe  and  cerebellum. 
2.  Ninth,  tenth,  and  eleventh  cranial  nerves.  3.  Auditory  nerve  drawn  to  one  side  by  retractor  in 
order  to  expose.  4.  The  facial  nerve  which  lies  directly  beneath  it.  5.  The  root  of  the  trigeminus 
as  it  enters  the  groove  at  the  apex  of  the  petrous  portion  of  the  temporal  bone. 


cedures,  tapping  of  the  lateral  ventricles  or  removal  of  a  large  portion 

of  the  cerebpllar  hemisphere.     The  former  method  is  disapproved  of,  on 
56 


882 


THE    EYE    AND    NERVOUS    SYSTEM 


the  ground  that  it  is  so  fatal  in  its  tendencies.  The  alternative,  on  the 
other  hand,  is  attended  with  very  different  results.  The  impunity  with 
which  large  collections  of  cerebellar  tissue  may  be  removed,  not  only 
without  endangering  life  but  without  disturbance  of  function,  was  an  ob- 
servation made  by  physiologists  long  ago,  and  which  has  been  confirmed 
in  a  number  of  cases  in  my  own  experience.  When,  therefore,  it  is  dijfi- 
cult  to  expose  the  cerebellopontile  angle,  or  if  a  tumor  has  been  found 
and  there  is  not  sufficient  space  for  its  removal,  the  operator  should  not 
hesitate  in  removing  at  least  a  third  of  the  cerebellar  hemisphere  (Fig. 
5).  The  dangers  attending  the  manipulations  necessary  for  removal  of 
a  tumor  from  this  region,  owing  to  the  proximity  of  the  medulla  and 
pons,  are  not  imaginary,  and  it  is  important  to  avoid  infliction  of  a 


Fig.  6.— Operation  for  the  simultaneous  exposure  of  both  cerebellar  hemispheres,  necessitating 
ligation  of  the  occipital  sinus.  1.  The  occipital  sinus,  which  haa  been  ligated  previously  and  reflected 
with  the  dura.  2.  Mastoid  process.  3.  A  large  tributary  of  the  lateral  sinus,  invariably  opened  in 
cerebellar  craniectomies  and  of  varying  dimensions ;  said  to  be  sometimes  as  large  as  the  lateral  sinus. 
4.  Lateral  sinus.    5.  Occipital  protuberance.    6.  Occipital  sinus. 


degree  of  traumatism  upon  these  structures  which  may  determine  a  fatal 
result.  One  should  bear  in  mind  that  the  shortest  distance  from  the 
surface  of  the  skull  to  this  angle,  and,  therefore,  the  best  method  of 
approach,  is  along  a  line  parallel  to  the  petrous  portion  of  the  temporal 
bone.  The  region  to  be  exposed  should  be  approached,  therefore,  from 
the  lateral  rather  than  the  superior  or  inferior  aspect  of  the  cerebel- 
lum. Not  only  is  this  the  shortest  route,  but  the  safest  also,  in  that 
the  manipulations  are  carried  on  at  a  point  farthest  distant  from  such 
vital  structures  as  the  pons  and  the  medulla. 

Simultaneous  Exposure  of  Both  Hemispheres;  Bilateral  Craniectomy. — In 
many  cases  it  will  be  impossible  to  determine  beforehand  whether  the 


CEKEBELLAK   0PERATI0:N^S  883 

tumor  is  in  the  right  or  the  left  lobe,  so  that,  failing  to  find  the  tumor  on 
one  side,  one  must  proceed  at  once,  or  after  a  proper  interval  of  time,  to 
explore  the  opposite  side.  In  deciding  this  question  one  must  be  gov- 
erned entirely  by  the  condition  of  the  patient.  The  incision  is  extended 
from  the  termination  of  the  point  at  the  occipital  protuberance  to  the 
opposite  mastoid  process  and  the  flap  reflected  in  the  manner  already 
described  (Fig,  6).  An  opening  in  the  bone  is  made  in  the  same  manner 
and  of  the  same  dimensions  as  on  the  opposite  side  and  the  intervening 
bridge  of  bone  removed  with  a  Gigli  saw.  The  f  alx  cerebelli  is  punctured 
on  either  side  of  the  occipital  sinus  and  the  latter  divided  between  two 
ligatures.  This  will  enable  one  to  reflect  a  flap  of  the  dura  covering  both 
hemispheres  and  to  displace  the  cerebellum  with  greater  freedom  than 
would  be  possible  if  an  intervening  bridge  of  bone  remained  between  the 
two  openings.  Bilateral  craniectomy  should  not  be  resorted  to  as  a 
routine  procedure,  but  should  be  restricted  to  those  cases  in  which  the 
tumor  is  believed  to  occupy  a  position  near  the  mesial  surface  or  in 
which  the  tumor  w^as  not  found  on  the  side  first  exposed.  In  most 
instances  it  should  be  practised  in  two  sittings. 

Palliative  Operations. — Under  certain  circumstances  we  despair  of 
being  able  to  perform  a  radical  operation:  either  the  tumor  cannot  be 
found  01  cannot  be  localized;  it  may  be  inaccessible  or  it  may  have 
attained  such  proportions  as  to  make  its  removal  impracticable.  In  any 
of  these  contingencies  a  palliative  operation  is  justifiable,  and  in  some 
cases  should  be  regarded  as  imperative.  The  headache,  vertigo,  and 
vomiting,  so  constant  in  cerebellar  tumors,  makes  the  life  of  the  patient 
pitiable,  and  yet  he  may  be  relieved  of  all  these  symptoms  for  a  consider- 
able time  by  adopting  such  measures  as  will  relieve  pressure.  But  the 
strongest  argument  against  delay  of  operation  in  the  treatment  of  cere- 
bellar tumors  is  the  possibility  of  being  able  to  save  the  patient's  vision : 
choked  disk  is  one  of  the  most  constant  symptoms  and,  if  permitted  to 
continue  too  long  unrelieved,  an  optic  atrophy  will  develop,  and  the 
time  for  complete  or  even  partial  restoration  of  vision  will  have  passed. 
Palliative  operations  upon  the  cerebellum  do  not  consist  alone  in  the 
removal  of  the  overlying  bone  for  the  relief  of  tension,  as  in  such  opera- 
tions upon  the  cerebrum,  but  in  the  removal  of  a  portion — one-third  to 
one-half — of  the  cerebellar  hemisphere.  Such  a  procedure,  mutilating 
as  it  seems,  does  not  appear  to  add  to  the  gravity  of  the  operation,  nor 
does  it  cause  any  demonstrable  disturbance  of  function,  but  affords  that 
relief  of  pressure  which  alone  will  secure  the  desired  results. 

Hemorrhage. — Hemorrhage  is  not  troublesome  once  the  bone  has 
been  removed ;   occasionally,  however,  there  may  be  an  alarming  hemor- 


884  THE    EYE    AND    NERVOUS    SYSTEM 

rhage  from  one  or  another  of  the  various  sinuses  that  traverse  the  occip- 
ital bone.  In  exceptional  cases  these  sinuses  are  so  large  that  a  patient 
may  lose  an  enormous  amount  of  blood  in  a  very  short  time,  so  that  one 
should  be  prepared  always  with  suitable  means  for  controlling  hemor- 
rhage from  this  source.  Horsley's  wax  should  be  tried  first,  but,  if  this 
fails,  the  outlet  of  the  sinus  should  be  closed  with  plugs  of  wood  which 
can  be  whittled  to  the  proper  size  and  length  from  ordinary  swab-sticks. 
Nothing  but  carelessness  would  permit  of  injury  to  the  lateral  sinus 
during  operation.  Should  such  an  accident  occur,  it  is  quite  feasible  to 
control  bleeding  by  ligation  of  the  sinus. 

Results. — ^ly  personal  experience  leads  me  to  believe  that  the  dan- 
gers attending  cerebellar  operations  have  been  somewhat  exaggerated. 
I  have  been  struck  especially  with  the  comparatively  slight  depression 
attending  these  operations  and  with  the  rapidity  with  which  reaction 
ensues.  It  should  be  borne  in  mind,  however,  that,  when  the  tumor  is 
found,  the  act  of  removing  it  is  not  of  itself  attended  with  any  additional 
risks.  ^lention  has  already  been  made  of  the  fact  that  patients  with 
cerebellar  tumors  are  not  subjects  favorable  for  operation,  yet  the  risk 
lies  not  in  any  feature  or  stage  of  the  operation,  but  in  the  operation 
per  se;  therefore,  the  mortality  attending  those  cases  in  which  the  tumor 
has  been  found  and  removed  should  not  be  greater  than  that  attending 
an  operation  which  has  been  solely  exploratory.  The  mortality  will  be 
reduced  to  a  minimum  by  observing  most  scrupulously  those  measures 
which  are  said  to  be  preventive  of  shock:  securing  prompt  hn?mostasis, 
inflicting  a  minimum  degree  of  traumatism  upon  the  cerebellar  tissue, 
avoiding  undue  traction  or  pressure  upon  the  pons  and  medulla,  and 
resorting  to  the  two-stage  operation  according  to  the  principles  already 
laid  down. 

The  effects  of  the  removal  of  tumors,  or  in  palliative  operations  of 
the  removal  of  a  portion  of  the  cerebellar  hemisphere,  are  as  prompt  as 
they  are  gratifying.  Headache  and  nausea  cease  almost  at  once ;  choked 
disk  subsides,  and  vision  may  be  almost  restored  or  considerably  im- 
proved within  a  few  days ;  the  ataxia  disappears  and  the  stability  of 
gait  is  often  restored.  The  results  of  operations  upon  the  cerebellum, 
whether  radical  or  palliative,  are  usually  more  striking  and  give  a  degree 
of  relief  greater  than  after  similar  operations  upon  the  cerebrum.  The 
following  statistics  were  compiled  from  one  hundred  and  sixteen  cases 
of  cerebellar  tumors  selected  bv  the  author :  ® 


•New  York  Medical  Journal  and  Philadelphia  Medical  Journal,  February'   18, 
1905. 


SURGERY  OF  FACIAL  NERVE  885 

CEREBELLAR  TUMORS. 

Per  cent 

Tumors  found    45 

Tumors    not    found    55 

Removal  with  recovery    15 

■     Removal    with    improvement    13.9 

Removal  without  improvement 0.9 

Improvement  without  removal    13.9 

No  improvement  without  removal 13.9 

Death  when  tumor  was  removed    12.9 

Death  when  tumor  was  not  found  and  not  removed   28.7 

A  comparison  of  the  statistics  of  successive  dates  shows  an  increase 
in  the  percentage  of  recoveries  and  improvements  and  a  reduction  in  the 
mortality : 

Frazier's  Duret's  Oppenheim'3 

Table,  1904.  Table,  1903.  Table,  1902. 

Results.                                                              Percent  Percent.  Percent. 

Recovery    15  14  7.5 

Improved     28  25  7.5 

Unimproved    15  . .  13 

Mortality 42  60  71 

A  comparison  of  the  statistics  of  the  total  number  of  cases  in 
Frazier's  collection  with  the  statistics  of  the  last  five  years  shows  a 
manifest  improvement  in  the  results : 

Total  Number    Cases  reported  during 
of  Cases.  past  five  years, — 

Results.  Per  cent.  1899-1904.    Per  cent 

Recovery    15  24 

Improved    28  28.5 

Unimproved     15  11 

Mortality     42  25.8 

SURGERY  OF  THE  SEVENTH  NERVE;  OPERATIVE 
TREATMENT  OF  FACIAL  PALSY. 

Historical. — It  has  been  proved  by  experimental  work  that  regenera- 
tion in  a  partially  degenerated  nerve  will  occur  when  the  peripheral 
segment  of  the  affected  nerve  is  united  to  the  proximal  segment  of  a 
jierfectly  healthy  nerve.  The  first  application  of  the  principles  here 
involved  to  the  treatment  of  facial  palsy  is  credited  to  Ballance  and 
Stewart,  who,  in  1895,  performed  the  first  operation  upon  the  human 
subject,  uniting  the  facial  to  the  spinal  accessory  nerve.  Since  that  time 
this  operation,  modified  in  several  ways,  has  been  performed  a  number 
of  times,  with  varying  degrees  of  success. 

Indications. — Xot  every  case  of  facial  palsy  presents  the  indications 
for  the  operative  treatment.  To  prevent  the  method  from  falling  into 
disrepute,  the  cases  should  be  selected  with  the  utmost  care.  In  the 
consideration  of  the  treatment,  cases  of  facial  palsy  for  convenience  sake 
may  be  classified  as  follows: 


886  THE    EYE    AND    NERVOUS    SYSTEM 

(1)  Cases  due  to  traumatism, — e.g.,  gunshot  and  stab  wounds  or 
fracture  of  the  base  of  the  skull.  (2)  Cases  due  to  an  infective  neuritis 
secondary  to  mastoiditis.  (3)  Cases  due  to  Bell's  palsy.  (4)  Cases  of 
paralysis  following  section  of  the  nerve  in  the  removal  of  a  tumor  from 
the  face  (Ballance). 

As  to  the  traumatic  cases,  the  sooner  the  operation  is  performed  the 
better  the  prognosis;  therefore,  when  the  nerve  is  believed  to  be  de- 
stroyed, the  operation  should  be  performed  without  delay. 

In  those  cases  secondary  to  mastoiditis  operation  should  be  resorted 
to  as  soon  as  the  infection  has  subsided  and  the  possibility  of  wound 
infection  from  that  source  eliminated. 

Delay  is  not  only  justified  but  proper  in  all  cases  of  Bell's  palsy,  but 
only  for  a  limited  time.  If  after  the  expiration  of  six  months  the 
paralysis  has  not  responded  to  appropriate  treatment  (when  the  muscles 
are  almost  completely  paralyzed  and  the  reaction  of  degeneration  is  very 
pronounced),  the  operation  should  be  performed  at  once.  Does  the  opera- 
tion hold  out  any  hope  to  those  who  have  suffered  from  this  condition  for 
years — two,  five,  fifteen,  twenty  years  or  more  ?  In  answer  to  this 
question  it  should  be  said  that  this  is  of  comparatively  little  significance 
in  so  far  as  the  nerve  itself  is  concerned.  The  keynote  to  thQ  situation  is 
the  condition  of  the  muscles.  If  the  facial  muscles  are  completely 
atrophied  and  will  respond  no  longer  to  galvanic  stimulation,  the  pros- 
pects of  restoration  of  function  are  extremely  doubtful.  Up  to  the 
present  time  seven  years  and  nine  months  (Ilackenbruch's  case)  is  the 
longest  period  at  the  expiration  of  which  the  function  of  the  nerve  has 
been  restored.  Since  the  condition  of  the  muscles  plays  such  an  im- 
portant part  in  the  prognosis,  too  much  emphasis  cannot  be  laid  upon 
the  importance  of  persisting  in  the  employment  of  electricity  and 
massage, — i.e.,  such  measures  as  will  tend  to  preserve  muscle  tone. 

As  contraindications  to  the  operation  may  be  mentioned  cases  of 
congenital  facial  palsy,  provided  the  palsy  is  the  result  of  failure  of 
development  of  the  nerve  and  its  muscles  in  the  medulla  oblongata,  and 
cases  of  such  long  duration  that  the  nmscles  are  so  wasted  that  there 
is  little  substance  capable  of  contraction  and  the  nerve  is  so  atrophied 
as  to  render  it  improbable  that  it  could  be  found. 

Technic. — Before  undertaking  the  operation  two  questions  must 
be  considered, — first,  the  selection  of  the  nerve  with  which  the  anasto- 
mosis is  to  be  effected,  and,  second,  the  method  by  which  the  anasto- 
mosis is  to  be  made.  The  facial  being  a  motor  nerve,  one  should  select 
as  its  anastomotic  complement  a  motor  or  a  mixed  nerve.  Two  nerves 
have  been  proposed, — the  spinal  accessory  and  the  hypoglossal.    Of  these 


SUKGERY  OF  FACIAL  NERVE  88Y 

the  hypoglossal  is  to  be  preferred,  chiefly  because  the  disagreeable  com- 
plication of  associated  shoulder  movements  is  avoided  and  because  the 
cortical  centre  of  the  tongue  is  situated  nearer  to  the  facial  centre. 
After  the  facial  nerve  has  been  anastomosed  to  the  spinal  accessory  there 
follows  an  association  of  the  movements  of  the  shoulder  with  those  of  the 
face, — that  is  to  say,  a  voluntary  or  involuntary  contraction  of  the 
muscles  of  the  face  is  accompanied  by  a  contraction  of  the  muscles  of 
the  shoulder,  and  vice  versa.  In  course  of  time  these  once  conjoint 
movements  become  to  a  limited  extent  dissociated, — to  the  extent  that 
the  movements  of  the  face  may  become  independent.  The  patient  may 
exercise  control  over  certain  groups  of  facial  muscles  independently  of 
any  associated  movements  of  the  shoulder,  but,  so  far  as  I  have  been  able 
to  discover  in  the  records  of  cases  hitherto  reported,  there  has  not  been 
a  single  instance  in  which  a  voluntary  effort  to  move  the  shoulder  was 
not  attended  with  contraction  of  the  facial  muscles.  These  so-called 
associated  movements  constitute,  without  doubt,  a  very  serious  objection 
to  the  selection  of  the  spinal  accessory  nerve. 

Another  argument  in  favor  of  the  hypoglossal  nerve  has  to  do  with 
the  relative  positions  of  the  cortical  centres  (Fig.  7).  The  cortical 
centre  of  the  tongue  is  situated  nearer  the  facial  centre  than  that  pre- 
siding over  movements  of  the  shoulder.  Whether  it  is  true  that  disso- 
ciated movements  of  the  face  are  matters  of  education  of  the  newly 
established  cortical  centre  or  that  the  impulses  that  precipitated  those 
movements  originate  in  the  old  facial  centre  or  in  the  tongue  centre,  the 
hypoglossal  nerve  should  be  chosen.  If  the  first  or  "  educational"  theory 
is  correct,  the  hypoglossal  nerve  should  be  preferred  because  the  move- 
ments of  the  tongue  are  much  more  closely  allied  with  those  of  the  face 
than  those  of  the  shoulder,  and  it  is  reasonable  to  infer  that  the  newly- 
selected  cortical  centre  could  for  this  reason  be  more  easily  and  effect- 
ually educated  to  perform  its  new  function;  or  if  the  second  theory 
should  be  proved  the  correct  one, — viz.,  that  the  impulses  must  originate 
in  the  original  facial  centre, — the  tongue  centre  has  the  advantage  over 
the  shoulder  centre  in  that  the  impulses  would  not  have  to  travel  as  far 
from  the  tongue  to  the  facial  centre  as  from  the  shoulder  to  the  tongue 
centre. 

In  this  connection  Spiller  says,  "  In  selecting  the  hypoglossal  nerve 
we  select  one  whose  cortical  centre  is  functionally  more  allied  to  that  of 
the  facial  than  is  the  centre  of  the  spinal  accessory,  or,  let  us  say,  more 
accurately,  than  is  the  centre  for  the  lateral  movement  of  the  head  or 
elevation  of  the  shoulder,  inasmuch  as  movements  and  not  muscles  are 
represented  in  the  cerebral  cortex.    Even  if  associated  movements  persist 


888 


THE    EYE    AND    NERVOUS    SYSTEM 


ill  the  tongue  after  division  of  the  hypoglossal, — and  I  cannot  at  present 
understand  how  they  can  do  so  if  the  motor  supply  of  one  side  of  the 
tongue  is  completely  destroyed, — these  movements  would  be  hidden  from 
observation,  and  would  not  cause  the  annoyance  to  the  patient  produced 
by  spasm  of  the  face  every  time  the  shoulder  or  upper  limb  on  the  same 


5TERNO-MA5TOI0 
an  6 

TRAPEZIUS. 

Fig.  7.— Showing  cortical  origin  of  nerves  supplying  muscles  to  face,  tongue,  and  shoulder.  (From 
Ballance  and  Stewart's  article  on  the  Operative  Treatment  of  Facial  Palsy,  British  Medical  Journal, 
May  2,  1903.) 


side  is  raised,  after  the  spinal  accessory  has  been  employed  for  the 
anastomosis.  Turning  of  the  head  or  raising  the  shoulder  is  only  partly 
controlled  by  the  spinal  accessory,  and  we  can  readily  understand  why 
an  overflow  into  the  anastomosed  facial  nerve  occurs  if  the  movement  in 
which  the  spinal  accessory  normally  has  an  important  i)art  is  produced. 


SUKGERY  OF  FACIAL  NERVE  889 

The  cortical  centre  for  the  spinal  accessory  being  only  a  part  of  the 
centre  for  turning  the  head  or  raising  the  shoulder,  I  doubt  very  much 
whether  this  centre  can  become  so  educated  that  it  can  assume  perfectly 
the  function  normally  exercised  by  the  centre  for  the  movements  of  the 
face." 

Of  less  significance  is  the  question  as  to  whether  the  nerve  should 
be  completely  severed  and  the  movements  of  the  muscles  it  supplied 
sacrificed  or  whether  only  a  portion  of  the  nerve  should  be  used  to  com- 
plete the  anastomosis.  If  the  spinal  accessory  was  severed,  there  would 
be  complete  paralysis  of  the  sternocleidomastoid  and  partial  paralysis 
of  the  trapezius  and  drooping  of  the  shoulder.  Division  of  the  hypo- 
glossal will  be  followed  by  paralysis  of  the  depressors  and  some  of  the 
elevators  of  the  hyoid  bone  and  with  atrophy  of  one-half  the  tongue.  As 
compared  with  the  deformity  which  it  is  hoped  will  be  relieved,  either 
of  these  disturbances  may  be  regarded  as  equally  insignificant.  An  end- 
to-end  anastomosis  should  be  preferred  to  a  lateral  anastomosis,  for  the 
following  reasons :  in  the  first  place,  the  operation  is  easier  of  execution, 
if  one  relies  on  the  entire  nerve-trunk  and  not  a  portion  or  a  branch  with 
which  to  make  the  anastomosis;  second,  because,  if  it  is  true  that  the 
selected  cortical  centre  must  be  educated  to  preside  over  the  facial 
muscles,  the  unlearning  of  its  original  and  the  learning  of  its  new  func- 
tion will  be  facilitated  if  the  connection  is  severed  completely  from  the 
peripheral  muscle  which  it  supplied  originally  and  from  its  accustomed 
centripetal  excitation ;  lastly,  as  Spiller  has  pointed  out,  the  chances  of 
full  restoration  of  power  are  greater  after  end-to-end  anastomosis  because 
the  whole  restorative  force  of  the  central  end  of  the  completely  divided 
nerve  is  directed  into  the  peripheral  end  of  the  facial.^  No  matter  which 
view  of  regeneration  is  accepted,  reunion  of  the  divided  nerves  is  abso- 
lutely essential  to  complete  regeneration.  If,  therefore,  only  a  part  of 
the  healthy  nerve  is  brought  in  contact  with  the  facial,  we  cannot  expect 
so  satisfactory  results  as  when  the  entire  transverse  surface  of  the 
healthy  nerve  is  sutured  to  the  end  of  the  facial  stump. 

Xo  matter  which  nerve  is  selected,  the  operation  is  not  difficult 
(Fig.  8).     The  facial  nerve  is  exposed  by  hugging  closely  the  mastoid 


'  If  from  clinical  observation  it  is  proved  that  restoration  of  function  will  occur 
equally  well  after  a  lateral  anastomosis,  the  latter  operation  may  be  substituted 
for  an  end-to-end  anastomosis,  providing  the  stump  of  the  facial  nerve  is  long  enough 
to  reach  the  hypoglossal  nerve.  Taylor  recommends  that  the  nerve  be  divided  as  far 
up  in  the  canal  as  possible,  in  order  to  render  available  a  longer  section  of  the 
nerve,  and  thus  make  it  possible  to  bring  the  facial  nerve  in  apposition  to  the 
hypoglossal  nerve  without  undue  tension. 


890  THE    EYE    AND    NERVOUS    SYSTEM 

process  and  displacing  the  parotid  gland  forward  and  outward  (Fig. 
9).  The  nerve  will  be  seen  entering  the  substance  of  the  gland  about 
one  centimetre  above  and  one  centimetre  to  the  inner  side  of  the  tip  of 
the  mastoid  process.  If  the  hypoglossal  nerve  has  been  chosen,  it  may 
be  found  crossing  the  external  carotid  artery  just  below  the  point  at 
which  the  occipital  artery  is  given  off.  The  nerve  is  traced,  freed,  and 
finally  divided  at  such  a  distance  to  the  inner  side  of  the  external  carotid 
artery  as  will  enable  the  proximal  end  to  be  brought  in  contact  with  the 
peripheral  stump  of  the  facial  without  undue  tension.  The  point  of 
anastomosis  will  be  upon  the  posterior  belly  of  the  digastric  muscle.   The 


.Facial  Merve. 


t1\jpoqios;alN 
(Xcipiltt! 


.ParoUiGI. 


StprnoClfiio 


^/ 


Dic)a5tri(i1. 


ExLCarohAAf 


FiQ.  9.— Author's  operation  for  the  facio-hypoglossal  anastomosis. 

spinal  accessory  nerve  may  be  found  at  the  junction  of  the  sternocleido- 
mastoid muscle  and  traced  upward.  In  some  instances  the  branch  to  the 
trapezius  has  been  used  instead  of  the  main  trunk. 

In  making  an  end-to-end  anastomosis  three  or  four  sutures  will  suffice 
to  effect  apposition.  These  should  be  introduced  only  through  the  sheaths 
of  the  respective  nerves,  using  for  this  purpose  the  finest  curved  needles 
and  silk.  In  lateral  or  end-to-side  anastomosis,  either  the  end  of  the 
divided  facial  may  be  buried  within  the  fibres  of  the  hypoglossal  or 
spinal  accessory  and  secured  in  place  by  sutures,  or,  as  recommended  by 
Ballance  and  Stewart,  a  longitudinal  incision  may  be  made  in  the  sheath 


PLATE  C. 


Author's  operation  for  anastomosis  of  the  facial  with  the  hypoglossal  nerve. 
1.  Stumpof  the  central  segment  of  the  facial  nerve;  2.  Spinal  accessory  nerve ;  3.  Reflected  portion 
of  the  hypoglossal  nerve ;  4.  Descendens  noni ;  5.  Occipital  artery ;  6.  Internal  carotid ;  7.  Parotid 
gland  drawn  forwards  with  a  retractor ;  8.  Digastric  muscle  ;  9.  Course  of  hypoglossal  nerve  before  it 
was  reflected  ;  10.  External  carotid  arterv. 


SUKGERY  OF  FACIAL  NERVE 


891 


of  both  nerves  sufficient  to  expose  the  fibres,  and  the  sutures  so  intro- 
duced through  the  sheaths  of  each  nerve  as  to  bring  the  two  surfaces 
of  exposed  nerve-fibres  into  apposition. 

Clark's  method  effects  an  anastomosis  by  embedding  the  pointed 
end  of  the  facial  nerve  into  the  substance  of  the  hypoglossal  and  securing 
it  there  with  sutures  (Fig.  10). 

It  is  needless  to  say  that  with  each  of  these  methods  success  depends 
upon  perfect  union  of  the  nerve,  and  this  can  only  be  obtained  by  proper 
coaptation  and  by  providing  every  condition  which  will  favor  uncompli- 
cated repair  of  the  wound.  Delicacy  of  manipulation,  use  of  the  mini- 
mum number  of  sutures,  perfect  aj^position,  and  complete  asepsis  may  be 
considered  absolutely  essential  to  ultimate  success. 


Fig.  10.— Clark's  method  of  lateral  anastomosis :  1,  facial  nerve ;  2,  styloid  process ;  3,  internal 
jugular  vein ;  4,  hypoglossal  nerve ;  5,  pneumogastric  nerve ;  6,  posterior  belly  of  digastric  muscle ;  7, 
transverse  process  of  atlas ;  8,  occipital  artery ;  9,  sterno-mastoid  muscle ;  10,  parotid  gland ;  11,  mastoid 
process. 


Results. — There  may  be  said  to  be  three  grades  or  degrees  of  im- 
provement, leading  to  complete  recovery,  which  may  be  anticipated  after 
the  performance  of  this  operation.  The  first  and  the  least  degree  of 
improvement  which  could  be  expected  is  the  restoration  of  muscular 
tone :  the  muscles  are  no  longer  flaccid ;  the  face  in  repose  is  sym- 
metrical,— not,  as  before,  asymmetrical.  If  no  greater  improvement  was 
anticipated,  the  prospect  of  obliterating  the  deformity  in  repose  should 
settle  any  doubt  in  the  mind  of  the  patient  as  to  the  advisability  of 
undergoing  or  in  the  mind  of  the  surgeon  as  to  the  advisability  of  recom- 
mending the  operation.  The  second  stage  or  grade  of  improvement,  and 
one  which  has  been  obtained  in  a  majority  of  cases,  is  the  voluntary 
control  of  individual  muscles  or  groups  of  muscles,  such  as  those  con- 


892  THE    EYE    AND    NERVOUS    SYSTEM 

cerned  in  raising  the  eyebrows,  in  closing  the  lids,  in  puckering  the  lips, 
or  in  whistling.  Such  restoration  is  a  very  material  gain,  for  not  only  is 
the  patient's  face  symmetrical  in  repose  but  in  action  as  well.  The  third 
or  last  grade,  the  attainment  of  which  signifies  complete  recovery,  is  the 
return  of  the  expressional  or  emotional  movements,  as  laughing,  crying, 
etc.  The  mechanism  of  these  movements  is  much  more  complicated  and 
a  matter  of  delicate  co-ordination.  This  is  the  ideal  result,  but  one 
which  has  as  yet  not  been  obtained.  Symmetry  of  face  in  repose, 
symmetry  of  the  face  in  certain  uncomplicated  movements,  and,  lastly, 
symmetry  in  the  more  elaborate  movements,  as  those  of  expression — 
these  are  the  improvements  aimed  at  in  the  surgical  treatment  of  facial 
palsy.     (For  further  remarks  on  facial  palsy  see  Chapter  VII,  p.  332.) 

VISUAL    DISTURBANCE    DUE   TO    INTERNAL    HYDROCEPHALUS. 

The  acquired  or  secondary  form  of  hydrocephalus  is  very  frequently 
associated  with  disturbance  of  vision ;  in  fact,  optic  neuritis  and  its 
results,  choked  disk  and  atrophy,  are  almost  invariably  present.  Bitem- 
poral hemianopsia  may  be  present,  too,  as  a  result  of  the  pressure  upon 
the  middle  portion  of  the  optic  chiasm  exerted  by  the  distended  third 
ventricle.  These  conditions  may  be  relieved  only  by  the  withdrawal  of 
cerebrospinal  fluid.  To  this  end  one  of  two  methods  may  be  resorted 
to, — Quincke's  lumbar  puncture  and  puncture  of  the  lateral  ventricles 

1.  Lumbar  Puncture. — Of  the  two  operations  lumbar  puncture  is 
the  safer  procedure,  although  the  fatal  tendencies  of  both  are  consider- 
able. Gumprecht  collected  fifteen  cases  of  sudden  death  after  lumbar 
puncture  in  cases  of  cerebral  tumors,  and  of  eight  cases  of  lumbar  punc- 
ture employed  in  cerebellar  tumors  four  died  within  a  few  days.  The 
sudden  disturbance  of  pressure  is  no  doubt  responsible  for  the  large 
majority  of  fatalities.  Furbinger  attributes  death  to  pressure  exerted 
upon  the  bulb  by  the  arrest  of  cerebrospinal  fluid  from  the  ventricles 
at  the  foramen  of  Magendie,  and  it  is  no  doubt  especially  dangerous 
when  the  eoniniunication  between  the  lateral  ventricles  and  subarach- 
noid space  and  the  canal  of  the  spinal  cord  is  interrupted.  Under  these 
conditions  the  equilibrium  between  the  cerebral  and  spinal  fluid  is 
disturbed ;  the  medulla  and  pons  are  drawn  forcibly  downward  into  the 
foramen  magnum,  with  resulting  traumatism,  followed  by  serious  if  not 
fatal  consequences.  When  the  communication  with  the  lateral  ventricles 
is  cut  off,  lumbar  puncture  will,  of  course,  be  ineffective.  The  results  of 
this  treatment  for  the  relief  of  chronic  hydrocephalus  are  on  the  whole 
unsatisfactory.  The  condition  may  be  ameliorated  temporarily  by  the 
relief  of  pressure,  but  only  in  the  rarest  instances  will  the  results  be 


IXTERXAL    HYDROCEPHALUS  893 

permanent.  GoAvers,  for  example,  secured  a  permanent  result  in  a  case 
of  serous  meningitis  of  the  chronic  type ;  the  headache,  vomiting,  ataxia, 
and  paralysis  of  the  ocular  muscles  completely  disappeared,  and  the 
optic  neuritis  subsided.  Oppenheim  recommends  lumbar  puncture  for 
those  cases  in  which  the  tumor  is  associated  with  an  internal  hydro- 
cephalus and  especially  when  the  tumor  encroaches  upon  the  posterior 
fossa  and  threatens  the  life  of  the  patient.  To  withdraw  fluid  from  the 
lower  portion  of  the  dural  sac  a  hollow  needle  from  two  and  a  half  to 
three  inches  long,  armed  with  a  stilet,  is  introduced  between  the  second 
and  third  lumbar  vertebrae.  With  the  patient  in  the  recumbent  posture 
and  the  back  strongly  arched,  the  needle  is  introduced  at  a  point  about 
five  millimetres  to  the  right  of  the  median  line,  on  a  level  with  the  space 
between  the  second  and  third  spinous  processes.  The  needle  is  directed 
slightly  inward  and  upward  until  the  dural  canal  is  reached.  It  is  not 
necessary  to  aspirate  the  fluid,  as  there  is  enough  pressure  in  the  canal 
to  force  the  fluid  out.  In  children  the  needle  may  be  introduced  exactly 
in  the  median  line  just  below  the  spinous  process  of  the  second  lumbar 
vertebra.  Whenever  it  is  desirable  to  determine  the  pressure  under 
which  the  cerebrospinal  fluid  exists,  a  U-shaped  manometer  may  be 
attached  by  a  rubber  tube  to  the  needle.  The  manometer  may  be  used 
to  advantage  in  all  cases  as  a  guide  to  the  amount  of  fluid  which  can 
with  safety  be  withdrawn.  A  pressure  of  twenty  millimetres  of  water 
should  be  preserved. 

2.  Ventricular  Puncture. — Puncture  of  one  of  the  lateral  ventricles 
has  been  performed  for  the  relief  of  hydrocephalus,  but,  as  in  the  case  of 
lumbar  puncture,  the  effect  will  be  only  palliative.  This  is  an  operation 
which  is,  unfortunately,  of  unusual  gravity,  and  the  danger  attending  it 
is  so  great,  in  comparison  to  the  possible  benefit,  as  to  make  it  a  pro- 
cedure of  questionable  propriety.  Many  cases  have  been  reported  in 
which  the  results  were  disastrous.  So  far  as  I  have  been  able  to  find, 
there  has  been  but  one  instance  in  which  the  treatment  was  successful. 
The  lateral  ventricle  may  be  entered  from  the  frontal  region,  as  recom- 
mended by  von  Bergmann,  or  from  the  parietal  region,  as  recommended 
by  Keen.  By  the  former  method  a  small  longitudinal  incision  is  made  a 
little  in  front  and  to  the  inner  side  of  the  tuberosity  of  the  frontal  bone. 
A  hole  is  bored  through  the  skull  and  a  long,  hollow  needle  is  introduced 
in  a  direction  from  before  backward  and  a  little  downward  and  inward 
and  pushed  steadily  into  the  brain  tissue  until  the  fluid  escapes.  In 
Keen's  method  the  needle  is  introduced  thirty-two  millimetres  above  a 
line  connecting  the  supraorbital  edge  with  the  external  occipital  protub- 
erance and  thirtv-two  millimetres  behincl  the  external  auditorv  meatus. 


894  THE    EYE    AND    NERVOUS    SYSTEM 


SURGERY  OF  THE  FIFTH  NERVE;  TRIFACIAL 
NEURALGIA;  TIC  DOULOUREUX. 

In  the  vast  majority  of  cases  tic  douloureux  is  a  disease  of  the  second 
and  third  divisions.  In  many  cases  the  pain  appears  first  in  the  distri- 
bution of  the  third  division  and  may  remain  so  for  a  long  time  before 
spreading  to  the  branches  of  the  second  division.  Of  these  two  divisions 
the  inferior  dental  and  the  infraorbital  nerves  are  the  branches  which 
are  almost  invariably  involved.  In  exceptional  cases  the  neuralgia  may 
begin  in  the  first  or  ophthalmic  division  and  never  extend  to  the  other 
branches ;  on  the  other  hand,  the  pain  may  be  referred  to  the  branches 
of  all  three  divisions.  In  addition  to  the  neuralgic  pains  we  find  in  some 
cases  certain  points  ("  tender  points")  along  the  course  of  the  nerve 
which  are  sensitive  to  pressure.  Thus,  when  the  first  division  is  involved, 
the  "  tender  points"  will  be  found  at  the  supraorbital  notch,  in  the  outer 
part  of  the  upper  eyelid,  and  at  the  emergence  of  the  nasal  branch  at  the 
lower  edge  of  the  nasal  bone ;  the  pain  radiates  over  the  anterior  half  of 
the  head,  to  the  eyelid  and  eye  itself,  and  to  half  of  the  nose ;  the  second 
division  gives  off  a  few  branches  as  it  traverses  the  sphenomaxillary 
fossa  and  appears  at  the  infraorbital  foramen  as  the  infraorbital  nerve, 
where  it  divides  into  the  palpebral,  nasal,  and  labial  branches.  AVhen 
this  division  is  involved,  the  "  tender  points"  will  be  found  at  the  infra- 
orbital foramen,  at  the  side  of  the  nose,  and  over  the  most  prominent 
portions  of  the  malar  bone.  If  the  dental  branches  are  involved,  pain 
will  be  referred  also  to  the  teeth  and  gums  of  the  upper  jaw.  Finally, 
if  the  third  division  is  involved,  pain  will  be  referred  to  the  distribution 
of  one  or  all  of  its  sensory  branches ;  to  the  parietal  eminence,  temple, 
and  external  ear  (temporal  and  auricular  temporal  branch),  to  the 
tongue  (lingual),  to  the  gums  and  teeth  of  the  lower  jaw  (inferior  dental 
branch),  and  to  the  lower  lip  (mental  branch).  The  "  tender  points"  are 
at  the  inferior  dental  foramen,  where  the  auriculo-temporal  crosses  the 
zygoma,  over  the  parietal  eminence,  in  the  tongue,  and  at  the  mental 
foramen. 

The  character  and  intensity  of  the  pain  are  very  variable.  As  in 
neuralgias  of  other  nerves,  the  pain  is  spontaneous  and  paroxysmal ;  a 
series  of  successive  pains  constitute  a  paroxysm  and  several  paroxysms 
an  attack.  The  pain  is  variously  described  as  darting,  stabbing,  sharp, 
pricking,  tearing,  and  boring;  as  to  intensity,  the  pains  vary  from  a 
slight  burning  sensation  to  one  of  excruciating  severity.  The  attacks  are 
sometimes  initiated  by  premonitory  signs,  such  as  a  feeling  of  tension, 
itching,  or  a  feeling  of  unrest  in  the  facial  muscles,  and  they  are  pre- 


SURGERY   OF   TRIGEMINAL   NERVE  895 

cipitated  in  some  instances  by  a  draught  or  exposure,  by  talking,  chew- 
ing, swallowing,  by  fatigue  and  emotion.  There  is  the  widest  variation 
in  the  frequency  with  which  attacks  recur :  there  may  be  an  interval  of 
only  a  few  hours,  or  several  months;  but  usually  as  time  goes  on  the 
attacks  increase  both  in  frequency  and  severity.  There  can  be  said  to  be 
no  periodicity  except  in  those  cases  in  which  the  neuralgia  is  of  malig- 
nant origin  or  in  which  the  attacks  occur  only  during  the  catamenial 
periods.     (Eor  further  remarks  on  fifth  nerve  see  Chap.  VII,  p.  320.) 

Concomitant  Symptoms. — Associated  with  the  neuralgic  attacks 
there  may  be  signs  of  irritation  of  the  secretory,  trophic,  and  vasomotor 
branches  as  well  as  of  the  motor  root  and  the  facial  nerve.  Thus,  one 
may  see  lachrymation,  flushing,  or  sweating  (vasomotor  disturbances)  ; 
loss  of  hair  and  hair-pigment,  and  herpes  (trophic  disturbances)  ;  con- 
traction of  masseter  muscles  (motor  root),  and  convulsive  movements 
of  the  face  (facial  nerve).  The  pulse  is  sometimes  accelerated,  some- 
times excited  and  irritable. 

Pathology. — In  order  to  comprehend  the  various  clinical  manifesta- 
tions of  trifacial  neuralgia  and  the  rationale  upon  which  the  indications 
for  treatment  are  based,  one  should  be  familiar  with  at  least  certain 
elementary  facts  relating  to  the  patholog;)^  of  the  affection.  There  may 
be  said  to  be  two  or  three  separate  and  distinct  types  of  tic  douloureux ; 
one  in  which  the  lesion  is  primarily  a  neuritis  of  the  peripheral 
branches,  which  in  course  of  time  may  or  may  not  extend  to  and  involve 
the  ganglion ;  another  in  which  the  ganglion  is  affected  primarily ;  and 
a  third,  perhaps,  in  which  the  lesion  is  neither  in  the  ganglion  nor  in  its 
branches,  but  in  the  central  nervous  system.  In  some  instances  the 
process  is  a  degeneration,  in  others  an  interstitial  neuritis,  or  the  primary 
lesion  may  be  a  neoplasm.  Were  it  possible  to  determine  by  the  clinical 
manifestations  whether  the  lesion  were  central  or  peripheral,  the  indica- 
tions for  treatment  would  be  more  clearly  defined.  Sometimes,  how- 
ever, it  is  possible.  According  to  Wagner  it  may  be  assumed  that  a 
neuralgia  is  of  cerebral  origin  providing  only  several  branches  are  in- 
volved, and  providing,  too,  that  in  the  other  cranial  nerves  there  is  fur- 
ther evidence  of  cerebral  disease. 

Prognosis. — Trifacial  neuralgia  is  of  itself  not  a  fatal  disease, 
although  if  unrelieved  the  patient  may  from  the  pain  and  suffering 
become  exhausted  and  more  easily  fall  a  prey  to  some  intercurrent 
affection.  In  some  instances  the  disease  may  be  said  to  be  self-limited 
and  without  any  operative  intervention  may  gradually  disappear.  This 
is  more  especially  true  of  the  so-called  migrainous  type,  which  is 
observed  in  women  in  early  life  suffering  from  migraine.    The  prognosis 


890  THE    EYE    AND    NERVOUS    SYSTEM 

in  the  minor  cases  is  naturally  better  than  in  those  of  the  major  or  more 
severe  variety. 

Classification — Cases  of  trifacial  neuralgia  ma.y  be  classified,  first, 
according  to  whether  the  lesion  is  peripheral,  ganglionic  or  central ;  sec- 
ond, according  to  the  distribution  of  the  pain,  whether  in  the  distribu- 
tion of  the  first,  second,  or  third  divisions,  in  one,  two  or  in  all  three 
divisions;  and,  third,  as  suggested  by  Dana,  into  two  classes,  one  the 
migrainous  type,  occurring  almost  always  in  women  in  early  life,  who 
have  been  subject  to  migraine;  after  a  number  of  years  it  becomes 
chronic  and  more  localized.  Dana  considers  this  type  to  be  an  evolution 
of  a  definite  trigeminal  neuralgia  on  the  basis  of  a  migrainous  neurosis. 
In  the  beginning  the  disorder  is  central,  neither  in  the  Gasserian 
ganglion  nor  its  branches,  but  later  certain  degenerative  changes  may 
develop  in  the  peripheral  branches.  The  second  type  of  Dana's  classifi- 
cation, and  the  more  common  of  the  two,  affecting  men  as  well  as  women, 
occurs  after  middle  age,  and  is  due  to  a  definite  lesion  either  in  the 
ganglion  or  in  its  branches.  Petres  attempted  to  determine  the  seat  of 
the  lesion  by  noting  the  effect  of  an  injection  of  a  2  per  cent,  solution  of 
cocaine.  If  the  injection  into  a  painful  area  stops  the  neuralgic  crisis 
for  a  time  the  lesion  is  presumably  peripheral;  if,  on  the  other  hand, 
the  injection  does  not  modify  the  attack  the  cause  is  higher  up,  either  in 
the  root  or  the  ganglion.  The  deductions  will  be  of  value  only  when  the 
injections  are  made  between  the  paroxysms.  If  the  paroxysms  do  not 
recur  until  the  cocaine  anaesthesia  vanishes,  it  is  justifiable  to  assume 
that  the  cocaine  has  acted  in  a  positive  manner. 

If  the  surgeon  had  some  means  of  determining  whether  the  pain  is  of 
peripheral,  ganglionic,  or  central  origin,  he  would  at  once  be  able  to 
decide  as  to  whether  in  a  given  case  an  extracranial  or  an  intracranial 
operation  were  called  for.  As  yet  no  means,  except  Petres's  method, 
have  been  suggested  whereby  such  a  differential  diagnosis  may  be  made. 

Treatment. — The  treatment  may  be  medicinal  or  operative,  but 
before  proceeding  with  any  definite  course  of  treatment,  one  should  seek 
for  a  cause,  and,  if  possible,  remove  it.  Particularly  in  those  cases  which 
are  of  peripheral  origin  may  we  hope  to  effect  a  cure  by  removing  the 
source  of  irritation ;  it  may  be  a  carious  tooth,  a  splinter  of  bone,  a 
foreign  body,  a  tumor,  catarrhal  conditions  of  the  antrum,  the  ethmoidal 
or  the  frontal  sinuses.  At  the  onset,  at  least,  the  treatment  of  all  cases 
of  trigeminal  neuralgia  belongs  to  the  domain  of  medicine  rather  than 
surgery.  Clinical  experience  teaches  us  that  in  20  per  cent,  of  cases  the 
disease  may  be  arrested  by  medicinal  measures,  if  not  permanently,  at 
least  for  long  intervals.    One  must  remember  also  that  in  some  instances 


SURGEEY   OF   TRIGEMINAL   KERYE  897 

the  disease  has  a  tendency  to  run  its  course  in  five  or  six  years  and  then 
exhibits  a  tendency  to  spontaneous  cure.  The  drug  which  is  par  excel- 
lence the  most  efficacious,  especially  in  the  anaemic  and  exhausted,  is 
strychnia.  In  cases  of  but  one  or  two  years'  standing  strychnia  prop- 
erly administered  will  arrest  or  control  the  disease  almost  invariably.  To 
obtain  the  best  results  the  drug  must  be  administered  in  heroic  doses,  and 
the  patient  should  be  kept  under  the  closest  observation,  and  should  be 
confined  to  bed.  The  remedy  is  administered  hypodermically  once  daily, 
in  ascending  doses,  until,  at  the  expiration  of  two  weeks,  the  physio- 
logical limit  is  reached.  Beginning  with  one-thirtieth  grain  the  dose 
may  be  increased  to  one-fourth  to  one-half  grain,  and  when  the  maximum 
dose  is  reached  it  should  not  be  given  oftener  than  on  alternate  days. 
Quinine,  arsenic,  potassium  iodide,  and  aconitine  nitrate  have  been 
recommended,  but  not  one  of  these  drugs  compares  in  efficacy  with 
strychnia. 

Extracranial  Operations. — These  operations  include  the  so-called  pe- 
ripheral operations  and  those  which  attack  the  nerves  at  their  exit  from 
their  respective  foramina  at  the  base  of  the  skull.  As  to  the  indications 
for  this  class  of  operations  the  general  statement  may  be  made  that  in 
€very  case  in  which  the  pain  seems  to  be  confined  to  a  branch  of  one  of 
the  three  divisions  of  the  ganglion  a  peripheral  operation  is  called  for. 
The  simplicity  of  such  a  procedure,  as  compared  with  an  operation  at 
the  base  of  the  skull,  whether  extra-  or  intracranial,  should  deter  us  from 
subjecting  the  patient  to  the  additional  risk  of  more  serious  procedures, 
«ven  taking  into  consideration  the  fact  that  there  may  be  a  recurrence. 
The  tendency  of  the  peripheral  nerves  to  regenerate  after  a  neurotomy 
or  even  a  partial  neurectomy  should  be  combated  by  an  extensive  extir- 
pation of  the  nerves,  thus  reducing  to  a  minimum  the  chances  of  recur- 
Tence. 

Given  a  case  in  which  the  pain  may  be  referred  to  more  than  one 
l)ranch  of  but  one  division,  it  is  proper  under  such  circumstances  to 
resort  to  an  operation  upon  the  nerve  trunk  immediately  after  its  exit 
from  the  foramen.  For  example,  in  neuralgia  of  the  second  division,  a 
neurectomy  just  above  Meckel's  ganglion  would  be  called  for.  For  those 
<3ases,  however,  in  which  at  least  two  of  the  three  divisions  are  believed 
to  be  involved  one  or  the  other  of  the  intracranial  operations  should  be 
resorted  to. 

Peripheral  Operations. — At  one  time  it  was  tliought  sufficient  simply 

to  expose  and  divide  the  nerve.     Owing  to  the  natural  tendency  of  the 

nerve  to  regenerate,  recurrences  were  frequent.     At  the  present  time  a 

peripheral  operation  should  not  be  considered  properly  executed  unless 

57 


898  THE    EYE    AND    NERVOUS    SYSTEM 

either  by  excision  or  avulsion  a  considerable  section  of  nerve  is  removed. 
The  longer  the  section  removed  the  less  the  likelihood  of  regeneration, 
and,  therefore,  of  recurrence. 

With  this  idea  Thiersch  devised  a  method  of  forcible  extraction, 
which  consisted  in  grasping  the  nerve  with  forceps  and  making  gradual 
traction  upon  the  nerve  both  centrally  and  peripherally  by  turning  the 
forceps  on  their  own  axis.  For  this  purpose  the  blades  of  the  forceps 
are  so  constructed  that  they  do  not  cut  through  the  nerve-fibres.  One 
blade  of  the  forceps  is  concave,  one  convex,  and  both  are  ribbed  trans- 
versely or  longitudinally,  care  being  taken  not  to  have  any  sharp  edges. 
After  the  nerve  is  dissected  free  from  its  surroundings  it  is  grasped  at 
right  angles  to  its  axis.  Traction  is  made  very  slowly,  rot  making  more 
than  one-half  turn  per  second.  By  this  method  not  only  may  a  greater 
portion  of  the  peripheral  segment  even  to  its  small  terminal  filaments 
be  extracted,  but  the  central  segment  of  the  nerve  up  to  the  ganglion. 

According  to  van  Gehuchten  avulsion  or  the  forcible  extraction  of 
the  peripheral  nerves  will  be  followed  by  degeneration  not  only  in  the 
ganglionic  cells  but  also  in  the  bulbospinal  root.  If  this  result  was  con- 
stant, restoration  of  function  and  recurrences  of  the  pain  would  be 
impossible.  Thus  far,  with  the  exception  of  a  few  cases,  the  results  are 
based  upon  animal  experimentation,  but  they  are  sufficiently  positive  to 
warrant  the  adoption  of  Thiersch  and  van  Gehuchten's  suggestion  in  all 
peripheral  operations. 

Exposure  of  the  Supraorbital  Nerve. — To  reach  this  nerve  an  in- 
cision is  made  parallel  with  the  superior  margin  of  the  orbit  for  an  equal 
distance  on  either  side  of  the  supraorbital  foramen.  The  periosteum  is 
divided  and  the  nerve  will  be  exposed  to  view  and  should  be  traced  back 
to  a  point  behind  that  at  which  the  supratrochlear  branch  is  given  off. 

Exposure  of  the  Infraorbital  Nerve. — The  infraorbital  foramen 
from  which  the  nerve  makes  its  exit  is  situated  about  one  centimetre 
below  the  inferior  margin  of  the  orbit  and  about  the  junction  of  its 
middle  and  inner  third.  A  line  drawn  from  the  supraorbital  notch 
downward  between  the  two  lower  bicuspid  teeth  intersects  tlie  infra- 
orbital and  mental  foramina  (Keen).  The  foramen  may  be  exposed  by 
a  short  incision  down  through  the  skin,  subcutaneous  fat  and  the  attach- 
ment of  the  levator  labii  superioris.  The  periosteum  is  divided  and 
separated  below  sufficiently  to  expose  the  plexus  and  above  sufficiently 
to  expose  the  floor  of  the  orbit.  The  roof  of  the  canal  is  so  thin  that  it 
is  easily  broken  open  with  a  groove  director.  The  nerve  is  now  grasped 
with  forceps  and  by  means  of  traction  or  with  the  aid  of  the  scissors 
resected  back  as  far  as  the  sphenomaxillary  fissure. 


SUKGERY  OF   TRIGEMINAL   NERVE  899 

Exposure  of  the  Inferior  Dental  Nerve. — The  inferior  dental  nerve 
enters  the  canal  at  a  point  about  three  centimetres  above  the  angle  of  the 
jaw.  In  order  to  avoid  injuring  the  branches  of  the  facial  nerve,  or 
Steno's  duct,  and  to  give  the  best  cosmetic  results,  the  incision  should  be 
made  just  beneath  the  lower  border  of  the  jaw.  A  musculo-periosteal 
flap,  including  the  masseter,  is  reflected  upwards  and  the  canal  opened 
with  a  chisel  or  trephine.  In  order  to  guard  against  regeneration  the 
nerve  should  be  resected  as  far  forward  as  the  mental  foramen  or 
forcibly  extracted  by  the  Thiersch  method.  As  a  further  safeguard 
against  recurrence  the  author  plugs  the  central  opening  of  the  canal  with 
some  impermeable  substance,  as  silver  foil. 

Extracranial  Operation  at  the  Base  of  the  Shull. — These  operations 
are  reserved  for  those  cases  in  which  recurrence  of  pain  has  followed 
one  or  more  peripheral  operations  upon  the  br*anches  of  the  second  or 
third  division,  as  infraorbital  or  inferior  dental  nerves,  and  especially 
when  pain  is  referred  to  the  entire  area  of  one  or  the  other  of  these  two 
divisions.  If  both  divisions  are  involved,  an  intracranial  operation 
should  be  performed. 

Second  Division. — To  reach  the  second  division  at  the  base  of  the 
skull  one  must  enter  the  sphenomaxillary  fossa  by  traversing  the  antrum 
(Carnochan's  method)  or  by  a  resection  of  the  malar  bone  (Liicke's 
method).  Neither  of  these  operations  is  difficult  of  execution,  although 
the  latter  is  to  be  preferred;  it  affords  more  readily  a  better  exposure 
and  avoids  the  possibility  of  wound-contamination  from  the  antrum  of 
Highmore. 

Method  of  LiXche. — Beginning  one  centimetre  above  the  outer  can- 
thus  and  two-thirds  millimetre  from  the  external  orbital  margin  an  ob- 
lique incision  is  made,  terminating  at  a  point  corresponding  to  the  posi- 
tion of  the  upper  third  molar.  At  right  angles  to  the  first  and  starting  at 
its  upper  end  a  second  incision  is  made  so  as  to  expose  the  zygomatic 
process  of  the  temporal  bone.  The  malar  bone  is  divided  in  the  line  of 
the  first  incision  with  a  chain  or  wire  saw  and  the  zygomatic  arch  is  cut 
through  at  and  in  front  of  the  articular  tubercle.  The  osteoplastic  flap 
thus  formed,  consisting  of  skin,  zygomatic  arch,  and  masseter,  is  now 
reflected.  Following  the  posterior  surface  of  the  upper  jaw  the  fat  and 
plexus  of  veins  are  displaced  backwards;  in  this  way  both  the  venous 
plexus  and  the  internal  maxillary  artery  may  be  protected  from  injury. 
Pressing  on  now,  in  the  direction  of  the  sphenomaxillary  fossa,  and 
removing  as  much  as  may  be  necessary  to  afford  adequate  exposure,  the 
operator  will  reach  the  superior  maxillary  nerve  at  a  depth  of  about  six 
centimetres  in  its  passage  from  the  foramen  rotundum  across  the  spheno-^ 


900  THE    EYE    AND    NERVOUS    SYSTEM 

maxillary  fossa  to  the  infraorbital  canal.  Before  proceeding  to  extract 
the  nerve  it  must  be  dissected  free  from  the  infraorbital  artery.  It  is 
then  grasped  with  a  pair  of  forceps,  divided  close  to  the  foramen,  and 
its  peripheral  portion  extracted  by  the  Thiersch  method.  Friedlander 
resects  the  entire  malar  bone,  and,  while  a  better  view  is  afforded,  the 
operation  is  more  radical,  and  there  is  always  the  possibility  of  opening 
the  antrum  of  Highmore. 

Chavasse's  Modification  of  Camochan's  Method. — A  horizontal  in- 
cision is  made  below  the  eye  and  from  the  centre  of  this  a  vertical 
incision  is  dropped,  exposing  the  infraorbital  nerve.  An  opening  an 
inch  in  diameter  is  made  with  trephine  or  chisel  in  the  anterior  wall  of 
the  antrum  and  in  the  posterior  wall  a  sorfiewhat  smaller  one  is  similarly 
made.  The  nerve  is  grasped  with  a  pair  of  forceps  and  divided  at  the 
infraorbital  foramen;  by  breaking  through  the  floor  of  the  orbit  the 
nerve  may  now  be  traced  through  the  antrum  and  sphenomaxillary  fossa 
back  to  the  foramen  rotundum,  where  it  is  divided  with  curved  scissors. 
Precautions  must  be  taken  not  to  injure  the  internal  maxillary  artery 
which  lies  in  close  relation  with  the  posterior  wall  of  the  antrum. 

Third  Division. — This  division,  with  all  its  branches,  the  lingual, 
inferior  dental,  buccinator,  and  auriculo-temporal  nerves,  may  be  ex- 
posed by  Kronlein's  method.  Beginning  one  centimetre  from  the  angle 
of  the  mouth  an  incision  is  made  to  a  point  one  centimetre  in  front  of 
the  lobule  of  the  ear.  The  buccinator  muscle  is  left  undisturbed,  but  the 
incision  is  carried  through  that  portion  of  the  masseter  not  covered  by 
the  parotid.  Steno's  duct  and  the  branches  of  the  facial  nerve  should 
not  be  injured.  After  separating  the  attachments  of  the  masseter  and 
internal  pterygoid  muscles  from  the  coronoid  process  the  latter  is  divided 
obliquely  forward  and  downward  and  as  low  down  as  possible.  The 
osteoplastic  flap  composed  of  the  resected  coronoid  process  and  the 
temporal  branches  is  reflected  and  the  inferior  dental  nerve  is  isolated 
and  traced  back  to  the  foramen  rotundum,  where  the  main  trunk  is 
divided.  To  enable  one  to  isolate  the  nerve  it  may  be  necessary  to 
dissect  away  the  fatty  tissue  of  the  cheek,  anterior  to  the  masseter 
muscle,  as  well  as  the  more  deep-seated  fat  in  relation  with  the  lateral 
aspect  of  the  internal  pterygoid  muscle. 

Intracranial  Operations. — Division  of  the  Sensory  Root  of  the  Gas- 
serian  Ganglion;  Physiological  Extirpation  of  the  Ganglion. — Upon 
the  assumption  that  roots  of  the  central  nervous  system,  when  divided, 
do  not  undergo  regeneration,  Spiller,  in  1899,  proposed  division  of  the 
sensory  root  as  a  substitute  for  excision  of  the  ganglion  for  the  treatment 
of  trifacial  neuralgia.     Before  putting  the  idea  into  practice  upon  the 


SURGERY  OF  TRIGEMINAL  NERVE      901 

human  subject  a  series  of  experiments  were  conducted  by  Spiller  and 
the  author  upon  dogs,  from  which  it  was  concluded  that  it  has  yet  to  be 
proven  that  regeneration  of  the  central  roots  can  occur.  With  the  assur- 
ance derived  from  this  experimental  evidence  the  first  operation  was 
conducted  upon  the  human  subject  in  1901.  In  tliis  case  the  symp- 
toms were  relieved,  and  there  has  been  no  recurrence.  Since  then  the 
operation  has  been  practised  a  number  of  times  with  equally  gratify- 
ing results. 

This  operation  was  proposed  by  Spiller  with  the  hope  that  it  would 
be  found  to  be  easier  of  execution  than  excision  of  the  ganglion,  and 
from  our  clinical  experience  it  may  be  said  that  this  expectation  has 
been  fully  realized.  The  advantages  of  this  procedure  over  any  other 
having  for  its  object  the  removal  of  the  ganglion  may  be  summed  up  as 
follows :  ( 1 )  It  is  easier  of  execution.  The  most  difficult  step  of  the 
operation  for  excision  of  the  ganglion  is  not  undertaken  until  the  attempt 
is  made  to  free  the  ganglion  from  its  base.  To  divide  the  sensory  root 
the  ganglion  is  left  undisturbed  once  the  dura  propria  is  reflected  from 
its  superior  aspect.  All  that  remains  to  complete  the  operation  is  the 
division  of  the  root ;  thus  before  the  most  difficult  stage  of  one  opera- 
tion is  even  approached  the  other  operation  is  finished.  (2)  There  is  less 
hemorrhage.  The  most  annoying  feature  of  these  operations  as  a  class 
is  hemorrhage,  and  the  most  troublesome  hemorrhage  begins  when  one 
starts  to  elevate  the  base  of  the  ganglion,  since  the  ganglion  receives  its 
largest  blood  supply  from  vessels  at  the  base.  In  division  of  the  sensory 
root  hemorrhage  from  this  source  does  not  occur  because  the  base  of  the 
ganglion  is  not  disturbed.  (3)  The  adjacent  structures  are  not  exposed 
to  injury.  The  proximity  of  the  cavernous  sinus,  and  of  the  third,  fourth 
and  sixth  nerves  to  the  inner  aspect  of  the  ganglion  exposes  them  to  in- 
jury whenever  the  attempt  is  made  to  extract  the  ganglion ;  in  point  of 
fact  the  cavernous  sinus  has  been  frequently  torn  and  one  of  the  nerves 
divided  in  extraction  of  the  ganglion.  (4)  Ocular  disturbances,  such 
as  corneal  ulceration,  are  less  likely  to  occur.  This  is  probably  due  to  the 
fact  that  the  sympathetic  fibres,  which  pass  through  the  ganglion,  exercise 
a  trophic  influence  on  the  eye.  In  division  of  the  sensory  root  these  sym- 
pathetic fibres  are  not  disturbed.'  (5)  The  operation  is  easier  of  execu- 
tion. (6)  It  should  be  attended  with  a  lower  mortality.  It  may  be 
assumed  that  of  two  operations  the  safer  would  be  the  one  in  which 
there  is  less  hemorrhage,  fewer  technical  difficulties,  and,  therefore, 
greater  economy  of  time. 

Technic. — The  technic  of  the  operation  is  precisely  the  same  as  that 
for  the  extraction  of  the  ganglion  up  to  the  point  at  which  the  superior 


902  THE    EYE    AND    NERVOUS    SYSTEM 

and  posterior  aspects  of  the  ganglion  have  been  exposed.  At  this  junc- 
ture the  sensory  root  is  picked  up  on  a  small  blunt  hook,  grasped  witli  a 
haemostatic  forceps,  and  a  small  section  excised.® 

Excision  of  the  Gasserian  Ganglion. — Although  it  has  been  said  that 
the  ganglion  may  be  reached  readily  through  the  occipital  fossa,  the  best 
method  of  approach  unquestionably  is  through  the  temporal  region,  as 
advocated  first  by  Hartley  and  a  few  months  later  by  Krause.® 

Although  the  operator  must  be  thoroughly  familiar  with  the  anatomy 
of  the  structures  concerned,  it  would  not  be  amiss  to  call  attention  here 
to  some  points  of  considerable  practical  importance.  In  the  first  place, 
an  attempt  should  be  made  to  avoid  injuring  the  branch  of  the  facial 
nerve  which  supplies  the  orbicularis  palpebrarum.  The  inability  to 
close  the  eye  becomes  a  very  serious  matter  after  operations  in  which 
there  is  always  the  possibility  of  corneal  ulceration.  The  incision  usu- 
ally recomm.ended  for  this  operation  is  so  placed  that  this  branch  of 
the  facial  nerve  will  be  cut  almost  invariably.  Gushing  suggested, 
therefore,  that  the  flap  be  so  outlined  that  the  integrity  of  the  nerve 
be  not  endangered.  Second,  one  should  be  familiar  with  the  course  of 
the  middle  meningeal  artery,  and  also  with  the  fact  that  in  some  cases, 
in  its  passage  across  the  anterior  inferior  angle  of  the  parietal  bone  the 
artery  is  encased  in  a  bony  canal.  The  superior  margin  of  the  flap 
should  be  low  enough  to  avoid  injuring  the  vessel  at  this  point.  Other- 
wise the  artery  would  inevitably  be  torn.  This  is  not  a  serious  compli- 
cation ;  but  would  delay  the  operation  at  the  most  only  a  few  moments. 
The  foramen  spinosum  is  easily  identified  by  tracing  the  middle  me- 
ningeal artery  to  the  base  of  the  skull.  The  position  of  this  foramen 
is  important  on  account  of  its  relation  to  the  ganglion  and  its  branches. 
The  foramen  lies  a  little  external  to  the  posterior  aspect  of  the  ganglion 
and  a  little  behind  and  external  to  the  foramen  ovale  through  which 
passes  the  third  division.  It  is,  therefore,  an  important  landmark,  and 
a  knowledge  of  its  whereabouts  will  be  especially  helpful  to  those  who 
have  had  limited  experience  in  this  field  of  surgery.  Finally,  mention 
should  be  made  of  the  intimate  relation  of  the  third,  fourth,  and  sixth 
cranial  nerves,  as  well  as  of  the  cavernous  sinus,  with  the  internal 
aspects  of  the  ganglion. 


*Tlie  early  and  subsequent  stages  of  the  operation  are  described  in  the  teehnic 
for  excision  of  the  ganglion. 

•  No  one  should  undertake  the  operation  for  the  first  time  upon  the  living  sub- 
ject. The  operation  should  be  repeated  several  times  upon  the  cadaver  in  order  that 
at  least  some  of  the  difficulties  may  be  appreciated  and  a  vivid  impression  of  the 
exact  anatomical  relations  acquired. 


SURGEEY  OF   TRIGEMINAL   NERVE  903 

The  Operation. — A  horseshoe-shaped  incision  is  made  with  its  base 
corresponding  to  the  level  of  the  zygoma  and  its  summit  three  or  four 
centimetres  above.  The  musculo-cutaneous  flap  is  reflected  and  the 
zygoma  resected.  The  latter  step  is  important,  as  it  enables  the  operator 
to  approach  the  ganglion  from  a  plane  on  a  level  with  the  base  of  the 
skull ;  the  nearer  one  is  to  the  base  of  the  skull  the  shorter  the  distance 
to  be  traversed  before  the  ganglion  is  reached.  "With  mallet  and  gouge 
an  opening  is  made  in  the  skull  and  then  further  enlarged  with  rongeur 
forceps  until  the  opening  measures  three  or  four  centimetres  in  diam- 
eter. With  a  blunt  instrument,  as  the  handle  of  a  knife,  one  then  proceeds 
to  separate  the  dura  from  the  base  of  the  skull,  following  in  a  general 
way  the  direction  of  the  petrous  portion  of  the  temporal  bone.  At  this 
stage  of  the  operation  hemorrhage  may  be  a  troublesome  though  never 
an  alarming  feature.  The  firmer  the  adherence  of  the  dura  to  the  skull, 
and,  as  a  rule,  the  older  the  patient  the  freer  will  be  the  hemorrhage. 
Thus  it  may  be  necessary  from  time  to  time  to  stop  to  control  hemor- 
rhage by  packing  firmly  with  strips  of  gauze  and  allowing  the  same  to 
remain  in  situ  for  one  to  three  minutes. 

When  the  foramen  spinosum  is  reached  the  operator  separates  the 
dura  anterior  to  the  foramen  until  the  second  and  third  divisions  are 
exposed  as  they  pass  into  the  foramina  rotunda  and  ovale,  respectively. 
The  field  of  operation  during  this  and  the  subsequent  stages  of  the  opera- 
tion can  only  be  exposed  to  view  by  elevating  the  tempero-sphenoidal 
lobe,  and  for  this  purpose  a  suitable  retractor  should  be  provided.  The 
exposure  of  the  ganglion  is  never  difficult,  although  hemorrhage  may 
be  so  free  as  to  necessitate  one's  proceeding  rather  slowly.  The  most 
difficult  step  of  the  operation  is  now  approached.  To  thoroughly  iso- 
late the  ganglion  it  must  be  separated  from  its  dura  propria ;  to  accom- 
plish this  an  incision  is  made  with  an  Allis  director  or  other  blunt 
instrument  into  the  dura  from  the  second  to  the  third  division,  and 
by  blunt  dissection  the  superior  aspect  of  the  ganglion  is  exposed. 
After  dividing  the  second  and  third  divisions  the  ganglion  is  sepa- 
rated from  its  base,  grasped  with  a  pair  of  forceps  and  forcibly  ex- 
tracted. In  doing  this  the  cavernous  sinus  and  one  of  the  contiguous 
cranial  nerves  have  been  torn.  Hemorrhage  from  the  lacerated  sinus 
may  be  controlled  by  packing,  and  if  the  middle  meningeal  artery  has 
been  injured  near  the  foramen  spinosum  bleeding  may  be  checked  by 
ligating  the  vessel  by  plugging  the  foramen  or  by  gauze  packing.  The 
wound  is  irrigated  with  normal  saline  solution  and  a  strip  of  rubber 
tissue  introduced  for  drainage.  Certain  precautionary  measures  should 
be  adopted  to  protect  the  eye  and  prevent  corneal  ulceration.     Imme- 


904  THE    EYE    AND    NERVOUS    SYSTEM 

diately  before  or  after  the  operation  a  Buller  bandage  should  be  applied 
and  the  eye  irrigated  twice  daily  with  a  warm  boracic  solution.  In  addi- 
tion to  this  Keen  sews  the  eyelids  together  before  the  operation  is  begun, 
and  does  not  remove  the  stitches  until  the  third  day.  The  immediate 
results  of  the  operation  will  vary  considerably  according  to  the  experi- 
ence of  the  operator.  There  is  no  field  of  surgery  for  which  there  is  a 
greater  justification  in  specialization  than  in  the  surgery  of  the  central 
nervous  system.  The  mortality  of  Gasserian  ganglion  operations  has 
been  estimated  as  12  per  cent. ;  but  there  is  no  doubt  that  with  greater 
attention  to  those  requirements  of  technic  which  have  been  elaborated 
only  in  recent  years,  and  especially  in  the  hands  of  those  who  have  had 
exceptional  opportunities,  the  mortality  will  be  reduced. 

Abbe's  Method. — As  a  substitute  for  excision  of  the  ganglion  Abbe 
recommended  the  forcible  extraction  of  the  second  and  third  divisions 
and  the  interposition  of  a  piece  of  gutta-percha  tissue  one  and  one-half 
inches  by  three-quarters  of  an  inch  to  prevent  degeneration.  His 
technic  differs  somewhat  from  that  already  described  in  that  he  prac- 
tises preliminary  ligation  of  the  external  carotid  artery  and  exposes  the 
bone  by  a  vertical  incision  over  the  middle  of  the  zygoma.  Two  objec- 
tions to  this  method  of  treatment  at  once  suggest  themselves:  the  first, 
that  the  operation  is  applicable  only  to  those  cases  in  which  the  pain  is 
distributed  only  in  the  second  and  third  divisions ;  the  second  that  there 
is  a  possibility  of  the  rubber  tissue  acting  in  the  role  of  a  foreign  body 
setting  up  a  reaction  in  the  tissues,  which  would  terminate  in  abscess 
formation  and  necessitate  the  removal  of  the  foreign  material.  Abbe 
says :  "  It  is  certainly  past  dispute  that  there  is  no  need  for  the  removal 
of  the  first  branch  of  the  fifth  pair  in  any  case  of  grave  tic  douloureux 
unless  the  origin  is  to  be  found  in  a  tumor  of  the  Gasserian  ganglion  or 
behind  it."  This  may  apply  to  the  majority  of  cases,  but  it  certainly  is 
not  a  rule  without  exception.  In  fact,  in  two  cases  under  my  own  obser- 
vation the  pain  was  most  intense  in  the  distribution  of  the  first  division. 
It  might  also  be  said  of  Abbe's  operation  that  it  offers  no  assurance 
against  subsequent  involvement  of  the  first  division,  and,  so  far  as  the 
mortality  is  concerned,  it  should  l)e  no  less  than  that  following  division 
of  the  sensory  root,  since  both  are  intracranial  operations,  the  only 
difference  being  that  in  one  two  peripheral  branches  are  divided,  while 
in  the  other  one  divides  the  central  root  of  the  ganglion. 

Osmic  Acid  Injection. — Wliile  Billroth  and  Xeuber  were  the  first  to 
employ  osmic  acid  in  the  treatment  of  trifacial  neuralgia,  they  used 
repeated  injections  in  the  skin  without  exposing  the  nerve.  Burnett 
(1899)  was  the  first  to  recommend  the  more  careful  intraneural  injec- 


SUKGERY  OF   TRIGEMINAL   XERVE  905 

tions  into  the  exposed  nerve,  and  more  recently  (1904)  Murphy  has 
become  an  advocate  of  this  mode  of  treatment.  Up  to  the  present  time, 
however,  it  has  not  been  proven  that  the  results  are  much  better  than 
would  be  expected  after  an  excision  of  the  nerve  trunk.  The  injection 
of  osmic  acid  no  doubt  makes  it  more  difficult  for  the  nerve  to  undergo 
regeneration,  but  as  yet  we  have  no  assurance  that  regeneration  will  not 
eventually  occur.  Besides,  cases  have  been  reported  ^^  in  which  the 
treatment  was  a  failure.  The  immediate  results,  as  regards  the  harm- 
lessness,  and  at  least  the  temporary  relief,  might  be  said  to  justify  the 
use  of  this  treatment  as  a  substitute  neurectomy,  before  resorting  to  pro- 
cedures of  such  severity  as  intracranial  operations.  It  can  in  no  wise, 
however,  be  regarded  as  a  substitute  for  the  intracranial  operations  in 
those  eases  of  major  tic  in  which  the  pain  is  widely  distributed.  As  to 
the  technic,  a  one  and  one-half  to  two  per  cent,  solution  of  osmic  acid 
should  be  injected  in  many  places  into  the  exposed  nerve  trunk  and  also 
into  the  foramen.  Murphy  has  injected  the  palatine,  mandibular, 
lingual,  supraorbital  and  infraorbital  branches.  The  injections  should 
be  made  under  general  anaesthesia,  and  all  of  the  branches,  except  the 
supraorbital,  can  be  exposed  through  mouth  incisions. 

'"  Wright,  Medical  Chronicle,  Fourth  Series,  vol.  vi. 


CHAPTER    XXII. 

TREMORS  ;  REFLEXES ;  GAITS. 

Bv  JOSEPH  SAILER,  M.D. 

TREMORS. 

By  tremor  we  mean  an  oscillatory  movement  of  some  part  of  the 
body,  occurring  as  a  result  of  more  or  less  rhythmical  muscular  con- 
tractions, which  alternate  in  opposed  groups  of  muscles. 

Carefully  to  be  excluded  are  all  the  forms  of  pseudotremor  pro- 
duced by  rhythmical  shocks  occurring  within  the  body,  such  as  Musset's 
sign  in  aortic  regurgitation,  certain  movements  occurring  as  a  result 
of  aneurism,  and  movements  in  the  extremities  which  are  the  result 
of  vascular  pulsation.  There  is  some  doubt  as  to  whether  fibrillary 
contractions  within  a  certain  muscle  or  group  of  muscles  which  do  not 
produce  movements  at  any  of  the  joints,  or  waves  of  contraction  passing 
along  the  muscles,  should  be  included  under  tremors  or  not. 

Tremors  are  not  necessarily  signs  of  morbid  conditions.  They 
may  occur  as  a  result  of  certain  physiological  states.  Thus,  tremors 
of  the  extremities  of  more  or  less  severity  occur  during  and  after  pro- 
longed severe  exertion,  in  the  course  of  violent  emotional  disturbance, 
after  chilling,  the  most  frequent  example  being  chattering  of  the  teeth, 
after  excesses  of  various  kinds,  and  during  convalescence  from  severe 
infectious  disease.  They  may  also  be  produced  voluntarily,  either  for 
purposes  of  simulation  or  experimentation.  In  all  these  forms  the 
movement  is  slow  and  rhythmical,  and  the  duration  is  short.  The  head 
and  limbs  are  the  parts  most  commonly  involved,  and  the  movements 
are  at  least  partially  under  the  control  of  the  will. 

Among  the  causes  of  pathological  tremor  may  be  mentioned  par- 
ticularly the  poisons.  Alcohol,  especially  chronic  alcoholism,  is  apt 
to  be  associated  with  a  fine  tremor  of  the  hands,  lips  and  tongue. 
Tobacco  is  said  to  cause  a  fine  tremor  of  the  hands  and  eyelids.  Lead, 
mercury,  anilin,  and  various  other  poisonous  substances,  may  also  pro- 
duce tremors.  These  are  nearly  all  fine,  slow  tremors,  often  increased 
by  voluntary  movement,  and  affecting  chiefly  the  upper  extremities 
and  the  muscles  of  the  face.  In  the  course  of  various  diseases  of  the 
nervous  system  characteristic  tremors  occur  that  aid  materially  in  the 
diagnosis. 
906 


TREMORS  .  907 

CLASSIFICATION. 

Tremors  are  classed,  first,  as  rhythmical  and  arhythmical.  The 
rhythmical  tremors  frequently  show  in  addition  a  rhythmical  increase 
and  decrease  in  their  severity.  We  also  speak  of  fine  and  coarse  tremors. 
In  the  former  the  oscillation  is  very  slight,  perhaps  imperceptible;  in 
the  latter  it  is  of  greater  amplitude.  Tremors  may  be  regarded  as  rapid 
when  the  rate  of  oscillation  is  from  eight  to  twelve  times  per  second, 
and  slow  when  it  is  less  than  five  times  per  second.  Intermediate  forms 
also  occur.  Tremors  are  spoken  of  as  static  if  they  are  present  during 
rest,  and  as  movement  or  intention  tremors  if  they  are  either  produced 
or  increased  by  voluntary  movements.  If  the  tremor  resembles  some 
familiar  motion  it  is  usually  called  by  that  name,  as  the  tremor  of 
negation  when  the  head  is  rotated  rhythmically  from  side  to  side;  the 
nodding  or  salaam  tremor  when  the  head  oscillates  forward  and  back- 
ward, and  the  pill-roller's  tremor  of  paralysis  agitans. 

Numerous  efforts  have  been  made  to  obtain  some  information  re- 
garding the  nature  of  tremors  by  means  of  recording  apparatus.  These 
all  consist  essentially  of  a  moving  surface  and  some  transmitting  mech- 
anism by  means  of  which  the  oscillations  are  recorded.  It  has  been 
possible  by  these  methods  to  determine  the  rhythm,  the  rate,  and,  in 
a  relative  manner,  the  amplitude  of  the  oscillations,  but  practically 
nothing  more,  and  the  studies  have  not  contributed  materially  to  our 
knowledge  of  disease  of  the  nervous  system. 

It  will  be  most  convenient  to  discuss  briefly  those  tremors  which 
occur  in  association  with  nervous  disease,  and  are  therefore  of  more  or 
less  diagnostic  significance.  There  is  one  condition  in  which  the  tremor 
is  practically  the  only  sign  of  a  morbid  process.  This  is  the  simple 
hereditary  or  congenital  tremor.  It  occurs  sometimes  sporadically, 
sometimes  in  several  members  of  the  same  family,  and  occasionally 
exists  in  successive  generations.  The  tremor  is  usually  rather  slow  in 
rate  and  fairly  rhythmical.  It  may  affect  almost  any  part  of  the  body, 
but  as  a  rule  the  head  or  upper  extremities  are  particularly  involved. 
The  tremor  sometimes  develops  only  during  activity  or  emotional  stress, 
but  often  continues  during  rest,  and  indeed  may  be  most  pronounced 
during  rest.  Occasionally,  but  not  often,  it  is  under  voluntary  control. 
It  may  possibly  be  regarded  as  a  stigma  of  degeneration,  if  this  term 
has  any  real  significance,  but  it  is  often  found  in  intellectual  persons, 
and  even  when  it  involves  the  upper  extremities,  does  not  appear  to 
interfere  with  occupations  requiring  delicate  manipulations.  Usually 
it  does  not  develop  until  adolescence,  or  adult  life,  but  occasionally  it 
is  present  in  infants  (Raymond). 


908  THE    EYE    AND    NERVOUS    SYSTEM 

Senile  Tremor. — In  old  age  tremor  frequently  develops.  It  usu- 
ally affects  the  hands,  and  most  commonly  is  in  the  form  of  flexion  and 
extension  at  the  elbow  and  metacarpophalangeal  joints,  although  other 
parts  are  frequently  involved.  It  often  affects  the  head,  giving  rise  to 
a  nodding  movement.  It  is  usually  static;  the  movements  are  slow, 
not  often  exceeding  five  per  second,  and  usually  less  than  five.  Volun- 
tary motion  often  abolishes  the  tremor  for  a  short  time. 

The  tremor  of  paralysis  agitans  is  characteristic.  In  addition  to 
the  rigidity  of  the  nmscles  and  peculiar  attitude  and  expression  which 
constitute  the  essential  signs  of  the  disease,  there  occurs,  with  very  few 
exceptions,  a  fine  static  tremor  of  the  fingers,  which,  in  its  most  charac- 
teristic form,  consists  of  opposition  and  withdrawal  of  the  thumb  and 
flexion  and  extension  at  the  metacarpophalangeal  joints.  The  hand, 
therefore,  assumes  a  position  somewhat  similar  to  that  seen  in  the  spasm 
of  tetany,  and  the  movements  of  the  fingers  are  supposed  to  resemble 
the  movements  made  in  rolling  pills,  hence  the  name,  "  pill-roller's 
tremor."  The  tremor,  however,  often  involves  the  arms  or  head,  and 
even  the  trunk.  Its  most  characteristic  peculiarity  is  that  it  is  abolished 
or  markedly  diminished  by  voluntary  movements,  although  upon  con- 
tinuance of  voluntary  movement,  such  as  writing,  the  tremor  soon  re- 
appears. 

The  tremor  of  multiple  sclerosis  is  the  most  typical  of  all  forms  of 
movement  of  intention  tremors.  It  develops  usually  only  when  the 
patient  makes  a  voluntary  movement,  becoming  more  and  more  violent 
until  the  movement  is  completed,  when  it  ceases.  The  oscillations  are 
coarse,  irregular,  arhythmic,  and  slow,  ranging  from  one  or  two  to  five 
or  six  per  minute.  In  many  respects  they  resemble  the  movements  of 
ataxia,  and  no  sharp  distinction  can  be  drawn  between  them.  It  may 
be  said,  however,  that,  as  a  rule,  the  tremor  of  multiple  sclerosis  is  less 
under  the  control  of  the  patient,  even  when  guided  by  vision,  than  is  the 
tremor  of  ataxia.  The  symptom  can  best  be  elicited  by  having  the 
patient  grasp  at  an  object  held  at  arm's  length  from  him,  or  by  asking 
him  to  carry  a  glass  of  w^ater  to  the  lips,  or  by  directing  him  to  touch 
the  tip  of  his  nose  with  his  finger. 

The  tremor  of  exophthalmic  goitre  resembles  most  closely  the  toxic 
tremors.  It  usually  occurs  in  the  hands,  and  is  only  elicited  when  the 
muscles  are  made  tense  by  extreme  extension  of  the  fingers.  The  oscil- 
lations are  of  small  amplitude,  rhythmical,  rapid,  and  cease  only  when 
the  muscles  are  relaxed. 

The  tremors  of  hysteria  and  allied  functional  conditions  of  the 
nervous  system  are  of  various  types.     The  patient  may  exhibit  a  fine 


TKEMORS  909 

constant  tremor  of  the  extremities  and  head,  not  much  affected  by  move- 
ment, nearly  always  disappearing  during  sleep,  slightly  arhythmic  and 
moderately  rapid.  The  tremor  is  frequently  unilateral,  and  is  to  be 
distinguished  as  hysterical  only  by  the  presence  of  the  other  stigmata 
of  that  condition.  In  common  with  all  hysterical  stigmata,  it  may  be 
temporarily  or  permanently  abolished  by  some  profound  emotional 
shock.  Hysterical  patients  may  also  manifest  from  time  to  time  irreg- 
ular waving  motions,  sometimes  tremor-like,  but  often  so  slow  that  they 
cannot  properly  be  included  under  this  designation.  They  may  resemble 
gestures,  and  occasionally  have  a  distinctly  histrionic  character.  They 
often  have,  in  addition,  a  rapid,  violent  tremor  that  may  be  produced 
by  different  attitudes,  movements,  or  even  associations.  This  tremor 
may  affect  one  extremity,  the  side,  or  perhaps  the  whole  body.  The 
movements  are  rhythmic,  rapid,  and  of  considerable  amplitude.  They 
usually  increase  in  violence  until  the  particular  action  that  causes  them 
to  cease  is  applied.  This  may  be  either  the  assumption  of  some  attitude 
on  the  part  of  the  patient,  pressure  upon  the  trembling  extremity,  or 
some  other  purely  suggestive  influence.  Occasionally,  however,  the 
tremor  increases  until  it  becomes  actually  a  clonic  convulsion,  or  it  may 
terminate  in  a  hysterical  crisis.  Tremors  of  this  type  resemble  very 
closely  the  clonic  spasms  of  various  conditions,  particularly  epilepsy. 

The  third  group  are  the  imitation  tremors:  Krafft-Ebing  called 
particular  attention  to  the  frequency  with  which  hysterical  patients 
simulate  the  tremor  of  paralysis  agitans,  and  various  other  types  of 
tremor  in  organic  disease  are  also  imitated  more  or  less  closely.  It 
seems  to  be  common  to  all  these  tremors  that  they  are  more  in  evidence 
when  the  patient  is  under  observation  than  when  apparently  alone ;  that 
their  amplitude  is  greater  than  the  real  organic  tremors,  that  they  are 
not  likely  to  be  rhythmic ;  and  that  they  do  not  cease  as  a  result  of  the 
conditions  that  cause  the  cessation  of  the  organic  tremors. 

In  neurasthenia  a  fine  tremor  of  the  extremities,  static  in  character, 
may  often  be  detected.  In  epilepsy  a  tremor  of  the  hands  has  been 
noted  during  periods  of  disturbance  of  consciousness  that  replace  the 
convulsive  attack. 

In  general  paresis  there  is  a  fine  tremor  of  the  tongue  and  lips 
and  occasionally  a  slight  tremor  of  the  hands.  In  hemiplegia  and  in 
other  forms  of  spastic  paralysis  tremors  are  very  apt  to  occur.  They 
are  usually  coarse,  irregular,  and  sometimes  closely  simulate  the  move- 
ments of  ataxia.  They  are  nearly  always  of  the  movement  or  intention 
type,  and  arc  exaggerated  by  emotional  disturbances.  Often  with  a 
return  of  power  the  tremor  gradually  improves  and  may  ultimately 


910  THE    EYE    AND    NERVOUS    SYSTEM 

entirely  cease.    Occasionally  a  very  fine  tremor  persists  in  the  side  that 
has  been  paralyzed,  even  after  an  apparent  complete  return  of  power. 

Akin  to  tremors  are  the  choreiform  movements,  the  movements  that 
occur  in  ataxia,  and  various  types  of  clonic  spasm,  as,  for  example,  ankle- 
clonus.  Their  description,  however,  will  be  found  in  other  sections  of 
this  book. 

REFLEXES. 

By  the  term  "  reflex"  we  understand  some  manifestation  of  vital 
activity  that  occurs  as  a  result  of  obvious  stimulation.  Possibly  all 
forms  of  vital  activity  are  the  result  of  stimulants  of  one  kind  or 
another,  and  therefore,  properly  speaking,  reflexes,  but  unless  the  stim- 
ulus can  be  detected  we  are  not  justified  in  using  this  term.  Clinically, 
we  make  a  distinction  between  the  psychical  and  the  mechanical  re- 
flexes. By  the  former  we  mean  the  various  actions  that  occur  as  a 
result  of  the  stimulation  of  the  perceptive  centres  of  the  brain,  of  which 
an  example  is  the  secretion  of  the  saliva  at  the  sight  of  food.  Such 
reflexes  have  to  do  rather  with  psychology  than  with  clinical  medicine, 
and  will  not  be  further  considered  in  this  article. 

By  a  mechanical  reflex  we  understand  the  involuntary  contraction 
of  a  muscle  or  group  of  muscles  as  a  result  of  the  stimulation  of  certain 
sensory  fibres.  This  definition  implies  that  the  stimulus  is  transmitted 
along  the  sensory  fibre  to  some  part  of  the  central  nervous  system  in 
which  there  is  a  path  of  communication  between  the  receptive  sensory 
cells  and  the  emissive  motor  cells,  and  then  from  the  motor  cells  along 
the  motor  nerve-fibres  to  the  muscle  or  muscles  involved.  This  is  spoken 
of  as  a  reflex  arc,  and  it  is  considered  that  whenever  its  continuity  is 
broken  the  reflex  necessarily  ceases  to  occur. 

Theoretically  a  sharp  distinction  is  drawn  between  reflexes  and 
muscular  contractions  which  result  from  direct  stimulation  of  the  mus- 
cle involved,  or  of  its  motor-nerve  supply,  but  in  clinical  work  this 
distinction  cannot  always  be  maintained,  and  there  are  not  wanting  in- 
vestigators who  contend  that  many  of  the  reflexes  upon  which  most 
dependence  is  placed  in  diagnosis,  including,  for  example,  the  knee- 
jerk,  are  the  result  rather  of  direct  irritation  of  the  muscle,  either  by 
sudden  elongation  or  by  some  other  method,  than  of  the  completion  of 
a  reflex  arc  by  the  primary  stimulation  of  the  peripheries  of  the  sensory 
nerves.  It  must,  of  course,  be  understood  that  the  integrity  of  the  nerve 
supply  of  the  muscle  by  which  its  tone  is  maintained  is  essential  to  the 
production  of  the  normal  contraction.  The  distinction  between  the  two 
theories  is  more  philosophical  than  practical.  Muscular  contractions 
can,  of  course,  also  be  produced  by  the  direct  irritation  of  motor  nerves, 


REFLEXES  911 

either  at  their  termination  or  in  the  course  of  the  nerve-trunk,  as,  for 
example,  the  contraction  of  the  little  and  ring-fingers  which  occurs 
when  the  ulnar  nerve  is  forcibly  pinched.  It  is  customary  to  speak  of 
a  reflex  whenever,  as  a  result  of  mechanical  stimulation,  particularly 
in  the  form  of  percussion,  or  stroking,  a  motor  response  occurs.  The 
number  of  reflexes  is  already  considerable,  and  every  year  three  or  four 
additions  are  made,  most  of  them  unimportant.  They  are  classified 
in  various  ways ;  sometimes  anatomically,  as  reflexes  of  the  head,  trunk, 
or  extremities;  sometimes  according  to  the  part  to  which  the  sensory 
stimulation  is  applied,  as  the  skin,  periosteum,  bone,  and  tendon  re- 
flexes ;  sometimes  into  the  skin  or  "  superficial"  and  the  "  deep"  re- 
flexes.        ,    t 

The  mechanism  of  the  reflex  action  has  been,  ever  since  the  first 
description  of  the  knee  reflex  by  Erb  and  Westphal,  a  matter  of  pains- 
taking investigation,  and  a  considerable  number  of  theories  have  been 
evolved.  Taking  the  knee-jerk  as  the  type  of  the  deep  reflexes  the  fol- 
lowing facts  appear  to  have  been  definitely  ascertained : 

The  refiex  is  best  elicited  when  the  muscle  is  moderately  stretched. 
It  varies  in  general  as  the  muscle  tone;  but  this  rule  is  subject  to 
many  exceptions,  in  states  of  hypotonia  exaggerated  reflexes  being 
present  (Muskens).  It  is  modified  by  various  external  conditions, 
thus,  attention  to  the  reflex  diminishes  it;  distraction  of  the  patient's 
attention  by  either  psychical  or  motor  occupation  increases  it.  It  is 
usually  increased  as  a  result  of  fatigue  or  cachexia.  Any  diminution 
of  the  functional  activity  of  the  sensory  fibres  leading  to  the  lumbar 
portion  of  the  spinal  cord  diminishes  or  abolishes  the  reflex.  Complete 
destruction  of  the  motor  fibres  leading  from  the  lumbar  portion  of  the 
cord  to  the  quadriceps  extensor  also  abolishes  the  reflex.  Partial  de- 
struction of  the  peripheral  motor  neurones  may  be  compatible  with  the 
persistence  of  the  reflex,  and  even  some  degree  of  exaggeration,  as  in 
amyotrophic  lateral  sclerosis.  Destruction  of  the  centre  for  the  reflex  in 
the  lumbar  cord  causes  abolition  of  the  reflex.  Complete  destruction  of  a 
segment  or  segments  of  the  spinal  cord  above  this  centre  usually  causes 
loss  of  the  reflex,  but  occasionally  it  persists,  and  is  even  exaggerated. 
If  the  posterior  columns  of  the  spinal  cord  are  destroyed,  as,  for  exam- 
ple, in  tabes  dorsalis,  it  is  abolished.  If  the  lateral  pyramidal  columns 
are  destroyed  it  is  greatly  increased.  Lesions  in  the  brain  involving 
the  motor  centres  commonly  cause  a  great  exaggeration.  Lesions  not 
involving  the  motor  centres  usually  cause  a  moderate  exaggeration,  but 
the  reflex  may  be  lost  if  the  lesion  is  situated  in  the  frontal  lobes  or  in 
the  cerebellum. 


912  THE    EYE    AND    NERVOUS    SYSTEM 

It  appears  from  an  analysis  of  these  facts  that  the  mechanism  of 
the  reflex  involves  more  than  a  sensory  and  a  motor  fibre  and  the  con- 
necting link  in  the  spinal  cord.  Therefore  it  has  been  customary  to 
assume  the  existence  of  a  second  arc,  the  afferent  fibres  being  the  con- 
tinuation upward  of  the  sensory  fibres  passing  from  the  periphery  to 
the  cord,  and  the  efferent  fibres  being  the  axis  cylinders  of  the  upper 
motor  neurones,  the  arc  being  completed  by  some  of  the  commissural 
fibres  of  the  brain.  This  second  arc  appears  to  have  a  double  function ; 
that  is  to  say,  the  persistence  of  the  sensory  part  is  necessary  to  the 
persistence  of  the  reflex ;  the  loss  of  the  motor  portion  tends  to  enhance 
the  reflex.  It  must  be  remembered,  however,  that  the  destruction  of 
tlie  sensory  portion  some  distance  above  the  entrance  of  the  nerve  fibres 
into  the  cord  occasionally  may  occur  without  loss  of  the  reflexes,  and 
if  this  injury  to  the  sensory  portion  occurs  above  the  nuclei  of  the 
columns  of  Goll  and  Burdach  in  the  medulla,  the  loss  rarely  occurs. 

It  is  of  course  difficult  to  understand  how  the  two  limbs  of  an  arc 
could  exhibit  so  much  difference  in  action.  It  may  be  assumed,  for 
the  sake  of  argument,  that  the  upper  arc  is  not  really  an  arc  at  all; 
that  tlie  motor  fibres  passing  from  the  cortex  are  concerned — with  ref- 
erence only  to  reflex  action — merely  with  the  control  of  muscle  tone, 
and  particularly  with  the  inhibition  of  involuntary  movements  (Drei- 
faserschema).  Therefore,  if  for  any  reason,  this  inhibitory  influence 
is  removed,  the  reflex,  of  necessity,  becomes  exaggerated.  It  is  more 
difficult  to  understand  why  the  destruction  of  the  sensory  columns 
should  diminish  or  abolish  the  reflex.  It  is  supposed,  for  example,  that 
this  destruction  always  involves  the  peripheral  sensory  nerves  that  lead 
to  the  lower  reflex  arc.  But  this  does  not  accord  with  the  observation 
so  frequently  made  that  it  can  now  be  regarded  as  thoroughly  estab- 
lished, that  complete  or  even  partial  transverse  lesion  of  the  spinal  cord 
in  the  upper  dorsal  or  lower  cervical  portion  is  usually  but  not  invaria- 
bly associated  with  loss  of  the  reflexes,  which  is  not  due  merely  to  loss 
of  sensation.  Numerous  explanations  have  been  suggested,  according 
to  which  this  loss  is  not  due  so  much  to  the  destruction  of  the  sensory 
columns  above  the  point  of  lesion  as  it  is  to  a  degeneration  of  the 
peripheral  motor  neurones  and  of  the  cells  of  Clarke's  column,  which 
has  been  found  in  the  segments  in  which  the  reflex  arc  is  completed,  or 
to  an  oedema  of  the  lower  part  of  the  spinal  cord  inhibiting  its  func- 
tional activity,  or  to  loss  of  certain  influences  emanating  from  the 
cerebrum  or  the  cerebellum,  or  to  disturbance  of  the  circulation  in  the 
lower  part  of  the  cord  leading  to  degeneration  of  the  ganglion  cells,  or 
to  the  effect  of  shock  upon  the  lower  part  of  the  cord,  although  the 


REFLEXES  '  913 

reflexes  in  the  part  above  the  lesion  are  not  affected ;  or  to  degenera- 
tion of  the  posterior  lumbar  roots,  this  is  called  tertiary  degeneration 
(Brasch)  ;  or  to  hypotonia.  Van  Gehuchten  suggests  that  there  may 
be  increased  inhibitory  action.  Aside  from  the  degeneration  of  the  cells 
in  Clarke's  column,  no  histological  evidence  has  been  discovered  of  in- 
jury to  the  sensory  neurones.  There  appears  to  be  some  relation  be- 
tween the  amount  of  injury  done  to  the  cord  and  the  duration  of  the 
inhibition  of  the  reflexes,  at  least  in  dogs.  This  loss  is  associated  with 
a  highly  developed  nervous  system,  for,  according  to  Sherrington,  in 
the  dog  and  cat  transverse  sections  of  the  cord  cause  exaggeration  of 
the  knee-jerks,  and  sometimes  in  human  beings  and  in  the  ape  the  loss  is 
not  permanent. 

The  way  in  which  the  sensory  stimulation  occurs  has  been  the  sub- 
ject of  some  dispute.  In  all  the  cutaneous  reflexes  it  appears  to  be 
due  to  the  stimulation  of  the  cutaneous  sensory  nerves,  whether  that 
stimulation  is  perceived  in  consciousness  or  not.  In  the  periosteal 
reflexes  it  is  supposed  that  the  sensory  nerves  in  the  periosteum  receive 
and  transmit  the  impulse.  In  the  case  of  the  simple  muscular  con- 
tractions due  to  direct  irritation  of  the  substance  of  the  muscle,  or  its 
fascia,  reflex  action  probably  does  not  occur,  or  plays  a  subordinate  role, 
and  the  same  is  true  of  muscular  contractions  due  to  irritation  of  a 
nerve  trunk  such  as  occurs  in  Chvostek's  phenomenon.  The  dispute, 
however,  has  referred  particularly  to  the  tendon  reflexes.  The  reflex 
occurs  because  a  sharp  blow  is  struck  upon  the  moderately  stretched 
superficial  tendon  of  the  muscle.  Tendons  are  so  poorly  supplied  with 
nerves,  and  particularly  sensory  nerves,  that  it  does  not  seem  pos- 
sible that  the  reflex  is  a  result  of  direct  irritation.  Therefore,  it  has 
been  surmised  that  it  is  due  to  the  irritation  of  the  overlying  skin,  the 
underlying  periosteum  (both  of  which  hypotheses  may  be  dismissed, 
particularly  the  former,  as  the  reflexes  occur  in  conditions  of  anaesthesia 
of  the  thigh  or  if  the  tendon  is  exposed)  ;  or  to  stimulation  of  the  sen- 
sory nerves  in  the  muscle  by  vibrations  communicated  to  it,  or  by  its 
simple  sudden  elongation.  There  can  be  no  doubt,  with  reference  to 
the  knee-jerk,  at  least,  that  a  distinct,  powerful  sensory  impression 
occurs,  sufficient — when  the  inhibitory  influence  of  the  motor  fibres  is 
removed — to  cause  contraction  not  only  of  the  muscles  affected  by 
the  blow  upon  the  patellar  tendon,  unimuscular,  but  also  of  other 
groups  of  muscles  in  the  same  leg,  multimuscular  or  even  in  the  oppo- 
site leg.  ^Mien  this  occurs  it  is  spoken  of  as  a  crossed  reflex.  From 
the  fact  that  degeneration  of  the  posterior  columns  of  the  cord  inhibits 
the  reflex,  it  seems  not  unlikely  that  the  fibres  of  muscular  sense  are 

58 


914  THE    EYE    AND    NERVOUS    SYSTEM 

the  ones  chiefly  concerned  in  the  production  of  the  so-called  tendon 
reflexes.  But  the  impossibility  of  separating  tliese  fibres  in  the  nerve 
trunks  renders  all  views  upon  this  subject  entirely  hypothetical. 

The  essential  nature  of  reflex  action  is  not  clearly  understood.  Re- 
flexes were  originally  supposed  to  be  involuntary  movements  of  escape. 
Thus,  the  drawing  up  of  the  leg  when  the  sole  of  the  foot  is  tickled 
is  apparently  an  attempt  to  remove  the  part  from  the  source  of  the 
irritation.  But  it  is  impossible  to  explain  all  reflexes  in  this  manner. 
It  has  been  supposed  that  reflex  action  was  the  muscular  response  to 
a  local  irritation,  psychology  indicating  that  in  the  vast  majority  of 
cases  an  external  stimulus  precedes  muscular  activity,  and  that  the 
psychical  stimulus  which  may  occasionally  replace  this  represents 
merely  stored-up  external  sensory  impressions.  According  to  this  view, 
all  action  is  essentially  reflex  in  nature,  but  some  of  the  reflexes  are 
comparatively  simple  and  quite  regular,  whilst  others  are  complex, 
irregular,  and  apparently  purposeful.  Although  this  view  may  be 
entirely  satisfactory  to  the  psychologist,  it  is  of  no  particular  advantage 
to  the  clinician.  He  is  compelled  to  regard  certain  activities  produced 
by  sensory  stimuli  as  reflexes  upon  whose  existence  or  alteration  diag- 
noses may  be  based.  All  other  forms  of  movement,  if  of  clinical  sig- 
nificance, must  be  considered  from  a  totally  diflFerent  standpoint.  It 
is  therefore  from  a  clinician  that  we  have  received  the  most  ingenious 
and,  in  some  respects,  most  satisfactory  explanation.  Von  Striimpell 
believes  that  these  reflex  movements  are  vestiges  of  the  complex  co- 
ordinated reflexes  of  the  lower  animals,  which  in  human  beings,  as  a  result 
of  the  increase  in  the  cerebral  motor  activities  at  the  expense  of  the 
spinal  motor  activities,  have  been  reduced  to  insignificant  and  appar- 
ently purposeless  movements.  Therefore,  he  believes  that  when  the 
cerebral  controlling  influence  is  removed,  the  reflexes  become  exag- 
gerated. As  a  matter  of  fact,  the  reflex  interval  is  shorter  in  apes 
and  idiots  than  in  normal  persons  (Silbertini).  Of  course  it  is  neces- 
sary to  omit  from  consideration  all  those  movements  due  either  to 
direct  irritation  of  the  muscles  or  to  direct  irritation  of  the  motor  nerves, 
which  are  not  properly  reflexes,  but  merely  manifestations  of  the  in- 
herent and  essential  contractility  of  muscular  substance. 

Unfortunately,  there  is  still  considerable  dispute  regarding  the 
division  in  which  many  of  the  reflexes  should  be  classified;  some  hold- 
ing that  they  are  due  merely  to  direct  irritation  of  the  muscle  or  its 
tendon ;  others  that  they  are  true  reflexes  following  the  sensory  nerve 
to  the  central  nervous  system,  and  returning  along  the  course  of  a  motor 
nerve.     It  is  not  impossible  that  in  a  few  instances  the  reflexes  may  be 


KEFLEXES  915 

mixed,  or  at  least  in  health  may  be  merely  muscular  contractions,  but 
when  the  inhibitory  influence  of  the  central  motor  mechanism  is  re- 
moved become  true  reflex  movements. 

In  studying  reflexes  for  the  purpose  of  utilizing  the  knowledge 
obtained  for  diagnosis  or  prognosis,  certain  factors  must  be  considered : 
(1)  The  condition  of  the  patient  at  the  time  the  reflex  is  tested;  this 
involves  not  merely  the  jjhysical,  but  also  the  mental  condition,  states 
of  excitement,  fright,  or  preoccupation  often  having  considerable  in- 
fluence upon  reflex  activity.  (2)  The  temperature  of  the  room.  (3) 
The  mechanism  employed  for  eliciting  the  reflex.  (4)  The  position  of 
the  patient.  With  regard  to  the  reflex  itself,  it  should  be  noted  (1) 
whether  it  occurs  promptly  or  slowly;  (2)  whether  the  response  is  nor- 
mal, exaggerated,  or  diminished;  (3)  whether  other  muscles  than 
those  usually  concerned  are  involved  in  the  response,  and  (4)  the  effect 
of  the  investigation  upon  the  patient. 

In  general  it  may  be  said  that  conditions  causing  increased  myo- 
tatic  irritability  increase  reflex  activity.  Mental  emotion,  such  as 
fright,  often  inhibits  reflex  action.  Great  excitement,  on  the  other 
hand,  may  enhance  it.  Chilliness  increases  it,  and  extreme  cold,  suffi- 
cient to  numb  the  peripheral  nerves,  appears  either  to  diminish  or 
abolish  it.    In  sleep  it  is  diminished  or  even  absent  (Rosenbach). 

Nearly  all  reflexes  may  be  elicited  by  the  hand,  but  a  great  variety 
of  instruments  have  been  devised  for  the  purpose  of  demonstrating  them 
more  readily.  The  most  generally  useful  is  the  rubber  percussion 
hammer  which  has  a  triangular  piece  of  firm  white  rubber  for  its  head, 
the  blow  being  struck  with  the  apex  of  the  triangle.  The  ordinary  per- 
cussion hammer  may  also  be  employed.  Instruments  have  also  been 
devised  particularly  for  measuring  the  knee-jerk,  so  that  the  blows 
struck  upon  the  tendon  shall  be  uniform  in  force.  It  may  be  said  in 
reference  to  all  these  instruments  that  up  to  the  present  time  no  clinical 
method  for  measuring  reflex  action  has  been  devised  that  has  such  a 
degree  of  accuracy  as  to  be  in  the  least  useful,  and  it  is  still  necessary 
to  estimate  all  reflexes  according  to  the  scale  of  "  diminished,  normal, 
or  exaggerated,"  the  determination  to  which  one  of  these  three  classes 
any  given  reflex  belongs  depending  entirely  upon  the  judgment  of  the 
physician.  Doubtless  many  errors  are  made,  but  on  the  whole  the 
results  have  been  of  great  service.  It  is  possible  that  the  method  of 
Pandy  for  measuring  the  force  of  the  knee-jerk  may  be  of  clinical 
value  for  the  detection  of  marked  paresis. 

For  the  purpose  of  description,  the  reflexes  will  here  be  considered 
according  to  a  convenient  rather  than  a  logical  classification.     The  re- 


916  THE    EYE    AXD    NERVOUS    SYSTEM 

flexes  of  the  head,  upper  and  lower  extremities,  and  of  the  trunk  will 
be  grouped  into  tendon,  cutaneous,  and  muscle  reflexes.  Xo  attempt 
will  be  made  to  treat  exhaustively  those  forms  which  are  of  doubtful 
nature  and  little  or  no  clinical  significance. 

REFLEXES    OF   THE   HEAD. 

There  are  no  tendon  reflexes  in  the  head.  The  other  forms  of  reflex 
action,  aside  from  the  pupillary  reflexes,  that  may  be  elicited  are: 

The  Supraorbital  Reflex. — This  reflex  was  described  by  McCarthy 
in  1900  and  consists  of  a  slight,  brief  twitching  of  the  orbicularis  pal- 
pebrarum, most  pronounced  in  the  outer  half,  when  a  blow  is  struck 
upon  the  trunk  of  the  supraorbital  nerve.  The  reflex  disappears  in 
cases  of  destructive  injury  to  the  supraorbital  nerve,  causing  anesthesia 
in  its  area  of  distribution,  and  in  peripheral  facial  paralysis.  In  cases 
of  injury  to  the  central  nervous  system  no  characteristic  alteration 
usually  occurs.  A  crossed  supraorbital  reflex  may  develop  (Sailer). 
This  may  occur  either  under  normal  conditions,  in  which  the  twitching 
of  the  eye  on  the  opposite  side  is  very  slight,  or  in  cases  of  peripheral 
facial  paralysis,  in  which  it  is  quite  distinct.  Occasionally  other  move- 
ments occur,  such  as  twitching  of  the  ala  of  the  nose  or  lifting  of  the 
corner  of  the  mouth.  These  phenomena,  however,  are  much  more  com- 
mon in  connection  with  the  malar  reflex.  It  has  been  claimed  that  the 
supraorbital  reflex  is  not  a  true  reflex,  but  is  due  to  percussion  upon 
the  fibres  of  the  orbicularis  palpebrarum  that  spread  upward  from  the 
eyebrow  (von  Bechterew).  The  crossed  reflex  is  explained  by  assuming 
tliat  the  fibres  on  the  opposite  side  may  interlace.  It  seems  difiicult, 
however,  to  account  for  the  disappearance  of  the  reflex  in  cases  in  which 
the  supraorbital  nerve  has  been  cut  or  injured  (McCarthy).  The  chief 
clinical  significance  of  this  reflex  appears  to  be  that  when  it  is  present  it 
indicates  that  the  supraorbital  nerve  on  that  side  is  intact,  and  that  there 
is  no  peripheral  or  facial  palsy.  When  it  is  absent  it  indicates  that 
either  the  supraorbital  or  facial  nerve  has  ceased  for  some  reason  to 
convey  impulses.  As  facial  paralysis  is  usually  easily  detected,  when 
it  is  absent  the  lesion  can  be  localized  to  the  supraorbital  nerve.  Of 
course,  when  present  it  may  be  uncertain  whether  the  supraorbital  ner\'e 
is  affected  or  not.  Under  these  circumstances,  the  existence  of  the 
crossed  reflex  would  serve  to  indicate  that  the  supraorbital  nerve  upon 
the  paralyzed  side  is  intact. 

Chvostek's  phenomenon  consists  of  a  sudden  twitching  of  the  muscles 
of  the  corner  of  the  mouth,  ala  of  the  nose,  and  the  external  portion  of 
the  palpebral  fissure  when  the  trunk  of  the  facial  nerve  is  struck  just 


REFLEXES  917 

after  it  winds  around  the  posterior  ramus  of  the  lower  jaw.  This  is 
not  a  true  reflex,  but  simply  exalted  susceptibility  of  the  nerve  trunk 
to  external  stimuli.  In  certain  cases  this  may  be  so  great  that  per- 
cussion merely  upon  one  of  the  branches  of  the  nerve  will  suffice  to 
produce  the  muscular  reaction.  Occasionally  simple  percussion  upon 
the  malar  bone  or  pressure  upon  the  eyeball  (Fuchs)  produces  the  same 
effect.  This  reflex  is  strictly  pathological,  and,  as  far  as  we  know, 
pathognomonic  of  tetany,  in  all  forms  of  which  it  may  occur.  Its 
absence  does  not  exclude  the  existence  of  tetany,  particularly  in  those 
forms  grouped  together  as  non-idiopathic ;  that  is,  associated  with  vari- 
ous other  conditions,  such  as  cachexia  thyreopriva,  pregnancy,  dilatation 
of  the  stomach,  etc.  It  is,  however,  regarded  as  of  such  importance 
that  when  it  is  absent  it  is  usual  to  speak  of  the  "  incomplete  type." 

The  malar  reflex  is  either  a  muscle  or  a  periosteal  reflex,  my  opinion 
inclining  strongly  to  the  former  view.  In  normal  conditions  percussion 
over  the  malar  bone  may  cause  a  slight  twitching  of  the  upper  angle 
of  the  mouth  on  the  same  side,  but  ordinarily  produces  no  muscular 
response.  In  cases  of  recent  facial  paralysis  of  peripheral  origin  the 
myotatic  irritability  of  the  degenerating  muscles  is  exalted,  and  per- 
cussion at  this  point  causes  contraction  of  the  elevator  of  the  upper 
angle  of  the  mouth  and  movements  of  the  ala  of  the  nose.  If  the  degen- 
eration— as  is  usually  the  case — is  at  all  pronounced,  the  movements 
will  be  exceedingly  sluggish,  the  angle  of  the  mouth  moving  up  slowly, 
and  slowly  resuming  its  previous  position.  In  facial  palsy  of  central 
origin  the  malar  reflex  occurs  more  frequently  than  in  health,  and  the 
movements  are  much  quicker.  Clinically,  the  presence  of  the  malar 
reflex  in  peripheral  facial  paralysis  merely  indicates  that  the  muscle 
is  not  wholly  degenerated,  a  fact  which  can,  of  course,  be  ascertained 
by  the  electric  current. 

The  chin-jerk  is  produced  by  tapping  downward  upon  the  teeth  when 
the  lower  jaw  is  relaxed  and  partially  open.  For  this  purpose  either 
a  small  flat  object,  such  as  tongue-depressor,  may  be  rested  upon  the 
lower  teeth,  or  the  finger  placed  over  the  mental  prominence.  If  the 
reflex  occurs  there  is  a  sharp  upward  movement  of  the  lower  jaw.  It 
appears  doubtful  whether  this  is  a  reflex.  It  is  unquestionably  exag- 
gerated in  cases  of  myotatic  irritation,  such  as  cachexia,  and  I  have 
obtained  it  in  deep  ether  anaesthesia.  It  is  also  likely  to  be  increased 
in  functional  nervous  conditions,  such  as  hysteria  with  exaggeration 
of  the  reflexes.  It  does  not  appear  to  be  altered  in  any  characteristic 
way  as  a  result  of  cerebral  lesions,  and  bilateral  peripheral  lesions  of 
the  motor  nerves  that  would  be  involved   (facial,  trigeminal)   are  so 


918  THE    EYE    AND    NERVOUS    SYSTEM 

rare  that  there  are  no  recorded  observations  regarding  their  influence 
upon  it.  It  may,  therefore,  be  regarded  as  of  no  clinical  significance. 
It  may  also  be  elicited  by  tapping  upon  the  insertion  of  the  masseter 
(von  Bechterew). 

Other  reflexes  of  the  head  involve  particularly  irritations  arising 
from  the  mucous  membrane  in  the  nose  and  throat.  These  include  the 
various  nasal  and  pharyngeal  and  palatal  reflexes.  Their  discussion, 
however,  does  not  come  within  the  scope  of  this  article.  Von  Solder 
has  described  a  lateral  movement  of  the  jaw  when  the  cornea  is  irri- 
tated. Pressure  upon  the  eyeball  through  the  eyelid  may  produce  the 
same  effect  (Fuchs). 

REFLEXES  OF  THE  UPPER  EXTREMITY. 
The  bicipital  reflex  is  a  tendon  reflex  that  is  obtained  by  striking 
upon  the  radial  tendon  of  the  biceps,  producing  a  contraction  of  the 
muscle  and  slight  lifting  of  the  upper  arm.  The  reflex  is  best  elicited 
in  the  following  manner.  The  forearm  is  flexed  upon  the  arm  at  little 
more  than  a  right  angle.  The  arm  is  then  grasped  just  above  the  elbow 
in  such  a  manner  that  the  thumb  rests  upon  the  biceps  tendon.  This 
is  then  slightly  compressed  and  a  blow  struck  upon  the  dorsum  of  the 
thumb.  It  is  often  convenient  to  allow  the  arm  of  the  subject  to  rest 
upon  that  of  the  examiner.  The  biceps  is  a  true  tendon  reflex.  Its  arc 
appears  to  consist  of  the  sensory  nerve-fibres  passing  from  the  muscle 
to  the  fourth  and  fifth  cervical  segments  of  the  spinal  cord,  and  then 
downward  along  the  brachial  plexus  and  tlie  musculo-cutaneous  nerve. 
The  reflex  is  not  invariably  present  in  health.  It  is  usually  exaggerated 
in  functional  nervous  conditions  associated  with  a  general  increase  in 
the  reflex  activity,  and  is  almost  invariably  increased  in  lesions  of  the 
brain  involving  the  motor  mechanism  of  the  arm.  It  is  lost  in  lesions 
of  the  posterior  columns  of  the  cervical  cord,  as  in  late  tabes  dorsalis 
or  in  the  cervical  form  of  this  disease ;  possibly  even  more  constantly 
than  the  patellar  reflex.  It  is  also  lost  in  cases  of  destruction  of  the 
peripheral  nerves,  such  as  injury  to  the  brachial  plexus.  It  diminishes 
in  proportion  to  the  wasting  of  the  muscle  in  the  various  forms  of  myop- 
athy that  involve  the  muscles  of  the  arms.  Owing  to  its  occasional 
absence  in  perfectly  healthy  individuals,  inability  to  obtain  it  is  not 
of  great  clinical  significance  unless  it  is  unilateral.  Exaggeration 
of  this  reflex,  if  associated  with  paresis  or  paralysis  of  the  arm,  may 
be  regarded  as  a  definite  sign  of  injury  to  the  motor  portion  of  the  cere- 
bral hemisphere  of  the  opposite  side.  Bicipital  and  tricipital  clonus 
are  very  rare. 


EEFLEXES  919 

The  tricipital  reflex  consists  of  contraction  of  the  triceps  muscle  when 
its  tendon  is  struck  a  sharp  blow.  It  is  best  elicited  by  bending  the 
forearm  upon  the  arm  at  about  a  right  angle  and  then  striking  just 
above  the  prominence  of  the  olecranon.  If  the  reflex  is  present  there 
will  be  a  sharp  contraction  of  the  muscle  and  extension  of  the  arm. 
Care  must  be  taken  not  to  strike  upon  the  muscle.  This  reflex  is  in  all 
respects  closely  analogous  to  the  biceps  and  is  subject  to  the  same  modi- 
fications by  various  lesions.  It  occurs  in  about  two-thirds  of  all  healthy 
persons. 

The  supinator  reflex  is  a  contraction  of  the  supinator  muscle  when 
a  blow  is  struck  upon  the  tendon  as  it  passes  over  the  radial  bone.  It 
is  best  elicited  by  having  the  forearm  flexed  upon  the  arm  and  at  rest 
There  is  usually  slight  flexion  and  supination  of  the  arm.  There  is 
some  doubt  as  to  whether  this  is  really  a  tendon  and  not  a  periosteal 
reflex.  It  is  absent  in  about  one-eighth  of  all  healthy  persons,  is  likely 
to  be  exaggerated  in  functional  nervous  disease,  and  is  occasionally 
exaggerated  on  the  paralyzed  side  in  cerebral  hemiplegia.  It  is  of  little 
clinical  significance. 

Striking  upon  the  extensor  or  flexor  muscles  of  the  forearm  causes 
them  to  contract,  giving  rise,  respectively,  to  extension  and  flexion  of  the 
fingers.  This  is  not  a  reflex,  but  merely  an  expression  of  muscular  irri- 
tability, the  reaction  occurring  most  distinctly  in  states  of  cachexia,  and 
in  conditions  in  which  the  muscles  are  easily  stimulated  mechanically. 

The  hypothenar  reflex  consists  of  a  contraction  of  the  abductor  of 
the  little  finger  when  pressure  is  made  over  the  pisiform  bone  or  when 
the  end  of  the  little  finger  is  pinched.  The  contraction  is  usually  tonic 
in  character  and  is  indicated  by  the  appearance  of  a  groove  on  the  ulnar 
side  of  the  hand.  Occasionally  there  are  a  series  of  light  twitches  in 
the  tonically  contracted  muscle.  It  is  not  constant,  and  nothing  defi- 
nite is  known  regarding  the  conditions  that  cause  it  to  be  increased — 
if  such  a  change  could  possibly  be  recognized — or  to  be  lost.  I  have 
observed  it  in  conditions  of  anaesthesia  of  the  hand,  such  as  unilateral 
anaesthesia  from  hysteria,  and  in  anaesthesia  associated  with  hemiplegia 
of  cerebral  origin. 

In  some  cases  a  palmar  reflex  may  be  obtained. 

REFLEXES    OF   THE    LOWER    EXTREMITY. 
Tendon  Reflexes. — The  knee-jerk  is  a  contraction  of  the  quadriceps 
tendon  in  response  to  a  blow  struck  upon  the  patellar  tendon,  and  was 
first  described  by  Erb  and  Westphal.     Clinically  it  is  the  most  impor- 
tant of  all  the  reflexes,  and,  as  one  of  the  flrst  known,  has  been  most 


920  THE    EYE    AND    NERVOUS    SYSTEM 

extensively  studied.  It  may  be  elicited  in  a  variety  of  wayg.  If  the 
patient  is  lying  on  tlie  back  the  hand  should  be  placed  under  the  knee 
and  lifted  until  the  angle  formed  by  the  thigh  and  leg  is  about  120°. 
A  sharp  blow  is  then  struck  upon  the  patellar  tendon  either  with  the 
finger,  tlie  side  of  the  hand,  or  a  percussion  hammer.  There  is  a  sudden 
contraction  of  the  quadriceps  which  causes  the  patella  to  move  upward, 
and,  if  sufficiently  vigorous,  raises  the  foot  slightly  from  the  bed  as  a  re- 
sult of  a  forward  kick.  If  the  patient  is  sitting  in  a  chair  one  thigh  may 
be  thrown  over  the  other  and  the  leg  allowed  to  hang  loosely.  A  blow 
is  tlien  struck  as  before  and  almost  always  results  in  a  slight  forward 
kick.  If  the  patient  sits  on  the  edge  of  a  table  and  allows  the  legs  to 
swing  the  reflex  can  often  be  obtained  very  readily.  In  certain  cases 
of  extreme  muscular  atrophy,  as  a  result  of  emaciation,  the  tendon  may 
be  so  loose  that  the  reflex  cannot  be  obtained  in  any  of  the  ordinary 
positions.  Under  these  circumstances,  if  the  leg  is  flexed  strongly 
upon  the  thigh,  it  may  be  possible  to  stretch  the  tendon  sufficiently 
to  obtain  a  slight  muscular  contraction  when  it  is  struck.  If  the  reflex 
is  so  diminished  that  it  cannot  be  obtained  by  any  of  these  procedures 
it  may  be  re-enforced  according  to  the  method  of  Jendrassik.  This 
depends  essentially  upon  the  fact  that  anything  in  the  form  of  mus- 
cular or  intellectual  effort  increases  the  vigor  of  the  reflex.  It  is  there- 
fore customary  to  give  the  patient  some  simple  task  to  perform,  such, 
for  example,  as  an  attempt  to  pull  apart  the  hands  with  the  fingers 
locked  together.  At  the  same  time  he  should  be  instructed  to  look  away 
from  the  knee.  Sometimes  it  is  sufficient  to  give  him  a  simple  calcu- 
lation in  mental  arithmetic.  A  convenient  modification  is  to  have  him 
grasp  a  dynamometer  or  the  physician's  left  hand  ;  the  physician  counts, 
"  one,  two,  three,"  the  grasp  and  the  blow  being  performed  as  nearly  as 
possible  at  the  same  moment. 

After  the  stimulus  is  applied  there  is  perceptible  interval  before  the 
muscular  contraction  occurs,  which  is  called  the  latent  period  or  reflex 
interval.  It  has  been  measured  by  numerous  observers  working  with 
a  great  variety  of  instruments  and  in  normal  persons  is  probably  about 
0.03  second.  It  varies  considerably,  however,  in  different  individuals, 
and  in  the  same  individual  under  different  circumstances.  It  is  short- 
ened by  conditions  that  increase  reflex  activity;  and  by  mental  condi- 
tions that  diminish  inhibition,  microcephaly,  mania  and  epilepsy. 
The  duration  of  the  contraction  is  also  variable  (0.1-0.5  second).  It 
is  greatly  increased  in  spastic  conditions.  The  curve  of  the  muscular 
contracture  has  also  been  studied,  but  has  yielded  no  information  of 
importance  regarding  reflexes. 


REFLEXES  921 

In  testing  the  knee-jerk  it  is  not  sufficient  merely  to  watch  the 
excursion  of  the  leg.  I^Tot  infrequently,  if  the  muscles  are  weak,  no 
movement  in  the  leg  is  produced,  and  yet  the  reflex  may  be  actually 
exaggerated,  as  is  shown  not  merely  by  the  violent  contraction  of  the 
muscle,  but  also  by  the  involvement  of  other  groups  of  muscles  in  the 
thigh.  It  is  therefore  always  advisable  to  rest  one  hand  upon  the  mid- 
dle of  the  upper  surface  of  the  thigh.  Occasionally  the  reflex  can  be 
detected  by  placing  the  finger  upon  the  tendon  and  striking  a  blow  upon 
the  finger,  the  twitching  produced  by  the  muscle  being  then  readily 
perceived.  Instruments  have  been  constructed  for  measuring  the  knee- 
jerk.  They  consist  of  an  arc  having  apj)roximately  the  radius  of  the 
length  of  the  leg,  with  an  indicator  that  is  thrust  forward  by  the  kick 
of  the  foot.  As  it  has  been  explained  that  the  kick  is  not  propor- 
tionate to  the  activity  of  the  reflex,  it  will  be  obvious  that  none  of 
them  is  of  any  value.  At  most  they  may  serve  to  indicate  an  increased 
degree  in  the  activity  of  the  reflex  in  the  same  individual,  and  there- 
fore may  be  useful  in  experimental  work  to  determine  the  effect  of 
various  influences.  It  is  always  advisable,  but  rarely  convenient,  to  test 
the  knee-jerk  with  the  thighs  bare.  Very  frequently  there  is  slight 
twitching  of  the  adductors  on  the  same  side,  and  occasionally  of  the 
adductors  or  of  the  quadriceps  on  the  opposite  side.  This  is  nearly 
always  true  if  there  is  much  exaggeration  of  the  reflex.  In  estimating 
the  reflex  we  have  no  standard  of  measurement,  and  are  accustomed  to 
use  the  terms  "  normal,"  "  diminished,"  "  absent,"  "  increased,"  and 
"  greatly  increased"  for  the  purpose  of  indicating  roughly  its  degree. 
Unfortunately,  the  degree  of  variation  is  considerable  in  different  indi- 
viduals, and  even  at  different  times  in  the  same  individual.  The  fol- 
lowing points  may  be  of  some  value  in  the  estimation. 

The  reflex  is  absent  if,  when  the  muscles  are  completely  relaxed, 
and  the  patient  is  intelligent  enough  to  employ  the  method  of  Jendras- 
sik,  and  the  physician  is  certain  that  the  blow  is  struck  over  the  patellar 
tendon, — that  is,  between  the  lower  edge  of  the  patella  and  the  tubercle 
of  the  tibia, — no  contraction  of , the  muscles  takes  place.  It  is  desirable 
that  the  leg  should  be  carefully  inspected,  and  at  the  same  time  that 
the  free  hand  should  be  laid  over  the  muscles  of  the  thigh  in  order  to 
perceive  a  contraction  that  might  be  invisible. 

The  reflex  is  certainly  diminished  if  under  ordinary  conditions  it 
cannot  be  obtained,  and  the  response  is  slight  when  re-enforcement  is 
employed.  A  visible  response  in  a  subject  who  has  not  previously 
been  tested  cannot  be  regarded  as  an  indication  of  any  pathological 
process. 


922  THE    EYE    AND    NERVOUS    SYSTEM 

The  knee-jerk  is  certainly  exaggerated  if  a  slight  tap  upon  the  tendon 
causes  the  leg  to  fly  out  in  an  almost  straight  line;  frequently,  under 
tliese  circumstances,  two  or  more  distinct  twitches  may  be  felt  in  the 
quadriceps  muscle;  if  other  muscles  of  the  thigh,  or  of  the  opposite 
thigh,  are  involved;  if  there  is  patellar  clonus;  if  the  latent  period 
is  shortened  and  the  duration  of  the  muscular  contraction  lengthened, 
or  if  it  is  elicited  by  tapping  on  the  periosteum  of  the  tibia. 

The  knee-jerk  is  said  to  occur  in  all  normal  individuals.  There  is 
no  doubt,  however,  that  the  limits  of  variation  are  very  wide.  In  some 
persons  apparently  normal  there  are  times  when  it  can  only  be  obtained 
by  re-enforcement.  In  others  it  is  invariably  abnormally  active.  It  is  sub- 
ject to  great  variation  in  disease.  It  is  usually  lost  in  cases  of  disease 
of  the  posterior  columns,  as,  for  example,  in  tabes  and  Friedreich's 
ataxia.  In  tabes  it  is  absent  on  both  sides  in  about  70  per  cent,  of  all 
cases,  and  in  about  15  per  cent,  more  is  pathologically  altered.  It  may, 
however,  even  when  lost,  reappear  in  the  later  stages  of  the  disease. 
It  is  lost  in  cases  of  total  destruction  of  the  peripheral  nerves.  It 
is  reduced  proportionately  to  the  degeneration  of  the  muscles  in  the 
various  forms  of  myopathy.  It  is  lost  in  cases  of  degeneration  of  the 
ganglion  cells  in  the  anterior  comua  of  the  cord  that  control  the  quad- 
riceps muscle.  In  partial  injury  of  the  peripheral  nerves,  such  as  occurs 
in  multiple  neuritis,  it  is  nearly  always  absent  or  greatly  diminished; 
but  in  a  few  instances,  whose  nature  is  not  clearly  understood,  it  may 
be  exaggerated.  It  is  somewhat  variable,  also,  in  total  transverse  lesion 
of  the  cord.  In  the  majority  of  recorded  cases  it  has  been  entirely 
abolished  by  total  transverse  lesions  in  the  dorsal,  or  even  in  the  lower 
cervical,  region,  but  a  few  cases  have  been  recorded  in  which  it  persisted 
and  was  even  exaggerated.  Reference  has  already  been  made  to  this 
subject.  There  is  no  constant  change  in  incomplete  transverse  lesion 
above  the  lumbar  enlargement. 

The  knee-jerks  are  always  exaggerated  if  there  is  degeneration  of 
the  central  motor  neurones  without  degeneration  of  other  parts  of  the 
spinal  cord ;  and  there  may  be  actual  clonus  of  the  quadriceps  muscle 
when  the  patellar  tendon  is  struck,  giving  rise  to  a  series  of  kicks  of  the 
leg,  and  these  kicks,  or  at  last  the  first  one  or  two,  may  be  so  violent  that 
the  leg  is  extended  ad  maximum.  Contractures  of  the  adductors  and 
occasionally  of  tlie  quadriceps  of  the  other  leg  are  also  nearly  always 
present.  Clonus  of  the  patella  usually  co-exists  and  may  be  of  a  perma- 
nent character, — that  is,  continue  as  long  as  the  pressure  is  maintained. 
This  degeneration  of  the  lateral  columns  occurs  in  the  doubtful  primary 
lateral  sclerosis,  and  in  cases  of  various  cerebral  lesions  involving  the 


EEFLEXES  923 

motor  tract.  It  occurs  in  the  apparently  idiopathic  degenerative  disease 
of  the  cord  known  as  amyotrophic  lateral  sclerosis.  In  syringomyelia  or 
in  partial  lesions  of  the  dorsal  or  cervical  cord  arising  from  pressure  as 
a  result  of  disease  of  or  of  injury  to  the  spinal  column,  the  knee-jerk  is 
usually  increased,  the  increase  ranging  from  a  moderate  exaggeration  to 
(rarely)  the  extreme  spastic  type.  In  nearly  all  these  cases  there  are 
associated  sensory  disturbances  which  aid  in  rendering  the  diagnosis 
clear.  These  may  be  para?sthesia,  often  in  the  form  of  a  girdle  sensation 
at  the  level  of  the  lesion,  dissociation  of  sensation,  as  a  rule  more  pro- 
nounced at  first  in  the  foot,  but  often  ascending;  a  partial  or  complete 
an£esthesia,  the  latter  sometimes  involving  all  forms  of  sensation  and 
limited  by  a  horizontal  line  extending  around  the  trunk  at  the  site  of  the 
lesion.  A  distinction  may  be  drawn  between  the  exaggeration  of  the 
patellar  reflexes  with  and  without  paralysis.  In  cases  of  hemiplegia  the 
knee-jerk  is  usually  greatly  increased  on  the  paralyzed  side,  in  92  per 
cent,  of  the  cases,  and  moderately  increased  on  the  sound  side,  the 
contractility  of  the  muscle  being  not  in  the  least  affected  by  the  absence 
of  voluntary  innervation.  In  the  first  few  days  the  reflex  may  be 
diminished  or  lost.  Striimpell  has  observed  a  diminution  in  old  hemi- 
plegias. In  the  syndrome  to  which  the  name  primary  spastic  paraplegia 
was  formerly  given,  the  power  of  the  muscles  may  be  only  slightly  im- 
paired, but  their  extreme  spasticity  renders  it  difficult  for  the  patient 
to  control  them.  In  lesions  of  the  upper  portion  of  the  cord  the  paralysis 
may  be  complete  and  yet  the  reflex  be  exaggerated.  In  functional  ner- 
vous conditions,  such  as  hysteria  and  neurasthenia,  the  knee-jerks  are 
nearly  always  increased ;  although  very  rarely  they  may  be  absent  and 
hypotonia  be  present.  There  is  no  clonus  and  the  patellar  jerk,  as  a 
rule,  does  not  show  nearly  the  same  degree  of  exaggeration.  The  power 
and  voluntary  control  of  the  muscles  in  these  cases  may  be  intact.  In 
general  diseases  of  the  brain  the  knee-jerks  are  variably  affected.  In 
cases  of  disease  of  the  cerebellum  or  frontal  region  they  may  be  in- 
creased or  decreased,  and  periods  of  increase  and  decrease  may  alternate 
in  the  same  case.  In  cases  of  disease  of  the  cerebral  hemispheres  asso- 
ciated with  increased  intracranial  pressure,  as,  for  example,  tumor, 
hydrocephalus,  or  meningitis,  the  knee-jerks,  as  a  rule,  are  increased. 
In  cases  of  brain  tumor,  careful  statistics  indicate  that  they  may  be  of 
some  aid  in  local  diagnosis,  although  their  behavior  is  not  constant. 
Miiller,  in  fifty-five  cases  of  tumor  of  the  frontal  lobes,  found  the 
patellar  tendon  reflex  absent  eleven  times,  normal  nineteen  times,  and 
increased  twelve  times;  in  two  cases  it  was  lost  on  the  side  opposite 
to  that  of  the  tumor.     Williamson  found  it  absent  in  20  per  cent,  of 


924  THE    EYE    AND    NERVOUS    SYSTEM 

forty-five  cases  of  lesion  of  the  frontal  lobes.  It  is  apparently  absent  in 
about  3  per  cent,  of  tumors  in  other  parts  of  the  cerebrum.  Reh  finds  it 
diminished  in  34  per  cent,  of  all  cases  of  brain  tumors.  In  tumors  of 
the  cerebellum  it  is  lost  in  41  per  cent,  of  the  cases  according  to  Voss, 
and  in  29  per  cent,  of  tlie  cases,  according  to  Dinkelburg.  In  a  case 
that  I  observed  it  disappeared  and  reappeared  twice.  The  abdominal 
reflex  is  usually  preserved,  but  this  appears  to  be  of  no  value  as  a  dif- 
ferential sign.  In  chronic  encephalitis — that  is,  in  general  paresis — 
the  knee-jerks  are  usually  increased,  but  occasionally  are  diminished  or 
absent,  especially  after  flaccid  apoplectic  attacks.  The  cases  in  which 
this  occurs  are  usually  recorded  as  the  tabetic  form,  and  lesions  of  the 
posterior  columns  of  the  cord  are  found  at  autopsy.  Sometimes  they  are 
unequal.  In  insanity  there  is  no  characteristic  change  in  the  knee-jerk. 
In  epilepsy  the  knee-jerks  are,  as  a  rule,  increased,  particularly  in  those 
congenital  epileptics  whose  brains  show  actual  lesions.  In  shock  they 
are  usually  lost,  and  for  this  reason  are  often  absent  immediately  after 
an  epileptic  attack.  In  uraemia  they  may  be  increased  (Fiirstner).  In 
certain  general  diseases  the  knee-jerks  may  be  altered.  In  diabetes  they 
are  usually  lost;  in  febrile  conditions,  especially  typhoid  and  tuber- 
culosis, they  are  increased.  In  croupous  pneumonia  it  has  been  asserted 
and  denied  that  they  are  diminished.  WTien  two  conditions  co-exist  that 
affect  the  reflex  in  opposite  ways,  the  result  is  variable.  It  has  been 
observed  to  appear  in  the  paralyzed  limb  in  hemiplegia  occurring  in  the 
course  of  tabes  (Westphal).  It  is  now  generally  admitted  that  the  arc 
is  completed  in  the  third  and  fourth  lumbar  segments,  with  occasional 
variation  to  a  segment  above  or  below.  There  have  not  been  wanting 
investigators,  however,  who  believe  that  the  arc  is  completed  in  seg- 
ments at  higher  levels,  or  even  in  the  brain,  but  little  importance  can  be 
attached  to  their  views.  According  to  Sherrington,  the  sensory  nerve 
in  apes  is  the  branch  leading  from  the  vastus  internus  muscle  to  the  lum- 
bar plexus.  After  section  of  all  other  sensory  nerves,  excepting  this,  the 
reflex  can  still  be  produced.  The  motor  nerves  are,  of  course,  those 
passing  to  the  different  members  of  the  quadriceps  and  adductor  muscles 
on  either  side. 

The  Patellar  Reflex. — This  consists  of  a  sharp  contraction  of  the 
quadriceps  muscle  drawing  up  the  patella  when  that  bone  is  forced 
do^vnward  by  a  blow.  In  other  words,  it  is  a  reaction  of  the  quadriceps 
muscle  to  sudden  extension.  The  reflex  is  best  elicited  if  the  leg  is 
extended.  The  patella  may  either  be  grasped  between  the  thumb  and 
index-finger  and  pushed  forcibly  down,  or  the  finger  may  be  laid  trans- 
versely across  the  upper  border  and  struck  a  smart  blow  with  the  per- 


REFLEXES  925 

cussion-hammer ;  or  some  round  object,  such  as  a  lead-pencil,  may  replace 
the  finger,  or  the  ingenious  instrument  devised  by  Mills,  which  consists 
essentially  of  a  ring  encircling  the  patella,  the  loAver  end  provided  with 
a  handle  and  the  upper  end  with  a  slight  enlargement  upon  which  the 
blow  is  struck.  The  patellar  jerk  is  not  nearly  so  constant  as  the  knee- 
jerk.  It  occurs,  however,  in  the  majority  of  normal  individuals.  In 
cases  of  spasticity  of  the  muscles  of  the  thigh  the  jerk  is  violent  and 
may  be  repeated  in  the  form  of  a  tonic  spasm,  known  as  patellar  clonus. 
This  reflex  has  the  same  significance  as  the  knee-jerk,  but  may  be  tested 
with  advantage  in  patients  who,  through  disease,  stupidity,  or  ignor- 
ance- of  the  language,  cannot  or  will  not  relax  the  leg  sufiiciently  to 
obtain  a  satisfactory  test  of  the  knee-jerk.  As  clonus  indicates  disease 
of  the  pyramidal  columns,  it  is  of  considerable  clinical  importance. 

The  adductor  reflex  consists  of  a  sudden  contraction  of  the  adductor 
muscles  on  the  inner  side  of  the  thigh  when  a  blow  is  struck  just  above 
the  internal  condyle  of  the  femur.  There  is  usually  a  slight  adduction 
of  the  thigh  and  occasionally  a  slight  painful  sensation.  The  reflex  is 
best  obtained  if  the  thigh  is  flexed  at  about  a  right  angle  upon  the  trunk 
and  the  knee  flexed  upon  the  thigh.  It  is  not  invariably  present  in 
health,  about  34  per  cent.,  but  is  always  present  in  spastic  conditions, 
or,  in  fact,  in  any  condition  that  gives  rise  to  exaggeration  of  the  knee- 
jerk.  Reflexes  may  also  be  occasionally  obtained  by  striking  upon  the 
hamstring  tendons.  For  this  purpose  the  patient  should  lie  upon  the 
face,  the  leg  should  be  lifted  from  the  bed  by  an  assistant  until  the 
tendons  of  the  biceps  or  of  the  semimembranosus  and  semitendinosus 
are  relaxed,  then  the  tendon  to  be  tested  should  be  moderately  com- 
pressed by  the  thumb  upon  the  dorsum  of  which  a  sharp  blow  is  struck 
Avitli  a  hammer.  For  some  reason  these  reflexes  have  never  excited  the 
interest  of  clinicians  and  are  rarely  tested.  It  is  not  knowTi  whether 
they  are  present  in  all  normal  persons,  but  they  appear  to  be  subject  to 
the  same  conditions  of  increase  and  decrease  as  are  the  other  tendon 
reflexes  of  the  leg. 

The  Achilles-tendon  reflex  consists  of  a  sharp  contraction  of  the  mus- 
cles of  the  calf  if  a  blow  is  struck  upon  the  Achilles  tendon  in  a  state 
of  moderate  tension.  To  elicit  this  reflex  the  leg  should  be  flexed  upon 
the  thigh  at  about  right  angles ;  the  foot  is  then  forcibly  thrust  upward 
until  the  tendon  is  tense,  when  it  is  struck  a  sharp  blow.  There  is  a 
contraction  of  the  soleus  and  more  or  less  extension  of  the  foot.  It 
seems  to  be  generally  admitted  now  that  this  reflex  is  produced  only 
by  the  soleus  muscle,  and,  in  fact,  is  more  easily  obtained  if  the  gastroc- 
nemius is  relaxed.    Both  tlie  afferent  and  the  efferent  fibres  pass  through 


92G  THE    EYE    AND    NERVOUS    SYSTEM 

the  tnmk  of  the  sciatic  nerve.  The  arc  is  completed  in  the  fifth  lumbar 
and  first  sacral  segments.  The  reflex  is  very  constant,  occurring,  accord- 
ing to  Bramwell  and  KoUarits,  in  all  healthy  persons  under  fifty  years, 
and  according  to  Strassburger  in  07  per  cent,  of  healthy  persons.  It 
disappears  in  disease  of  the  posterior  columns  or  of  the  posterior  roots 
of  the  lumbar  and  sacral  regions  of  the  cord,  sometimes,  indeed,  before 
the  knee-jerk.  Therefore,  its  loss,  even  if  only  unilateral,  may  be  an 
early  sign  of  tabes,  or  even  general  paresis.  It  is  also  lost  in  disease  of 
the  cord  which  contains  the  arc.  It  is  often  absent  in  sciatica,  in  which 
disease  the  knee-jerk  usually  persists,  and  when  absent  may  indicate 
an  actual  neuritis.  Under  these  circumstances  the  prognosis  is  graver. 
It  is  usually  lost  in  complete  transverse  lesion  of  the  spinal  cord  above 
the  level  of  the  arc.  Occasionally  when  it  persists  and  the  knee-jerk  is 
lost,  or  if  it  is  lost  and  the  knee-jerk  persists,  in  cases  of  traumatism  or 
focal  disease  of  the  lower  portion  of  the  cord,  its  study  may  contribute 
materially  to  correct  localization.  It  is  exaggerated  in  all  conditions 
that  give  rise  to  exaggeration  of  the  knee-jerk. 

Ankle-clonus  is  a  manifestation  of  the  Achilles-tendon  reflex  pro- 
duced by  a  slightly  different  technique.  The  leg  is  moderately  flexed 
upon  the  thigh,  the  muscles  completely  relaxed,  and  the  foot  then  thrust 
forcibly  upw^ard  and  the  pressure  maintained.  Normally,  after  the 
sudden  extension  of  the  muscle  there  is  a  sharp  contraction,  perhaps 
followed  by  a  slighter  one.  In  certain  functional  neuroses  or  in  states 
of  fatigue  there  may  even  be  a  series  of  contractions  diminishing  rapidly 
in  force.  If  there  is  actual  degeneration  of  the  superior  motor  neurones 
there  will  be  a  rhythmical  to-and-fro  movement  of  the  foot  which  will 
persist  as  long  as  the  pressure  is  continued,  sometimes  decreasing,  some- 
times increasing  in  rapidity  and  in  force,  this  variation  occasionally 
being  quite  rhythmical  in  character.  Ankle-clonus  is  an  almost  certain 
sign  of  lesions  of  the  central  nervous  system ;  indeed,  it  has  been  seri- 
ously doubted  by  many  competent  authorities  (Mills)  whether  or  not 
it  occurs  in  functional  nervous  disease,  although  there  appears  to  be 
no  doubt  that  in  rare  instances  it  does  so  occur ;  but  the  very  fact  that 
these  instances  are  so  uncommon  leads  unavoidably  to  the  suspicion 
that  temporary  structural  lesions  may  exist.  In  rare  conditions  it  may 
be  present  when  the  knee-jerk  is  absent.  A  form  of  ankle-clonus 
may  be  produced  in  perfectly  healthy  persons  if,  when  sitting  at  such 
a  height  that  the  foot  rests  easily  upon  the  ground,  it  is  lifted  upon 
the  toe  and  a  series  of  voluntary  oscillations  is  made.  After  a  longer 
or  shorter  time  the  oscillations  become  involuntary,  and  there  is  a  true 
clonic  spasm  that  usually  ceases  spontaneously  in  a  short  time.     In 


EEFLEXES  927 

states  of  fatigue,  cold,  or  exhaustion  from  prolonged  sickness  it  may 
merely  be  necessary  to  rest  the  ball  of  the  foot  upon  the  ground.  It 
is  important  to  differentiate  between  true  ankle-clonus  and  this  form 
which  occurs  in  states  of  impaired  muscular  activity. 

The  Skin  Reflexes. — These  are  of  considerable  importance. 

The  plantar  reflex  is  produced  by  tickling  the  sole  of  the  foot.  There 
is  a  sudden  withdrawal  upward  of  the  leg  as  a  result  of  flexion  at  the 
hip  and  knee,  dorsal  flexion  at  the  ankle,  and  plantar  flexion  of  the  toes. 
It  therefore  simulates  a  co-ordinated  movement  of  escape,  involuntary 
in  character,  produced,  apparently,  as  well  if  the  patient  is  unconscious 
as  if  conscious.  Xot  infrequently  only  the  toes  move.  The  afferent 
and  efferent  fibres  pass  through  the  sciatic  nerve,  and  the  reflex  arc  is 
completed  in  the  second  and  third  sacral  segments,  although,  on  account 
of  the  involvement  of  the  psoas  and  the  muscles  below  it,  the  motor 
centres  in  the  lumbar  and  lowest  dorsal  segments  must  also  be  involved. 
This  reflex  is  subject  to  great  variations  in  normal  persons.  In  some  it 
is  apparently  absent,  and  others  are  able  voluntarily  to  prevent  its 
occurrence.  The  thickness  of  the  sole  of  the  foot  appears  to  be  of  slight 
influence.  In  functional  nervous  diseases,  particularly  in  hysteria  and 
neurasthenia,  it  is  usually  increased,  and  may  even  be  crossed — that  is, 
occur  in  both  feet  at  the  same  time.  Occasionally,  in  hysterical  states, 
tickling  of  the  sole  of  one  foot  will  cause  a  general  convulsive  movement 
of  the  body,  even  although  the  patient  may  not  perceive  any  tickling 
sensation.  Organic  disease  of  the  central  nervous  system  usually  causes 
a  moderate  increase  in  the  reflex  which,  under  these  circumstances,  is 
limited  to  the  leg  to  which  the  sensory  stimulation  is  applied.  Not  in- 
frequently, however,  the  reflex  is  entirely  lost  in  hemiplegia.  It  is 
invariably  lost  in  disease  of  the  peripheral  sensory  nerves  of  the  legs. 

The  Babinski  phenomenon  was  first  described  in  1897.  According  to 
Schoenborn  it  is  the  only  reflex  of  clinical  value  discovered  since  1875. 
Babinski  observed  that,  when  there  was  disease  of  the  pyramidal  col- 
umns, instead  of  plantar  flexion  of  the  toes  when  the  sole  was  irritated 
there  was  a  slow  dorsal  upward  movement  of  the  great  toe,  sometimes 
accompanied,  sometimes  not  accompanied,  by  the  other  toes.  Xumer- 
ous  investigations  have  only  served  to  confirm  the  importance  and  value 
of  this  reflex.  Its  absence  does  not,  however,  exclude  pyramidal  disease. 
It  is  usually  easily  elicited  by  stroking  longitudinally  on  the  sole  of 
the  foot  with  some  blunt  object,  sometimes  on  the  inner,  but  appar- 
ently preferably  upon  the  outer  side.  Occasionally  the  reaction  is  vio- 
lent, and  then  the  stroke  should  be  very  delicate.  Sometimes  it  is 
necessary  to  use  a  hard  or  fairly  sharp  instrument,  and  to  pass  it  along 


928  THE    EYE    AND    NERVOUS    SYSTEM 

the  more  sensitive  inner  portion  of  the  sole  with  some  force.  There 
is  some  dispute  as  to  whether  a  sudden  dorsal  flexion  of  the  great  toe 
constitutes  the  Babinski  reflex  or  not.  As  it  appears  that  even  this 
sudden  movement  occurs  very  rarely,  if  at  all,  in  normal  persons  or  in 
those  sufi^ering  from  functional  nervous  disease,  it  may  be  accepted  that 
it  is  really  a  pathological  manifestation.  Although  usually  associated 
with  exaggeration  of  the  tendon  reflexes,  this  is  not  invariably  the  case. 
The  value  of  the  Babinski  reflex  compared  with  other  signs  of  disease 
of  the  pyramidal  tracts  in  the  cord  has  been  a  subject  of  considerable 
investigation.  It  appears  to  be  more  constant  than  ankle-clonus,  and 
as  constant  as  the  increase  of  the  knee-jerk.  It  is  said  not  to  occur  if 
the  thalami  have  been  injured,  but  this  lacks  confirmation.  In  my  ex- 
perience, moreover,  it  appears  to  occur  earlier  and  in  certain  cases  to 
persist  after-  the  other  signs  have  disappeared.  In  two  cases  of  valvular 
heart  disease  which  developed  hemiplegia  whilst  under  observation, 
probably  embolic  in  nature,  the  Babinski  reflex  appeared  during  the 
first  six  hours,  and  before  marked  exaggeration  of  the  other  reflexes 
was  apparent.  In  a  case  of  hemiplegia  with  complete  hemiansesthesia 
and  abolition  of  all  the  tendon  reflexes  throughout  the  course  of  the 
disease,  which  lasted  four  months  and  terminated  fatally,  the  Babinski 
reflex  was  occasionally  obtained.  In  three  cases  of  old  hemiplegia  with 
almost  complete  restoration  of  function  and  only  slight  increase  in  the 
knee-jerk  and  an  imperfect  clonus,  the  Babinski  reflex  was  invariably 
obtained  on  the  affected  side.  It  has  been  claimed  that  it  may  occur 
in  functional  nervous  diseases,  especially  hysteria,  epilepsy,  deep  sleep, 
coma,  and  narcosis,  and  in  typhoid  fever.  Miinch-Petersen,  whose  work 
requires  confirmation,  claims  to  have  obtained  it  in  a  considerable  pro- 
portion of  healthy  adults.  Cohn  regards  it  as  of  little  value.  It  often 
occurs  in  normal  infants,  and  therefore  is  of  no  clinical  value  before 
the  age  of  two  years  (Morse).  It  has  been  questioned  whether  a  perma- 
nent dorsal  extension  of  the  great  toe,  such  as  sometimes  occurs  in 
Friedreich's  ataxia  or  old.  hemiplegia,  may  not  be  a  form  of  the  Babin- 
ski j)henomenon.     Its  arc  is  completed  in  the  lower  sacral  region. 

Modifications  of  the  Babinski  Phenomenon. — The  discovery  of  the 
Babinski  reflex  stinuilated  great  interest  in  the  cutaneous  reflexes  in 
general,  and  particularly  in  those  of  the  lower  extremity.  Oppenheim 
has  obtained  dorsal  flexion  of  the  toes  in  pyramidal  disease  by  stroking 
the  inner  side  of  the  leg,  and  his  assistants  have  endeavored — although 
unsuccessfully — to  give  this  the  rank  of  an  independent  reflex. 
Schaefer  has  obtained  the  same  reaction  by  pinching  the  Achilles  ten- 
don.    Ilirschberg  produces  slight  adduction  of  the  foot  somewhat  sim- 


KEFLEXES  929 

ilar  to  the  Babinski  sign,  by  stroking  the  inner  side  of  the  instep.  He 
claims  that  this  reflex  never  occurs  unless  there  is  organic  disease  of  the 
pyramidal  columns,  but  his  statement  has  been  denied  by  Crocq.  Men- 
del and  von  Bechterew  have  described  a  somewhat  analogous  reflex  ob- 
tained by  striking  the  dorsum  of  the  foot.  Under  normal  conditions 
there  is  dorsal  flexion  of  the  second  to  tlie  fifth  toes.  In  pyramidal  dis- 
ease plantar  flexion  of  the  same  toes  may  occasionally  occur. 

The  femoral  reflex  was  described  by  Remak.  It  consists  of  a  plantar 
flexion  of  the  toes  and  extension  of  the  foot  and  leg  if  the  upper  portion 
of  the  anterior  surface  of  the  thigh  is  irritated.  It  only  occurs  in  severe 
lesions  of  the  cord,  such  as  transverse  myelitis  occurring,  above  the  eighth 
dorsal  segment,  the  arc,  according  to  Kemak,  being  completed  in  the 
lower  portion  of  the  dorsal  cord.  It  seems  not  unlikely,  however,  in 
view  of  the  muscles  involved,  that  the  segments  containing  the  arc  are 
really  lower  than  this. 

Unclassified  Reflexes. — Certain  phenomena  whose  exact  nature  has 
not  been  determined,  but  which  are  akin  to  reflex  action,  may  some- 
times be  observed. 

Kernig's  sign  was  first  described  in  1888  in  that  birthplace  of 
many  reflexes,  St.  Petersburg.  It  consists  of  a  spasmodic  contraction  of 
the  biceps,  semimembranosus,  and  semitendinosus  when,  if  the  thigh  is 
flexed  at  right  angles  to  the  trunk,  an  attempt  is  made  to  straighten  the 
leg  upon  the  thigh.  There  is  some  dispute  as  to  the  point  at  which  Ker- 
nig's sign  is  said  to  occur,  Kernig  himself  stating  that  the  reflex  was 
present  if  the  leg  could  not  be  extended  over  an  angle  of  135°  with  the 
thigh.  In  its  most  typical  form,  however,  the  contraction  takes  place 
as  soon  as  the  angle  of  90°  is  reached.  This  reflex  may  be  elicited 
either  by  haviu'  '^  c  patient  sit  in  a  chair  and  then  attempting  to 
pull  forward  the  leg,  or,  if  h:  is  lying  on  his  back,  flexing  the  thigh  upon 
the  trunk  at  right  angles,  and  then  attempting  to  extend  the  leg.  One 
hand  should  be  placed  in  the  popliteal  space  and  the  sudden  tension  of 
the  hamstring  tendons  is  a  very  characteristic  phenomenon.  Kernig 
originally  considered  the  sign  pathognomonic  of  meningitis,  in  practi- 
cally every  form  of  which  he  supposed  it  occurred,  and  subsequent  in- 
vestigations apparently  confirmed  this  opinion  (Friis,  Xetter,  Herrick). 
Of  late,  however,  it  has  been  found  to  occur  in  focal  brain  disease 
(Sailer)  and  in  the  course  of  certain  infectious  fevers  (Shields),  and 
it  does  not  occur  in  all  cases  of  meningitis,  and  in  particular  it  is  likely 
to  be  absent  in  tubercular  meningitis  (Dieulafoy).  It  is,  however,  still 
a  valuable  sign  because,  although  not  pathognomonic,  it  occurs  in  more 
than  90  per  cent,  of  all  cases  of  acute  meningitis,  and  very  rarely  in 
59 


930  THE    EYE    AND    NERVOUS    SYSTEM 

other  conditions.  Therefore,  if  found,  the  probabilities  are  strongly  in 
favor  of  the  existence  of  meningitis. 

A  reflex  whose  nature  is  not  clearly  understood  is  Sinkler's  toe-jerk. 
It  consists  of  forcible  flexion  at  the  hip  and  leg  when  the  great  toe  is 
flexed  strongly  on  the  sole.  It  appears  to  occur  in  states  of  extreme 
spasticity,  and  I  have  been  able  to  elicit  it  several  times  in  cases  of 
transverse  myelitis  or  spastic  paraplegia.  Nothing  appears  to  be  known 
concerning  its  mechanism,  but  the  arc  must  be  completed  in  the  lumbar 
portion  of  the  cord,  and  it  is  probably  one  of  those  vestigial  reflexes, 
only  developing  when  inhibition  is  entirely  removed,  of  which  Striim- 
pell  speaks.  The  excitation  appears  to  be  due  to  traction  upon  the 
extensor  muscles  of  the  foot. 

Front-tap  was  first  described  by  Gowers.  It  is  elicited  as  follows: 
The  leg  is  flexed  slightly  dbrsally  by  the  observer,  and  a  blow  is  struck 
upon  the  tibialis  anticus  muscle.  In  about  40  per  cent,  of  normal  per- 
sons there  is  plantar  flexion  of  the  toes.  The  reflex  appears  to  be 
more  common  in  various  forms  of  functional  nervous  disease,  hysteria, 
neurasthenia,  etc.,  and  to  be  absent  invariably  in  hypotonic  conditions, 
such  as  tabes  dorsalis. 

The  tibialis  phenomenon  (Striimpell)  consists  of  the  contraction  of 
the  tibialis  anticus  muscle  when  the  thigh  is  drawn  towards  the  body. 
As  the  patient  lies  upon  the  back,  one  hand  is  placed  over  the  knee  and 
the  other  over  the  dorsum  of  the  foot.  The  knee  is  then  lifted  by  the 
patient  when,  if  the  reflex  is  present,  the  tense  tendon  of  the  tibialis 
anticus  muscle  is  felt  by  the  hand  over  the  foot.  This  reflex  occurs  in 
cases  of  spastic  hemiparesis,  and  may  be  of  some  practical  value. 

REFLEXES    OF   THE   TRUNK. 

Apparently  there  are  no  tendon-reflexes  connected  with  the  trunk. 
Two  important  skin-reflexes  have  been  described,  the  abdominal  and  the 
cremasteric.  The  abdominal  reflex  consists  of  a  sharp  contraction  of 
the  muscles  on  one  side  of  the  abdominal  wall  when  a  light  stroke  is 
made  from  that  side,  passing  obliquely  downward  and  outward.  This 
reflex  is  produced  by  irritation  of  the  cutaneous  terminations  of  the 
intercostal  nerves  that  continue  downward  from  the  intercostal  spaces 
and  are  distributed  to  the  skin  of  the  abdomen.  The  afl"erent  and  effer- 
ent fibres  pass  through  the  intercostal  nerve  trunks,  and  the  reflex  is 
therefore  completed  in  the  dorsal  region  of  the  spinal  cord.  It  is 
customary  to  recognize  two  parts:  the  reflex  of  the  upper  and  of  the 
loAver  quadrants,  or  the  upper  and  lower  abdominal  reflexes.  There  is 
no  actual  anatomical   or  physiological   dividing  line.       This  reflex  is 


EEFLEXES  931 

present  in  98  per  cent,  of  normal  persons,  but  varies  very  considerably 
in  different  persons  or  in  the  same  person  at  different  times.  It  is  in- 
creased by  the  conditions  that  increase  the  skin  reflexes ;  it  is  abolished 
by  lesions  of  the  spinal  cord  which  destroy  the  dorsal  region.  Some- 
times if  only  one  part  and  not  the  other  is  involved  it  serves,"  in  a  sense, 
as  an  aid  to  localization.  It  is  often  active  in  tabes  dorsalis,  and  may 
be  present  in  cases  of  hysterical  anaesthesia,  but  is  lost  in  cases  of 
organic  anaesthesia  of  the  abdomen.  The  hypogastric  reflex,  recently 
described  by  von  Bechterew,  consists  of  a  deepening  of  the  suprain- 
guinal  region  when  the  skin  of  the  inner  upper  part  of  the  thigh  is  irri- 
tated. According  to  Crocq  it  does  not  occur  exclusively  in  women  as 
was  at  first  believed. 

The  cremasteric  reflex  consists  in  the  upward  jerking  of  the  testicle 
when  the  skin  on  the  inner  surface  of  the  thigh  is  irritated.  It  is  most 
readily  elicited  by  pinching,  scraping,  or  pricking  the  skin  over  the 
adductor  muscles  about  one  inch  below  Poupart's  ligament.  The  up- 
ward movement  of  the  testicle  is  sudden  and  independent  of  any  con- 
traction of  the  scrotum,  but  may  be  associated  with  twitching  of  the 
abdominal  muscles.  The  contractility  of  the  cremasteric  muscle  varies 
extraordinarily  in  different  individuals.  A  small  proportion — esti- 
mated at  1  or  2  per  cent. — have  voluntary  control  over  the  muscle 
and  can  elevate  the  testicle  at  will,  and  in  these  cases  the  reflex  is  pro- 
nounced to  the  degree  of  exaggeration.  In  other  individuals  without 
obvious  lesion  of  the  central  or  peripheral  nervous  systems  the  reflex 
may  be  persistently  absent.  In  other  cases  it  may  be  extremely  varia- 
ble. Under  pathological  conditions,  it  is  increased  in  functional  nervous 
states;  it  is  usually  increased  in  conditions  giving  rise  to  increased 
spasticity,  although  it  may  be  diminished  in  hemiplegia  on  the  affected 
side,  and  is  lost  in  conditions  causing  hypotonicity,  as,  for  example, 
tabes  dorsalis.  It  is  said  to  be  exaggerated  in  sciatica.  It  is  a  reflex 
of  moderate  clinical  importance.  The  contraction  of  the  dartos  muscle 
lining  the  wall  of  the  scrotum  is  occasionally  mistaken  for  the  cremas- 
teric reflex.  It  is,  however,  independent  and  differs  distinctly  in- char- 
acter, being  a  slow  contraction  coming  on  at  an  appreciable  interval 
after  the  skin  has  been  irritated.  It  persists  for  a  short  time,  and  then 
relaxation  ensues.  This  reflex  appears  to  be  independent  of  nearly 
everything  excepting  complete  organic  anaesthesia  of  the  part  irritated. 
It  is  of  no  clinical  importance. 

Von  Bechterew  has  described  a  pyramidalis  reflex,  a  slight  contrac- 
tion in  the  lower  part  of  the  abdomen  just  above  the  symphysis  when 
the  skin  above  this  region  is  irritated. 


932  THE    EYE    AND    NERVOUS    SYSTEM 

The  periosteal  and  muscle  reflexes  are  not  important. 

Scapulohumeral  Reflexes. — Von  Bechterevv  has  described  a  series  of 
reflexes  connected  with  the  scapula,  which  are  best  elicited  by  striking 
on  the  spinal  edge  just  below  the  superior  internal  angle.  This  causes 
a  slight  corttraction  of  the  infraspinatus  and  occasionally  of  the  supra- 
spinatus  muscle,  and  adduction  and  external  rotation  of  the  humerus. 
In  cases  of  hemiplegia  in  which  the  muscles  are  spastic  the  contractions 
may  be  more  extensive,  involving  also  the  trapezius  and  deltoid,  and 
causing,  therefore,  abduction  instead  of  adduction  of  the  arm.  Not 
infrequently  I  have  observed  a  contraction  of  the  muscles  of  the  oppo- 
site shoulder.  It  would  seem,  therefore,  that  the  scapular  reflex  must 
be  a  true  reflex  and  not  merely  the  resiilt  of  muscular  irritation.  There 
is  no  doubt,  however,  that  striking  upon  the  bellies  of  the  spinati  mus- 
cles gives  rise  to  contraction  of  these  muscles  with  results  that  simulate 
the  reflex  under  ordinary  conditions.  Steinhausen  regards  it  as  a 
periosteal  reflex  constant  in  normal  persons. 

Iliac  Reflexes. — Striking  upon  the  crest  of  the  ilium  gives  rise  to 
various  contractures  of  the  muscles  attached  to  the  pelvis.  These  re- 
flexes are  increased  by  all  conditions  giving  rise  to  increase  of  the  mus- 
cular irritability.  They  have  received  very  little  attention  from  neu- 
rologists, and  little  is  known  excepting  the  fact  of  their  existence. 

Negro  has  described  as  the  gluteal  reflex  a  sharp  tetanic  contrac- 
tion of  the  gluteal  muscles  when  pressure  is  made  over  the  sciatic 
foramen  in  cases  of  sciatica.  It  may  assist  the  diagnosis  in  doubtful 
cases. 

Mention  may  be  made  of  the  idiopathic  muscular  contraction,  or 
myoidema.  This  consists  of  a  tonic  spasm  of  isolated  fibres  of  a  muscle 
which  have  been  subjected  to  vigorous  mechanical  irritation.  It  is  best 
elicited  in  the  pectoral  muscles  by  tapping  sharply  upon  them  with  the 
finger  or  hammer.  This  produces  a  small  lump  which  rapidly  dis- 
appears. It  can  also  be  elicited  in  the  biceps  by  pinching  it  sharply, 
a  ridge  appearing  along  tlie  line  of  the  pinch.  This  reaction,  although 
it  can  usually  be  obtained  in  normal  persons  who  are  thin  and  whose 
muscles  therefore  are  not  protected  by  a  layer  of  fat,  is  most  striking 
in  cachectic  conditions.  It  also  is  common  in  certain  forms  of  insanity, 
as  general  paresis,  dementia  prascox;  in  epilepsy,  cerebral  syphilis,  etc. 
It  has  no  clinical  significance,  excepting  as  a  confirmation  of  cachexia, 
which  is,  of  course,  readily  recognizable  by  other  means. 


EEFLEXES  933 


LITERATURE  ON  REFLEXES. 

Reflex   action  was   first  clearly  described   by   Marshall   Hall    (Lectures  on  the 

Nervous  System  and  its  Diseases,  Philadelphia,  1836,  p.  33  et  seq.) .     His  first  com- 
munication  was  made  in   1832    (see  "  Memoires   of  the   Nervous   System,"  London, 

1837).    Among  the  earlier  writers  who  observed  some  of  the  phenomena  upon  which 

the  discovery  of  reflex  action  was  based  may  be  mentioned  VVhytt,  1751;    Prochasky, 

1807;     and   Miiller,   1833.     The   literature  of  this,  as  well   as  of  other   interesting 

discussions,  during  the  early  phases  of  neurology,   is   collected   in  a  curious  book, 

"  Documents    and   Dates   of   Modern   Discoveries   in   the   Nervous    System,    London, 

1839." 

General. 

GowERS.     Med.-Chir.  Trans.,  vol.  Ixii.     [Text-Book  of  Nervous  Diseases. — Ed.] 

Sherrington  and  Jendrassik.  C.  R.  de  la  Sec.  de  Neurol.,  Thirteenth  Internat. 
Cong.,  1900  (quoted  by  von  Kornilow)  ;  Philosoph.  Trans.  Royal  Society  of 
London,  Series  B,  1898,  vol.  cxc.  p.  45;    Neurolog.  Centralbl.,  1900,  p.  979. 

Sternberg.  Die  Sehnenreflexe.  [This  book  contains  a  bibliography  almost  com- 
plete to  the  time  of  its  publication.] 

SxRtJMPELL.  Neurolog.  Centralbl.,  1899,  p.  617;  Deutsche  Zeits.  f.  Nervenheilk., 
vol.  XX.  p.  436. 

Mann.     Monats.  f.  Psych,  u.  Neurol.,  1898,  7,  410. 

MusKENS.     Neurol.  Centralbl.,   1899,  p.    1074. 

LuGARO.  Riv.  di  Patolog.  Nerv.  e  Ment.,  vol.  iii.;  Neurolog.  Centralbl.,  1899,  p. 
264. 

Senator.     Zeitschr.  f.  klin.  Med.,  vol.  xxxv. 

Brauer.     Deutsche  Zeitschr.  f.  Nervenheilk.,  vol.  xviii.  p.  284. 

Fraenkel.     Deutsche  Zeitschr.  f.  Nervenheilk.,  vol.  xiii. 

Habel.     Archiv.  f.  Psych.,  vol.  xxix.  p.  25. 

Marinesco.  La  Semaine  Med.,  1898,  vol.  xviii.  p.  153;  Neurolog.  Centralbl.,  1898, 
p.  818,  criticism  by  Brune. 

Westphal.     Archiv  f.  Psych.,  vol.  xxx.  p.  554. 

Gerhardt.     Neurolog.  Centralbl.,  1898,  p.  612. 

Van  Gehuchten.  Journal  de  Neurologic  et  de  Hypnologie,  1897,  June  26;  1898, 
June  5. 

BiscnoFF.    Wiener  klin.  Wochenschrift,  1896. 

Kron.     Deut.  Zeitschr.  f.  Nervenheilk.,  vol.  xxii.  p.  24. 

Balint.     Deutsche  Zeitschr.  f.  Nervenheilk.,  vol.  xxiii.  p.  178. 

Brasch.     Neurolog.  Centralbl.,   1899,  p.   1115;     Fortschritte  der  Med.,  vol.  xviii. 

Sommer.     Lehrb.  der  Psychopath.  Undersuch.,  Berlin,  1899. 

Stewart.    Journal  of  Physiology,  1897,  p.  61. 

Bartels.     Neurolog.  Centralbl.,  1902. 

Turner.     Journal  of  Nervous  and  Mental  Disease,  1902,  p.  321. 

Collier.    Brain,  Spring,  1904. 

Erb.     Archiv  f.  Psych,  u.  Neurol.,  vol.  v.  p.  792. 

Westphal.     Ibid.,  p.  803. 

Pandy.     Neurolog.  Centralbl..  1904,  p.  449. 

ScHEVEN.     Neurolog.  Centralbl.,  1904,  p.  488. 

Reflexes  of  the  Head. 
Supraorbital  Reflex. 
McCarthy.    Neurolog.  Centralbl.,  1900. 
Von  Bechterew,  Neurolog.  Centralbl.,  1900. 


934  THE    EYE    AND    NERVOUS    SYSTEM 

HUDOVEBNIG.     Nourolog.  C'entralbl.,  1001,  p.  933. 
fSAii.ER.     Philadelphia  Medical  Journal,   1901. 
LuKAcz.     Neurolog.  Centralbl.,  1902. 

Malar  Reflex. 
Von  Bechtebew.     Neurolog.  Centralbl.,  1901,  p.  931. 
Saileb.     Philadelphia  Medical  Journal,   1901. 

Chin-Jerk. 
James.     Scottish  Medical  Journal,  Oct.  1900. 

Arm  Reflexes. 
MoiiR.     Deutsche  Zeits.  f.  Nervenheilk.,  vol.  xix.  p.  197. 
Fkaenkel.     Deutsche  Zeits.  f.  Nervenheilk.,  1900,  p.  197;     1900,  vol.  xvii. 
Rexnie.      Australian    Med.    Gazette,    March,    1900;     Neurolog.    Centralbl.,    1901,   p. 

4.50. 
Weisenburo.     Journal  of  Nervous  and  Mental  Disease,   1903,  p.   681. 

Reflexes   of   Loweb   Extbemity. 
Knee-Jerk. 
Ebb.     Arch.  f.  Psych,  u.  Neurologic,  vol.  v.  p.  792. 
Westphai,.     Ibid.,  p.  803.     Neurolog.  Centralbl.,  1885,  p.  412. 
Bernhardt.     Die  Periph.  Nerven.,  Nothnagel's  System,  vol.  xi.  37. 
Brissaui).     Recherclies  Anat.   Pathologiques  et  Physiologiques  sur  la  Contracture 

Pernianente  des  Heniiplogiques.  Paris,   1880. 
Eulexuerg.     Zeits.  f.  klin.  Med.,  vol.  Iv.  p.  179. 
Sternberg.     Loc.  cit.,  p.  31;    Die  Sehnenreflexe,  p.  30. 

Kollarits.     Zeits.  f.  Nervenheilk.,  vol.  xxiii.  p.  89.      (1000  normal  adults.) 
Blaucii.     Arch.   f.   Psych,   u.   Nervenheilk.,  vol.   xii.    1882,   p.   471.      (700  children; 

absent  five  times. ) 
Berger.      Centralbl.   f.   Nervenheilk.,    1879,   No.   4    (found   it   absent  in    1   per   cent. 

of  normal  persons). 
Pelizaeus.     Arch.  f.  Psych,  u.  Nervenheilk.,  vol.  xiv.  Heft  2. 
Spitzer.     Zeits.  f.  Nervenheilk.,  vol.  xix.  p.  215. 
Shoexbobn.     Ibid.,  vol.  xxi.  p.  273. 
Bbaueb.     Ibid.,  vol.  xviii.  p.  284. 
Balint.     Ibid.,  vol.  xix.  p.  414. 
STBiJMPELL.     Ibid.,  vol.  xvii.  p.  268. 

NoxxE.     Deutsche  Zeits.  f.  Nervenheilk.,  vol.  xxiv.  p.  474. 
Mi'LLEB.     Ibid.,  vol.  xxii.  p.  375. 
Voss.     Ibid.,  vol.  xxi.  p.  43. 
FiNKELBUBG.     Ibid.,  vol.  xxi.  p.  296. 
Reh.     Monats.  f.  Psych,  u.  Neurol.,  vol.  xv.  p.  182. 
Westpiial.     Charit*''  Annalen,  1S99. 

TuMPOWSKi.     Deutsche  Zeit.  f.  Nervenheilk..  vol.  x.   1897. 
Fi-RSTNKK.     Neurolog.  Centralbl..  1898,  p.  607. 
De  Moxtyel.     Annales  Med.-psycholog.,  1897,  March  and  April. 
LuGARO.     Neurolog.  Centralbl.,  1899,  p.  264   (abstracted). 
Adamkiewicz.     Ibid..  1899,  p.  338. 

Gaxault.     Th&se  de  Paris.  1898;    Neurolog.  Centralbl.,   1899.  p.  367. 
Bbuxx.     Neurolog.  Centralbl.,  1899,  p.  518. 
Bbaier.     Milnch.  Med.  Woch.,  1899,  No.  2. 


REFLEXES  935 

Williamson.    Glasgow  Medical  Journal,  ^Tov.  1899. 

LiBEBTixi.     Arch,  de  Med.  exp.  et  d'Anatomie  Pathologique,  Sept.,  1900. 

Dekcum.     Journal  of  Nervous  and  Mental  Disease,  1898,  p.  615. 

Pfaundleb.    Munch.  Med.  Woch.,  1902,  p.  1211. 

ROEDER.     Deut.  Archiv  f.  klin.  Med.,  1903. 

Pandy.     Neurolog.  Centralbl.,  1904,  p.  449. 

Collieb.    Brain,  Spring,  1904. 

Patellar-Jerk. 
GowERS.     Text-Book  of  Nervous  Disease,  2nd  Ed. 
Mills.     Journal  of  Mental  and  Nervous  Disease,  1899,  March. 

Adductor  Reflex. 
Remlinqeb.    Revue  de  Med.,  1901,  p.  72. 

Achilles  Tendon  Reflex. 
Kollabits.     Deutsche  Zeits.  f.  Nervenheilk.,  vol.  xxiii.  p.  89. 
Stbasbubgeb.     Ibid.,  vol.  xvii.  p.  306. 

Lembach.     Ibid.,  vol.  vii.  p.  943   (in  92  per  cent,  of  400  cases  of  tabes). 
Babinski.     Gaz.   des  Hopitaux,   1896;     Bull,  et  mem.  de  la  Soc.  des  HOpitaux  de 

Paris,  1898,  Oct.  21. 
Muskens.     Neurolog.  Centralbl.,  1899,  p.  1084. 
Ziehen.     Deutsche  Med.  Woch.,  1894,  No.  33. 
BiBO.     Deutsche  Zeits.  f.  Nervenheilk.,  vol.  xix.  p.  188. 
Knapp.     Monats.  f.  Psych,  und  Neurol.,  vol.  xvi.  p.  327. 
Remlinger.     Revue  de  Neurologic,  1901,  p.  72. 
Mills.     Journal  of  Nervous  and  Mental  Disease,  1899,  p.  131. 
Lebi  and  Follet.     Neurolog.  Centralbl.,  1901,  p.  481. 
BiEo.     Ibid.,   1901,  p.  686. 

Plantar  Reflex. 
Collieb.     Brain,  Spring,  1899. 
COHN.     Neurolog.  Centralbl.,  1899,  p.  582. 

Babinski  Phenomenon. 
Babinski.     Progr§s  Medicale,   1898,  p.   166;    La  Semaine  Med.,   1898.  July  27. 
Van   Gehuchten.     Journal   de   Neurologic,    1898,   April   5;     Revue   de   Neurologic, 

1899. 
Cbocq.    Ibid.,  1901. 

Langdon.     Cincinnati  Lancet-Clinic,  Feb.  1900. 
ScHOENBORN.     Zeits.  f.  Nervenheilk.,  vol.  xxi.  p.  273. 
Specht.     Monats.  f.  Psych.,  vol.  xix.  p.  81. 
Pfeiffer.     Monats.  f.  Psych.,  vol.  xiv.  p.  270. 

KoENiG.     Arch.  f.  Psych.,  vol.  xxxiii.  p.  311;    Neurolog.  Centralbl.,  1899,  p.  610. 
Passini.     Wien.  klin.  Woch..  1900,  No.  41. 
Schneider.     Berl;  klin.  Woch.,  1901,  p.  946. 
BicKEL.     Deutsche  Zeits.  f.  Nervenheilk.,  vol.  xxii.  p.  24. 
Munch-Petersen.     Deutsche  Zeits.  f.  Nervenheilk.,  vol.  xxii.  p.  177. 
Giudiceandrea   (quoted  by  von  Kornilow). 
Vires  and  Calmettes.     Revue  de  Neurologic,   1900. 
Von  Kornilow.    Zeits.  f.  Nervenheilk..  vol.  xxiii.  p.  216. 
Glorieux.     Policlinique,  1898,  p.  381. 
CoHN.     Neurolog.  Centralbl.,  1899,  p.  582. 


936  THE    EYE    AXD    NERVOUS    SYSTEM 

ScHUELEB.     Ibid.,  1899,  p.  585. 

Kalischeb.     Virchow's  Archiv,  vol.  civ. 

CoLLiEB.     Brain,  Spring,  1899. 

Lebi.     Neurolog.  Centralbl.,  1901,  p.  141;    Revue  Xeurolog.,  1903,  No.  14. 

Cbonzox.     Ibid.,  1901,  p.  142. 

MoBSE.     Pediatrics,  1901,  January. 

Waltox  and  Paul.    Journal  of  Nervous  and  Mental  Disease,  1900,  June. 

Cestan  and  Le  Soubd.     Gaz.  des  HOpitaux,  1899,  Nov.  22,  p.  1249. 

Buzzard.     British  Med.  Jour.,  1899,  vol.  i.  p.  1077. 

Van  Epps.     Journal  of  Nervous  and  Mental  Disease,  vol.  xxviii.  No.  4. 

TuMPOWSKl.     Medycyna,  1901;    Neurolog.  Centralbl.,  1901,  p.  663. 

Chodzko.     Gazeta  Lekarska,  1901 ;    Neurolog.  Centralbl.,  1901,  p.  663. 

ScHOEXBOBX.     Neurolog.  Centralbl.,  1901,  p.   10. 

HoMBURGEB.     Ibid.,  1901,  p.  698. 

(JoLDFLAM.     Ibid.,  1903,  December  1. 

Barns.    Review  of  Neurology  and  Psychiatry,  1904,  May. 

HoMBEBGER.     Neurolog.  Centralbl.,  1902. 

Bramwell.     Review  of  Neurology  and  Psychiatry,  1903,  June. 

Marinesco.     Revue  Neurologique,  1903,  No.  10. 

RiCHTER.     Miinchener  Med.  Woch.,  1903,  No.  24. 

Modifications  of  the  Babinski  Phenomenon. 
Oppexheim.     Monats.  f.  Psych,  u.  Neurol.,  1902. 
Cassirer.    Ibid.,  p.  37. 
Pfeiffer.     Ibid.,  pp.  270,  585. 
Verger  and  Abadie.     Progr6s  Medicale,  1900,  No.  17;    Neurolog.  Centralbl.,  1900, 

p.  859. 
SciiAEFEB.     Neurolog.   Centralbl.,   1899,  p.   1016. 
HiRSCHBEBG.     Revue  Neurologique,  1903,  No.   15. 
Mendel.     Neurolog.  Centralbl.,   1904,  p.   197. 
Von  Bechtebew.     Neurolog.  Centralbl.,  1904,  p.  609. 
Crocq.     Jour,  de  Neurologic,  1901. 

Unclassified  Reflexes. 
KEBxao.     Berlin  Klin.  Woch.,  1884,  p.  829. 
Fbiis.     Copenhagen  Thesis,   1877    (quoted  by  Netter;     also  Ugeskrift  for  Laeger, 

1892,  pp.  407,  431). 
Netteb.     La  Semaine  Medicale,  1898,  p.  281. 

Herrick.     American  Journal  of  Medical  Sciences,  1899,  vol.  cxviii.  p.  35. 
Sailer.     Ibid..  May,  1902. 
Shields.     Ibid. 

DiEULAFOY.     ('Unique  Med.  de  THStel  de  Dieu,  1898-1899,  p.  347. 
SiNKLEB.     Medical  News,  Dec.  1,  1888. 

Strumpell.     Deutsche  Zeits.  f.  Nervenheilk.,  vol.  xx.  p.  436. 
RoGLET.     Journal  de  Medecine,  1900,  Oct.  10. 

Front -Tap. 
Walton  and  Paul.     .Journal  of  Nervous  and  Mental  Disease,  1903,  p.  341. 

The  Abdominal  Reflex. 
ScilOEXBORX.     Deutsche  Zeits.  f.  Nervenheilk..  vol.  xxi.  p.  273. 
DiNKLER.     Ibid.,  vol.  xxii.  p.  23. 
OsTAXKOW.     Neurolog.  Centralbl.,  1898,  p.   140;     1900,  p.  765    (abstract). 


GAITS  937 

The  Cremaster  Reflex. 
Steiler.     Deutsche  Zeits.  f.  Xervenheilk.,  vol.  xxvi.  p.  285. 
Geigel.     Deutsche  Med.  Woch.,  1892,  p.  166. 
ScHOENBORN.    Deutsche  Zeits.  f.  Xervenheilk.,  vol.  xxi.  p.  273. 
Ganault.     These  de  Paris,  1898;    Neurolog.  Centralbl.,  1899,  p.  367. 
Gibson.    Edinburgh  Medical  Journal,  1901,  p.  459. 

The  Hypogastric  Reflex. 
Von  Bechterew.     Neurolog.  Centralbl.,  1901,  p.  647. 
Van  Gehuchten.     Journal  de  Neurologic,  1900. 
Von  Kohnilow.     Zeits.  f.  Nervenheilk.,  vol.  xxiii.  p.  216    (calls  attention  to  some 

early  references  to  this  reflex). 
Crocq.     Journal  de  Neurologic,  1901. 

The  Femoral   Reflex. 
Remak.    Neurolog.  Centralbl.,  1893,  No.  15,  1900,  p.  7. 

The  Scapulohumeral  Reflex. 
Von  Bechterew.     Neurolog.  Centralbl.,  1900,  pp.  208,   1042. 
Haenel.     Ibid.,  p.  399. 
Steinhausen.     Ibid.,  1901,  p.  507. 
Pickett.     Journal  of  Nervous  and  Mental  Disease,   1901,  No.  5. 

The  Gluteal  Reflex. 
Negro.     Bull,  del  Policlin-gen.  di  Torino,  II   (Neurolog.  Centralbl.,  1898,  p.  79). 

Myoidema. 
Bernstein.    Neurolog.  Centralbl.,  1899,  p.  1068. 

GAITS. 
THE   GAIT   AS   A   SYMPTOM    OF    NERVOUS   DISEASE. 

Walking  may  be  described  as  progression  by  placing  one  foot  on 
the  ground,  supporting  the  body  upon  it,  lifting  the  other  foot  to  a 
slightly  higher  level,  and  swinging  the  leg  forward;  this  leg  in  turn 
becomes  the  support,  and  the  first  leg  repeats  the  movements.  The  sup- 
porting leg  is  kept  rigid  by  the  contraction  of  the  muscles,  particularly 
those  of  the  calf,  the  quadriceps,  and  the  gluteal  and  psoas  muscles, 
although  practically  all  the  other  muscles  of  the  limb  are  brought  into 
play  for  the  purpose  of  limiting  movements  at  the  joints.  During  the 
act  of  walking  there  are  two  chief  movements  in  the  supporting  limb: 
at  the  ankle-joint,  in  which  the  tibia  rolls  forward  on  the  astragalus, 
and  at  the  hip-joint,  in  which  the  thigh-bone  rocks  backward  in  the 
acetabulum  as  the  body  moves  forward.  There  is  also  a  slight  rotary 
movement  in  the  hip  as  the  pelvis  is  swung  around  it  in  the  effort  of 
throwing  forward  the  opposite  leg.  The  knee-joint  usually  remains 
fixed  until  the  movement  forward  becomes  so  great  that  the  heel  of  the 


938  THE    EYE    AND    NERVOUS    SYSTEM 

supporting  leg  is  lifted  from  the  ground,  when  it  begins  to  bend  slightly. 
The  shortening  of  the  swinging  leg  is  accomplished  by  tilting  the  pelvis 
and  slight  flexion  at  the  thigh,  knee,  and  ankle.  The  swing  commences 
with  a  slight  throw  given  with  the  toes  as  the  foot  leaves  the  ground, 
and  terminates  as  the  heel  strikes  the  ground,  when  the  forward  incli- 
nation of  the  supporting  leg  becomes  so  great  that  the  swinging  leg  no 
longer  clears  the  surface. 

METHODS    OF   STUDY. 

The  ordinary  mode  of  walking  may  be  modified  by  structural  deform- 
ities, especially  if  in  the  skeleton,  or  by  functional  defects  either  in 
the  muscles  or  in  the  nervous  mechanism  controlling  them.  These  modi- 
fications have  been  studied  usually  by  simple  observation,  but  a  variety 
of  methods  have  been  employed  for  the  purpose  of  elucidating  the  final 
changes  and  making  permanent  records.  The  simplest  method  is  to 
measure  off  a  definite  path  on  which  the  patient  walks.  The  number 
of  steps  is  counted  and  divided  into  the  distance  travelled.  The  length 
of  each  step  is  thus  readily  determined.  By  observing  the  patient  care- 
fully from  behind  or  in  front,  lateral  deviations  in  the  movements  of 
the  legs  can  be  detected,  and  by  placing  the  eye  close  to  the  ground  it 
can  be  noted  whether  the  feet  are  abnormally  raised  or  not.  This 
method,  however,  is  scarcely  better  than  direct  observation  (Weber). 

It  is  a  distinct  advance  to  employ  some  method  by  which  the  foot- 
prints themselves  can  be  more  or  less  permanently  impressed  upon  the 
surface.  This  is,  of  course,  done  upon  soft  soil  or  sand,  but  the  yielding 
character  of  the  material  produces  certain  alterations  in  the  gait  which 
render  it  slightly  abnormal.  Moreover,  the  preservation  even  of  a  single 
footprint  by  the  method  of  making  plaster  casts  is  tedious  and  difficult. 

Vierordt  was  apparently  the  first  to  employ  a  color  material,  which 
was  exuded  by  a  specially  prepared  shoe.  Gilles  de  la  Tourette  covered 
the  sole  of  the  foot  with  powdered  oxide  of  iron,  and  then  had  the 
j)atient  walk  upon  strips  of  rough  paper,  enough  of  the  powder  being 
caught  in  the  interstices  to  leave  a  record.  Still  more  complicated  is 
the  method  of  Monkenmoller  and  Kaplan,  who  employ  an  alcoholic 
solution  of  chloride  of  iron  in  which  the  stockings  are  soaked.  The 
patient  walks  upon  a  piece  of  paper  which  is  afterwards  sprayed  with 
an  alcoholic  ethereal  solution  of  ammonium  sulphocyanide,  forming 
ferric  sulphocyanide,  and  leaving  a  permanent  dark  red  mark  that  can 
readily  be  photographed.  The  very  thick  wet  stockings  interfere  slightly 
with  the  gait. 

I  have  used  a  simple  modification  which  has  proved  so  satisfactory 


GAITS  939 

that  I  believe  I  can  commend  it  heartily.  It  consists  in  painting  the 
feet  with  ordinary  black  paint, — that  is,  lamp-black  dissolved  in  linseed 
oil.  The  paint  should  be  somewhat  thicker  than  that  ordinarily  supplied 
ready-made.  It  can  best  be  smeared  on  the  feet  with  a  wad  of  cotton 
or  gauze.  Ordinary  wrapping-paper  is  employed.  This  comes  in  rolls 
about  four  feet  wide,  from  which  strips  of  convenient  length  (usually 
from  fifteen  to  twenty  feet)  can  readily  be  cut.  A  single  painting  suf- 
fices for  about  twenty  clear  tracings  from  each  foot, — that  is,  the  patient 
can  walk  a  much  longer  distance  than  the  usual  length  of  the  strip  of 
wrapping-paper.  The  jiaint  dries  quickly  and  makes  an  impression  that 
is  practically  permanent.  I  have  records  over  four  years  old  that  are 
as  clear  as  they  were  originally.  The  strips  can  be  rolled  up  into  very 
small  compass,  can  easily  be  stored  and  classified,  and  photographic 
reproduction  is  very  satisfactory.  Jendrassik  condemns  these  methods 
because,  according  to  him,  it  gives  us  no  more  information  than  simple 
inspection.  In  this  I  think  he  is  mistaken.  Obviously  it  enables  us 
to  substitute  for  more  or  less  erratic  guessing  actual  measurements  re- 
garding the  length  of  the  steps,  the  difference  in  the  length  of  the  steps 
in  the  two  legs,  the  separation  of  the  legs,  and  whether  or  not  successive 
steps  of  the  same  leg  are  in  a  straight  line.  Moreover,  the  print  of  the 
foot  can  be  carefully  studied :  whether  the  whole  of  the  sole  touches 
the  ground,  whether  part  of  it  remains  permanently  above  the  ground, 
and  if  any  dragging  occurs  it  is  very  distinctly  shown.  The  great 
advantage  of  this  method,  however,  is  that  it  furnishes  a  permanent 
record  infinitely  preferable  to  any  description  that  can  be  written.  The 
chief  objection  to  it  is  that  no  record  is  made  of  the  movements  of  the 
feet  while  in  the  air. 

A  very  simple  method  which  may  be  employed  if  no  materials  are 
at  hand  is  to  allow  the  patient  to  walk  on  the  floor  after  the  feet  have 
been  wet.  Usually  from  five  to  ten  steps  with  each  foot  will  be  recorded, 
and  the  water  does  not  evaporate  for  several  minutes.  If  the  patient 
wears  thick  stockings  which  have  been  saturated  with  water  a  greater 
number  of  steps  can  be  obtained.  This  will  enable  the  observer  to  take 
satisfactory  measurements  of  the  foot-prints. 

The  application  of  instantaneous  photography  to  moving  objects 
provided  a  new  method  for  the  study  of  the  gait.  To  Muybridge, 
working  at  first  independently  and  subsequently  under  the  auspices  of 
the  University  of  Pennsylvania,  the  credit  for  the  first  complete  study 
of  the  human  gait  is  due.  Xot  only  can  the  individual  phases  of  motion 
be  studied  by  this  method,  but  also  by  various  methods  the  pictures  can 
be  thrown  upon  a  screen  in  such  rapid  succession  that  the  movement 


940  THE    EYE    AND    NERVOUS    SYSTEM 

appears  to  be  reproduced.  For  scientific  purposes  it  suffers  under  the 
disadvantage  that  pictures  taken  with  the  subject  advancing  directly 
towards  or  retreating  from  the  camera  are  comparatively  unsatisfactory. 
This  metliod,  with  various  changes,  has  also  been  employed  by  Marie, 
Dercum,  Braune,  Fischer,  and  Londe;  and  Marinesco  has  used  the 
more  modern  cinemetograph. 

Jendrassik  has  devised  a  modification  which  he  considers  superior 
to  all  other  methods.  It  consists  in  having  the  patient  walk  in  a  dark 
room  in  front  of  a  photographic  plate  and  lens,  and  then  by  means  of 
electricity  producing  momentary  flashes  of  light  at  regular  intervals. 
As  a  result  the  picture  of  the  individual  at  various  stages  of  the  gait 
appears  upon  the  plate.  The  objections  to  this  method  are  numerous. 
In  the  first  place,  the  subject  walks  in  a  room  alternately  dark  and 
bright,  and  as  a  result  the  gait  must  be  to  a  certain  extent  disturbed. 
In  the  second  place,  and  to  my  mind  this  is  a  more  serious  objection, 
the  pictures  upon  the  plate  are  superimposed ;  it  is  very  difficult,  there- 
fore, to  make  a  careful  analysis.  In  the  third  place,  it  is  practically 
impossible  to  take  pictures  with  the  patient  walking  toward  or  away 
from  the  camera,  Xevertheless,  this  method,  if  carefully  carried  out, 
yields  excellent  results,  and  some  of  the  pictures  published  by  Jen- 
drassik leave  little  to  be  desired. 

Other  methods  that  have  been  employed  for  attempting  to  record 
various  movements  graphically,  either  upon  a  rotating  drum  or  by  means 
of  an  apparatus  squirting  colored  fluids  upon  a  recording  surface,  are 
so  cumbersome  and  unsatisfactory  that  they  have  been  entirely  dis- 
carded. 

The  classification  of  the  pathological  varieties  of  gait  is  compara- 
tively simple  theoretically.  Practical!}^,  however,  it  is  difficult,  partly 
because  of  the  great  variation  occurring  in  many  of  the  forms,  partly 
because  of  the  not  infrequent  association  of  two  or  more  disturbing 
factors.  Broadly  we  may  recognize  two  groups:  those  due  to  actual 
deformity  of  tlie  skeleton  and  those  due  to  disturbances  in  the  neuro- 
muscular mechanism.  The  former,  with  the  exception  of  a  few  forms 
of  trophic  disturbance  of  the  bones,  belong  to  the  domain  of  surgery, 
and  will  not  be  considered  in  this  article.  The  latter  may  be  divided 
into  five  groups.  First,  flaccid  paralytic  lesions  of  the  neuromuscular 
mechanism ;  second,  spastic  paralytic  lesions  of  the  neuromuscular 
mechanism,  each  of  these  may  be  subdivided  into  the  unilateral  and 
bilateral  forms,  and  they  are  often  combined  ;  third,  the  group  character- 
ized by  loss  of  muscular  co-ordination, — that  is,  the  ataxic  gaits;  and, 
fourth,  a  group  due  to  involuntary  muscular  movements, — the  chorei- 


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I.  II.      .  III.  IV. 

I.  Case  of  Hemiplegia,  Two  Years  Dukation.— The  impression  of  the  right  foot  is  weak ;  there 
is  some  contraction  of  the  muscles,  causing  accentuation  of  the  arch.  The  alignment  is  good  and 
there  is  very  little  dragging  of  the  foot. 

II.  Case  of  Hemiplegia  of  Long  Duration.— The  right  foot  is  turned  out  and  drags  distinctly 
( unfortunately  the  illustration  has  been  retouched  and  does  not  show  this).  The  steps  are  uneven  and 
there  is  occasional  staggering. 

III.  Case  of  Cekebp.al  Spastic  Paraparesis.— The  steps  are  short  and  uneven  and  there  is  a 
tendency  to  crossing  the  legs. 

IV.  Case  OF  Primary  Neural  .\trophy:  that  is,  Paralytic  Paraparesis.— The  toes  are  turned 
out ;  the  alignment  is  poor.  The  peculiar  character  of  the  footprint  is  due  to  the  atrophy  of  the 
muscles  of  the  sole  of  the  foot. 


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V.  VI.  VII.  VIII. 

No.  .5.  The  Gait  of  a  Normal  Adult  Male.— The  alignment  is  good;  the  steps  arc  equal;  the  impres- 
sions are  clear  and  distinct.    Tlie  left  foot  is  turned  out  slightly  more  than  normal. 

No.  <;.  A  Case  of  Tabe.s  Dorsalls.— The  alignment  is  poor;  the  distance  between  the  -steps  is  unequal: 
at  one  point  the  leg  is  thrown  .slightly  to  the  side.  The  impressions  vary  in  intensity,  particularly  with  refer- 
ence to  the  arch  of  the  left  foot. 

No.  7.  A  Case  Sipposeo  to  be  Cerebellar  Disease.— The  type  of  gait  is  the  late  stage  of  ataxia  with 
paresis.  The  steys  are  very  short;  the  legs  are  widely  separated  ;  the  alignment  is  poor.  The  stejis  are  very 
unequal  in  length,  and  the  impressions  vary  in  intensity.  It  will  be  noted  that  the  number  of  toes  striking 
the  ground  varies  in  different  impressions,  showing  a  slightly  spa.stic  condition  of  the  extensor  muscles. 

No.  H.  The  (Jait  of  a  Normal  adult  Female.  Tlie  .steps  are  shorter,  and  the  separation  of  the  legs 
slightly  greater  than  in  the  tracing  taken  from  the  adult  male.    The  gait  presents  no  abnormal  features. 


GAITS  941 

form  gaits.  This  comprises  a  number  of  very  diverse  conditions. 
Finally,  there  are  several  forms,  different  in  themselves  and  not  be- 
longing to  any  of  the  regular  types.  Before  discussing  these  different 
classes  I  wish  to  mention  the  general  features  that  should  be  most  care- 
fully studied,  because  it  is  by  means  of  the  amount  and  degree  of  their 
deviation  from  the  normal  that  the  character  of  the  gait  is  determined. 
First,  the  length  of  the  steps ;  whether  the  step  made  by  one  foot 
is  longer  or  shorter  than  the  step  made  by  the  other, — that  is  to  say, 
whether  the  distance  from  the  toe  of  the  left  foot  to  the  heel  of  the  right 
foot  is  more  or  less  than  the  distance  from  the  toe  of  the  right  foot  to 
the  heel  of  the  left  foot.  Second,  the  degree  of  separation  of  the  two 
feet;  under  normal  circumstances,  a  straight  line  drawn  between  the 
footprints  should  touch  or  slightly  cut  the  inner  side  of  both  heels. 
Third,  the  degree  to  which  the  toes  are  turned  out;  this  is  modified 
more  or  less  by  habit,  but  normally  a  straight  line  drawn  through  the 
centre  of  the  heel  passes  through  the  ball  of  the  great  toe ;  however, 
moderate  deviations  from  this  rule  cannot  be  considered  pathological. 
Fourth,  the  part  of  the  foot  which  first  touches  the  ground,  whether  the 
heel  or  toe ;  this  is  also  modified  by  habit,  but  it  is  excessively  rare  for 
any  person  normally  to  strike  the  ground  with  the  ball  of  the  foot  first, 
although  Ellis  believes  that  certain  savage  races  normally  walk  in  this 
manner.  Fifth,  the  way  in  which  the  foot  leaves  the  ground ;  whether 
it  is  thrown  clear  by  the  spring  of  the  toes  or  whether  they  remain  in 
contact  with  the  surface  for  a  longer  or  shorter  time  while  the  foot  is 
dragged  forward.  Sixth,  the  way  in  which  the  sole  of  the  foot  rests 
upon  the  ground,  in  some  cases  the  arch  is  distinctly  lost,  or  the  patient 
may  walk  upon  the  toes  or  the  side  of  the  foot.  Seventh,  the  height  to 
which  the  foot  is  lifted  from  the  ground  during  the  step.  Eighth,  the 
amount  of  lateral  swing  in  the  leg.  Ninth,  the  manner  in  which  the 
foot  strikes  the  ground,  whether  forcibly  or  lightly.  Tenth,  the  align- 
ment of  the  steps.  In  addition,  such  gross  changes  as  cross-legged  pro- 
gression or  walking  with  crutches  should  be  noted,  and  such  finer 
changes  as  the  behavior  of  the  toes  during  the  step.  Under  certain  cir- 
cumstances, spastic  dorsal  flexion  may  prevent  one  or  all  of  the  toes 
from  touching  the  ground  at  any  point. 

THE    PARALYTIC    GAITS. 

The  flaccid  paralytic  gaits  are  characterized  by  the  absence  of  the 

reflexes  in  addition  to  the  weakness  of  the  limbs.     As  a  general  rule, 

these  gaits  occur  in  various  forms  of  peripheral  palsies  and  myopathies. 

If  bilateral,  such,  for  example,  as  occurs  in  peripheral  polyneuritis,  the 


942  THE    EYE    AXD    XERVOUS    SYSTEM 

gait  presents  the  following  peculiarities:  the  steps  are  short,  the  feet 
are  planted  rather  widely  apart,  and  there  may  or  may  not  be  irregu- 
larity in  alignment.  The  feet  are  lifted  from  the  ground  without 
swinging,  the  toe  leaving  last.  The  knee  is  lifted  somewhat  higher  than 
normal  from  the  ground,  in  order  to  permit  the  toe,  which,  as  a  result 
of  toe-drop,  is  not  raised,  to  clear  the  floor.  The  toe  strikes  the  ground 
first  and  then  the  rest  of  the  foot  is  planted  upon  the  surface.  During 
the  step  the  foot  remains  flat  upon  the  ground  and  does  not  aid  in  lift- 
ing the  body.  Walking  is  therefore  accomplished  chiefly  by  the  muscles 
that  move  the  thigh  upon  the  pelvis.  In  the  severer  forms  of  this  type 
of  paralysis  the  weakness  is  so  great  that  it  is  impossible  for  the  patient 
to  lift  the  feet  from  the  ground,  and  they  are  dragged  along,  usually 
turning  slightly  outward.  In  these  forms  additional  support  is  nearly 
always  required,  eitlier  by  canes  or  crutches.  As  a  rule,  walking  is 
attempted  only  in  the  milder  forms. 

In  anterior  poliomyelitis,  either  unilateral  or  bilateral,  the  type  of  gait 
depends  largely  upon  the  particular  muscles  affected.  If  the  involve- 
ment is  extensive,  walking  without  crutches  is  impossible.  The  legs 
are  drawn  forward  and  serve  as  a  sort  of  third  crutch  to  aid  in  pro- 
gression. Very  often  it  is  necessary  to  stiffen  the  joints  by  means  of 
braces.  If  the  peroneal  muscles  are  involved  there  will  be  foot^drop, 
the  leg  will  be  lifted  higher  from  the  ground,  and  will  be  planted  nearly 
flat,  the  toe  touching  first,  but  if  the  calf  muscles  are  preserved  there 
will  be  a  distinct  spring  at  the  end  of  the  step.  In  these  cases  the  foot 
is  usually  turned  out.  If  contractures  have  ensued,  giving  rise  to 
club-foot,  the  condition  becomes  surgical  rather  than  neurological,  and 
the  type  of  gait  depends  upon  the  nature  of  the  deformity. 

In  the  ordinary  forms  of  muscular  dystrophy  the  ability  to  walk  is 
preserved  for  a  long  time.  The  gait  is  quite  characteristic.  The  steps 
are  short,  usually  made  with  effort ;  the  legs  are  well  separated  so  that 
the  gait  has  a  waddling  character ;  the  feet  are  not  lifted  very  well  from 
the  ground,  and  the  knees  are  kept  nearly  completely  extended.  If  at 
any  time  they  become  flexed  the  patient  is  likely  to  fall.  The  foot, 
however,  usually  clears  the  ground,  and,  unless  the  weakness  is  extreme, 
the  alignment  is  good.  As  the  muscles  of  the  trunk  are  also  frequently 
involved  there  may  be  considerable  rocking  of  the  pelvis  which  accentu- 
ates the  waddling. 

In  pseudomuscular  hypertrophy  the  gait  is  essentially  the  same,  the 
only  difference  being  in  the  appearance  of  the  limbs.  In  both  forms 
the  patient  shows  a  peculiar  method  of  arising  from  the  floor  which  has 
been  frequently  described,  but  is  so  characteristic  that  it  serves  as  an 


GAITS  943 

important  symptom.  He  first  assumes  a  sitting  posture,  then  drawing 
tlie  body  fonvard  gets  upon  the  hands  and  knees,  then  pushing  the  body 
backward  he  brings  the  hands  close  to  the  feet,  grasps  the  legs,  and 
gradually  climbs  up  his  own  legs,  supporting  the  trunk  partly  upon  the 
arms  and  partly  upon  the  legs.  In  the  peroneal  form  of  muscular  dys- 
trophy and  in  some  forms  of  polyneuritis  the  predominant  characteris- 
tic of  the  gait  is  the  extreme  ataxia.  This  will  be  described  in  connec- 
tion with  ataxic  gaits. 

The  Spastic  Paralytic  Gait. — Lateral  Sclerosis. — Bilateral  paretic 
disturbance  of  gait  with  spasticity  of  the  muscles  occurs  as  a  result  of 
cord  or  brain  lesion.  Pressure  upon  the  cord  above  the  lumbar  enlarge- 
ment gives  rise  to  a  peculiar  so-called  spastic  gait  which  was  formerly 
believed  to  be  due  to  sclerosis  of  the  lateral  columns.  As  a  matter  of 
fact,  a  descending  sclerosis  of  these  columns  does  occur,  but  very  rarely 
as  an  idiopathic  lesion.  The  steps  are  equal  in  length  and  may  be  short 
or  long,  according  to  the  degree  of  the  paralysis :  the  less  the  paralysis 
the  longer  the  step.  The  feet  are  usually  kept  moderately  close  togther, 
often,  indeed,  there  is  a  more  or  less  pronounced  tendency  for  the  legs 
to  cross.  During  the  swing  the  leg  rotates  slightly  outward  to  pass  the 
other  leg ;  the  leg  is  kept  stiff  and  the  foot  extended.  As  a  result,  for 
a  greater  or  less  portion  of  the  stride  the  toe  remains  in  contact  with  the 
ground.  The  whole  gait  is  of  a  peculiar  stiff  and  awkward  character. 
Although  the  toe  touches  the  ground  first,  there  is  no  spring  to  it,  and 
the  patient  proceeds  very  much  as  if  walking  on  stilts.  It  is  charac- 
teristic of  this  condition  that  the  toes  of  the  shoes  are  very  rapidly 
worn  out,  and  that  frequently  the  inside  of  the  legs  of  the  trousers, 
particularly  at  the  knees,  are  also  soon  worn  through.  lu  forms  in 
which  the  spasticity  is  the  most  pronounced  condition  the  legs  may 
even  be  crossed,  giving  rise  to  cross-legged  progression.  A  modification 
of  this  form  occurs  in  Little'' s  disease.  In  this,  however,  there  is  marked 
foot-drop ;  the  patient  therefore  lifts  the  leg  high  from  the  ground  in 
order  to  clear  the  toe :   the  so-called  "  chicken-gait." 

The  unilateral  spastic  paretic  gait  is,  in  the  vast  majority  of  con- 
ditions, the  result  of  cerebral  lesion.  The  gaits  of  hemiplegia  are,  as 
a  rule,  quite  characteristic.  The  degree  of  modification,  of  course,  de- 
pends largely  upon  the  extent  of  the  paralysis.  In  the  mildest  forms 
there  is  merely  a  slight  limp.  Careful  observation  and  foot-print  rec- 
ords, however,  will  show  that  even  in  this  type  the  step  on  the  hemiplegic 
side  is  somewhat  shorter;  that  the  toe  drags  for  an  instant  after  the 
stej)  has  commenced,  and  that,  as  a  result  of  the  stiffness  of  the  leg, 
it  is  swung  slightly  to  the  side,  the  foot  being  lifted  less  than  the  leg 


944  THE    EYE    AXD    XERVOUS    SYSTEM 

on  the  sound  side.  The  steps  arc  usually  in  a  straight  line;  the  foot 
is  turned  out,  and  not  infrequently  the  feet  are  separated  slightly 
more  than  in  normal  walking.  The  more  severe  forms  are  merely  exag- 
gerations of  these  features,  but  of  course  present  a  very  different  appear- 
ance. The  step  is  short,  the  feet  are  well  separated,  the  toe  clings  to  the 
ground  for  a  considerable  distance,  sometimes  throughout  the  entire 
period  of  the  stej),  the  leg  is  swung  well  outward,  and  usually  the  foot- 
steps on  the  affected  side  are  no  longer  in  a  straight  line,  showing  the 
existence  of  a  greater  or  less  amount  of  ataxia.  As  the  muscles  on  the 
paralyzed  side  are  not  capable  of  tilting  the  pelvis,  this  is  accomplished 
by  throwing  the  body  over  to  the  opposite  side,  so  that  with  each  step 
it  oscillates  distinctly.  The  step  on  the  sound  side  is  usually  character- 
ized by  an  exaggerated  spring  from  the  toe,  and  a  marked  hurrying  of 
the  swing  so  that  as  little  time  as  possible  is  spent  resting  on  the  lame 
leg.  If  the  condition  becomes  more  severe  the  patient  is  obliged  to 
support  himself  with  a  cane  or  crutch  usually  carried  on  the  sound  side, 
although  if  the  arm  has  recovered  sufficiently  it  is  not  uncommon  for 
the  patient  to  carry  a  crutch  on  the  sound  side  and  a  cane  in  the  other 
hand. 

GAITS    DUE    TO    DISTURBANCE    OF   CO-ORDINATION. 

The  ataxic  gait  is  characterized  by  the  following  features :  inequal- 
ity in  the  length  of  the  steps ;  defective  alignment  of  successive  steps ; 
irregular  high  lifting  of  the  feet  from  the  ground,  and  usually  a  some- 
what stamping  type  of  progression, — that  is,  the  feet  strike  the  ground 
rather  vigorously.  As  a  result  of  the  uncertainty  and  irregularity  of 
the  footsteps,  the  centre  of  equilibrium  is  continually  disturbed,  and 
the  body  is  jerked  forward  and  backward  or  to  one  side  or  the  other  in 
order  to  preserve  the  balance. 

In  tabes  dorsalis  this  type  of  gait  occurs.  All  grades  may  be  ob- 
served, from  a  slight  impairment,  which  may  be  imperceptible  unless 
special  methods  are  used,  to  such  a  profound  paresis  and  incoordination 
that  the  j)atient  can  only  progress  by  the  aid  of  two  canes  or  crutches. 

The  tracing  of  a  tabetic  gait  is  usually  quite  characteristic.  The 
spaces  between  the  successive  footprints  show  a  considerable  amount  of 
variation.  Occasionally  a  footprint  is  seen  far  to  one  side,  due  to  the 
■effort  of  the  patient  to  recover  his  balance.  Even  where  the  walking 
is  more  regular,  it  can  be  seen  that  sometimes  the  feet  are  slightly 
crossed,  sometimes  more  widely  separated  than  normally.  The  char- 
acter of  the  impression  varies  considerably.  Sometimes  the  sole  of  the 
foot  is  pressed  firmly  against  the  ground ;    sometimes  the  patient  walks 


GAITS  945 

upon  the  inner  and  sometimes  upon  the  outer  side  of  the  foot.  In 
some  impressions  the  arch  is  distinct ;  in  others  it  is  faulty,  apparently 
the  result  of  irregular  contractions  of  the  muscles  controlling  it.  The 
changes  are  always  more  pronounced  the  further  the  patient  has  walked. 
In  the  beginning  the  footsteps  maintain  a  fairl}-  good  alignment,  pos- 
sibly due  to  the  greater  concentration  of  the  mind  in  the  beginning  of 
the  effort.  The  toes  are  sometimes  turned  out;  sometimes  the  foot  is 
directed  forward,  showing  irregularities  in  the  rotation  of  the  leg  at 
the  hip.  Even  slight  variations  in  the  intensity  of  the  impression  can 
be  made  out  in  most  of  the  tracings.  One  of  the  most  characteristic 
features  of  the  ataxic  gait  is  that,  in  spite  of  the  obvious  impairment 
of  equilibrium,  only  occasionally  is  an  effort  made  to  compensate  it 
by  separating  the  legs  and  thus  broadening  the  base.  Moreover,  a 
study  of  the  tracings  shows  that,  in  spite  of  the  apparently  long  stride 
due  to  the  elevation  of  the  foot  from  the  ground,  and  the  forcible  way 
in  which  it  is  placed  do^vnward,  the  average  distance  between  the  steps 
is  slightly  less  than  in  normal  persons  of  the  same  size. 

The  ataxic  usually  appears  to  walk  hastily  and  heedlessly,  but  it 
will  be  observed  that  he  keeps  his  eyes  fixed  carefully  upon  the  ground, 
and,  as  far  as  he  may,  chooses  his  path.  There  is  also  a  certain  element 
of  tenseness  and  rigidity  in  the  whole  poise  that  is  merely  an  indication 
of  the  effort  made.  In  nearly  any  form  of  ataxia  a  footprint  tracing 
will  exhibit  the  characteristic  features,  even  although  they  are  not  per- 
ceptible to  simple  inspection.  But  in  these  cases  the  alteration  may  be 
made  more  obvious  by  employing  certain  modifications  in  the  gait.  The 
simplest  of  these  is  to  have  the  patient  walk  with  the  eyes  closed.  Ordi- 
narily, as  soon  as  the  aid  of  vision  is  removed,  the  ataxia  becomes  much 
more  pronounced.  Walking  backwards  is  accomplished  with  difficulty, 
and  often  results  in  falling.  Walking  upon  an  uneven  surface  is  done 
very  badly.  For  this  reason  such  patients  are  very  apt  to  fall  over  slight 
obstacles,  particularly  if  entering  a  dimly  lighted,  unfamiliar  room. 
They  also  walk  badly  in  the  twilight  and  at  night.  Indeed,  it  is  in  a 
measure  a  characteristic  symptom  of  locomotor  ataxia  that  the  patients 
stagger  in  the  early  evening.  Certain  modifications  occur  in  the  gait 
as  the  result  of  the  progress  of  the  disease.  Tabetic  arthropathies  pro- 
duce such  a  relaxation  of  the  ligaments  of  the  joint  that  it  may  bend 
freely  in  all  directions.  Under  these  circumstances,  with  each  step  the 
leg  shortens  as  a  result  of  bowing,  either  backward  or  laterally.  The 
body  therefore  tilts  to  that  side,  and  the  other  leg  can  only  be  swung 
or  dragged  a  short  distance  forward  until  it  is  necessary  to  change  the 
step.    Walking  becomes  difficult,  but  the  deformity  may  become  extreme 

60 


946  THE    EYE    AND    NERVOUS    SYSTEM 

before  it  becomes  impossible,  even  although  both  knees  may  be  affected. 
Arthropathies  may  occur  in  the  hip,  but  do  not  give  rise  to  such  char- 
acteristic alteration  in  the  gait. 

In  the  advanced  fonns  of  tabes  with  marked  paresis  unaided  loco- 
motion is  impossible.  The  patient  usually  seeks  the  aid  of  one  or,  often, 
two  canes ;  the  body  is  tilted  forward,  a  good  deal  of  the  weight  resting 
upon  the  arms,  the  steps  are  short,  even  dragging,  and  quite  uncertain ; 
the  patient  keeps  his  eyes  fixed  upon  the  ground  as  well  for  the  purpose 
of  placing  the  canes  as  of  planting  the  feet.  Even  the  movements  of 
the  arms  are  uncertain,  and  the  canes  are  not  carried  forward  in  a 
straight  line,  but  waver  from  side  to  side  and  are  thrust  against  the 
ground  in  a  manner  that  resembles  the  stamping  of  the  feet.  In  cases 
of  blindness  as  a  result  of  optic  atrophy  in  the  course  of  the  disease  a 
curious  modification  takes  place.  The  ataxia  disappears  almost  entirely, 
and  the  patient  may  for  a  time  be  able  to  walk  quite  as  well  as  any 
other  blind  person.  As  a  rule,  however,  increasing  paresis  finally  causes 
disability.  As  a  result  of  careful  education  of  the  tabetic  by  means  of 
systematic  co-ordinated  movements,  gradually  increasing  in  complexity 
(Fraenkel's  method),  so  much  improvement  may  ensue  that  even  a 
severe  ataxia  may  be  converted  into  a  mild  form.  In  the  tabetic  form 
of  general  paresis  the  gait  is  modified  in  the  same  manner  as  in  tabes 
dorsalis. 

The  gait  in  Friedreich's  ataxia  is  intermediate  between  the  spinal  and 
the  pure  cerebellar  forms,  although  it  resembles  the  spinal  more  nearly. 
As  the  disease  progresses  the  inco-ordination  becomes  more  and  more 
severe  until  finally  the  patient  is  unable  to  walk  at  all.  Not  infrequently 
a  deformity  of  the  feet — a  form  of  talipes  equinus — adds  a  limp  to  the 
gait  of  inco-ordination. 

Cerebellar  ataxia  differs  from  spinal  ataxia  in  several  particulars. 
The  co-ordination  is  usually  more  disturbed,  therefore  the  steps  are 
exceedingly  irregular  and  lack  alignment.  Co-ordination  is  disturbed 
not  only  in  the  legs,  but  also  in  the  trunk,  and,  as  a  result,  there  is 
considerably  more  swaying  of  the  body,  partly  due  to  the  inco-ordina- 
tion, partly  to  the  violent  efforts  of  the  patient  to  maintain  equilibrium. 
The  gait  is  not  made  worse  by  closing  the  eyes, — that  is  to  say,  visual 
impressions  aid  the  patient  little  if  any.  There  may  or  may  not  be  a 
marked  sense  of  vertigo.  If  the  cerebellar  type  is  pronounced  the  patient 
frequently  falls,  and,  of  course,  in  the  severer  forms  walking  unaided 
is  impossible.  It  is  noteworthy  that  in  the  severe  forms  crutches  or 
canes  are  not  of  as  great  assistance  as  they  are  in  spinal  ataxia,  and 
the  patient  must  be  supported  by  one  or  even  two  attendants. 


PLATE  F. 


IX.  X.  XI. 

Xo.  9.  A  SrASTic  Paretic  Gait  from  a  Case  of  Syringomyema.— The  paresis  is  in  excess;  the  arch  of 
the  right  foot  is  wholly  lost,  and  that  of  the  left  foot  is  imperfect.  The  alignment  is  distinctly  poor  ;  the  length 
of  the  steps  is  unequal,  and  the  toes  are  turned  slightly  out.  It  will  be  noted  that  the  Intensity  of  the  impres- 
sions is  unequal. 

Xo.  10.  A  Case  of  Left  Hemiplegia  of  Six  Months  Standing. — The  arch  of  the  foot  is  wholly  lost  on 
the  right  side,  and  is  imperfect  on  the  leftside.  These  changes  possibly  antedate  the  lesion.  The  foot  on  the 
paralysed  side  is  turned  outward  at  an  angle  of  30  degrees.  The  feet  are  well  separated;  the  alignment  is 
poor ;"  the  steps  are  very  short.    There  is  no  distinct  dragging  of  the  toes. 

Xo.  11.  A  Bilateral  Spastic  Gait.— The  steps  are  short  •  the  alignment  is  poor.  The  separation  of  the 
feet  is  unequal,  but  occasionally  there  is  a  tendency  to  crossing  the  legs.  There  is  distinct  dragging  of  the 
toes  on  both  sides,  the  dragging  of  the  foot  being  outward  so  that  the  knee  can  clear  the  knee  upon  the 
opposite  side. 


GAITS  947 

Cerebral  ataxia  is  not  very  common.  Tt  occurs  occasionally  as  a  result 
of  focal  lesion,  and  is  usually  on  one  side  of  the  body.  Under  these 
circumstances,  if  slight,  it  may  be  detected  in  the  footprint  tracing  by 
noting  that  on  one  side  the  steps  are  slightly  unequal  in  length  and  the 
alignment  is  poor.  Bilateral  ataxia  may  occur  as  a  result  of  pressure 
from  brain  tumors  or  other  conditions.  It  is  usually  of  the  cerebellar 
type. 

In  multiple  neuritis  marked  ataxia  sometimes  occurs  constituting  the 
pseudotabetic  form.  This  is  especially  characteristic  in  the  hypertro- 
phic form. 

Choreiform  Gaits. — The  gait  of  athetosis  is  characterized  by  irregu- 
lar movements  not  only  of  the  legs,  but  also  of  the  whole  body.  The 
muscles  appear  to  relax  and  contract  slowly,  entirely  independent  of 
voluntary  control,  but  at  the  same  time  a  certain  amount  of  control  is 
possible,  so  that  these  irregular  arhythmical  movements  can  be  partly 
inhibited  and  directed.  By  the  exercise  of  great  effort  this  inhibition 
and  direction  nearly  always  suffices  to  maintain  the  equilibrium.  The 
patient  walks  with  most  irregular  movements.  The  steps  are  unequal, 
the  feet  are  planted  irregularly,  the  body  bends  from  side  to  side  or 
forward  and  backward,  the  head  is  continually  moving,  and  the  patient 
appears  as  if  falling  backward  or  sideways  and  to  recover  his  balance 
almost  by  a  miracle.  Actual  falling  rarely  occurs.  This  condition  is 
not  incompatible  w^ith  a  fair  degree  of  intelligence. 

In  ordinary  chorea  the  gait  is  not  typical.  There  is  some  jerkiness 
in  the  movements  and  occasionally  paresis  of  the  legs  or  of  one  side  of 
the  body,  causing  disturbances  that  resemble  slightly  those  of  hemi- 
plegia. 

THE    GAITS    OF    FUNCTIONAL    NERVOUS    DISEASE. 

In  hysteria  the  greatest  variety  of  disturbance  of  gait  may  occur. 
All  the  organic  forms  may  be  simulated  more  or  less  perfectly,  and,  in 
addition,  there  are  certain  types  that  are  peculiar  to  hysteria.  The 
commonest  is  the  so-called  histrionic  gait.  This  can  best  be  described 
as  a  series  of  attitudes  during  progression,  with  steps  of  an  exaggerated, 
but  not  particularly  abnormal,  character.  The  body  is  usually  held  at 
various  angles,  the  legs  are  draw^n  forward  with  a  sweeping  motion,  the 
feet  are  lifted  high  from  the  ground,  and  there  is  considerable  oscilla- 
tion of  the  trunk.  In  these  cases  there  is  not  really  a  true  ataxia, 
although  the  footsteps  may  be  somewhat  uneven.  In  hysterical  mani- 
festations the  symptoms  are  usually  more  pronounced  when  the  patient 
knows  he  is  under  observation,  but  this  is  not  invariably  the  case. 


948  THE    EYE    AND    NERVOUS    SYSTEM 

Astasia-ahasia  is  a  somewhat  more  rare  form  which  has  been  regarded 
as  a  separate  disease.  This  is  really  the  fear  of  falling.  Only  by  the 
strongest  urging  can  the  patient  be  persuaded  to  attempt  to  walk  at  all. 
When  he  finally  stands  upright  he  clings  to  any  object  that  is  near  him. 
If  at  last  the  patient  is  persuaded  to  walk,  the  steps  are  exceedingly 
short  (not  more  than  a  few  inches),  and  the  patient  appears  to  be  in 
the  utmost  terror.  If  surrounding  objects  are  removed  he  usually  stands 
reaching  out  for  support  in  an  appealing  fashion.  He  practically  never 
falls,  nor  does  he  seem,  as  in  the  case  of  athetosis,  to  be  continually 
upon  the  point  of  falling.  In  the  majority  of  these  cases  there  is  usually 
a  general  anaesthesia  which  may  account,  in  part,  for  the  peculiarity  of 
the  gait. 

Although  scarcely  a  disturbance  of  gait,  certain  other  manifestations 
of  hysteria  have  to  do  with  the  limbs.  Particularly  is  this  true  of  the 
dancing  forms  of  pandemic  hysteria,  the  jumper's  disease,  which  is 
practically  the  same  thing,  and  various  other  phenomena.  The  hysterical 
forms  of  ataxia,  hemiplegia,  spastic  paralysis,  etc.,  can  be  distinguished 
from  the  organic  diseases,  as  a  rule,  without  great  difficulty.  The  move- 
ments are  more  irregular,  exaggerated,  and  their  character,  in  addition 
to  the  hysterical  stigmata,  such  as  disturbance  of  the  color  fields,  anaes- 
thesia, tender  points,  etc.,  is  so  characteristic  that  a  diagnosis  can  be 
made  at  a  glance. 

In  Thomsen's  disease,  or  myotonia  congenita,  there  is  usually  difficulty 
in  starting  to  walk.  The  patient,  if  sitting  down,  will  have  to  wait 
an  appreciable  instant  before  arising;  if  standing,  there  will  also  be 
an  interval  before  the  muscles  relax  sufficiently  to  allow  the  limbs  to 
move.  Ordinarily,  in  the  milder  forms,  there  is  no  further  difficulty  in 
progression  after  this  preliminary  stage  is  passed.  The  muscular  spasm 
may,  however,  come  on  while  th§  patient  is  standing,  or  even  while 
walking,  and  then,  owing  to  the  inability  of  the  muscles  to  balance  the 
body,  the  patient  frequently  falls  heavily  to  the  ground. 

The  gait  of  paralysis  agitans  is  quite  typical.  It  is  described  as  the 
gait  of  festination,  and  it  is  usually  stated  that  patients  have  difficulty 
in  starting,  then  the  steps  increase  in  frequency  and  decrease  in  length 
until  the  patient  is  running  along.  In  this  condition  he  is  unable  to 
stop  until  he  guides  himself  against  some  obstruction  which  he  usually 
strikes,  apparently  with  considerable  force.  In  not  all  the  cases  of 
paralysis  agitans,  however,  is  the  festinating  gait  typical.  Yery  fre- 
quently patients  have  difficulty  in  starting  to  walk,  but  can  then  walk 
a  considerable  distance,  the  steps  being  made  slowly,  the  distance  be- 
tween the  feet  increasing,  and  the  length  of  the  steps  decreasing.     Even 


GAITS  949 

at  this  slow  rate  of  progression  voluntary  stoppage  is  frequently  im- 
possible. In  other  cases,  instead  of  becoming  shorter  as  walking  con- 
tinues, the  steps  become  distinctly  longer,  so  that  the  patient  is  soon 
running  with  long  strides.  In  taking  footprint  tracings  of  these  cases 
I  found  one  in  which  there  was  a  marked  dorsal  extension  of  the  toes 
when  the  sole  of  the  foot  was  on  the  ground,  so  that  the  balls  of  the  toes 
never  came  into  contact  with  the  ground,  the  patient  touching  with  the 
heels  and  springing  with  the  ball  of  the  foot.  As  a  matter  of  fact,  the 
spring  was  insignificant,  the  foot  being  lifted  and  held  almost  horizontal 
to  the  surface.  These  patients  also  show  retro-  and  propulsion, — that 
is  to  say,  when  standing  or  sitting  they  have  a  tendency  from  time  to 
time  to  move  or  fall  backward  or  forward.  Patients  with  paralysis 
agitans,  however,  rarely  fall,  and,  considering  the  difficulty  in  locomo- 
tion, it  is  remarkable  with  what  readiness  they  undertake  to  walk  either 
voluntarily  or  at  the  request  of  the  observing  physician.  The  nature 
of  this  gait  has  been  the  subject  of  some  speculation,  and  it  is  supposed 
that  the  muscular  rigidity  characteristic  of  this  disease  does  not  enable 
the  patient  to  recover  the  equilibrium  after  it  has  been  slightly  advanced 
in  the  course  of  movement.  The  station  in  these  cases  is  quite  typical. 
The  patient  bends  slightly  forward,  the  knees  are  slightly  bent,  the  head 
slightly  protruded,  the  neck  is  rigid,  giving  rise  to  a  curious  attitude, 
the  elbows  are  thrown  slightly  back,  the  forearms  are  bent,  and  the  hands 
slightly  advanced.  The  whole  attitude  expresses  an  extreme  degree  of 
rigidity. 

The  gait  in  sciatica  is,  as  a  rule,  characteristic.  The  patient  walks 
with  the  leg  flexed  slightly  upon  the  thigh,  giving  rise  to  shortening  and 
a  distinct  limp.  The  foot  is  usually  kept  nearly  horizontal  to  the 
ground,  and  there  is  a  little  spring  from  the  toe  at  the  end  of  the  step. 
The  swing  of  the  leg  is  shortened  and  the  patient,  in  order  to  put  as 
little  weight  as  possible  on  the  affected  side,  throws  the  trunk  toward 
the  other.  He  also  stands  in  a  somewhat  characteristic  attitude.  The 
leg  is  slightly  flexed,  the  heel  sometimes  a  little  drawn  up  from  the 
ground,  and  the  trunk  inclined  toward  the  sound  side. 

DELAYED    WALKING. 

The  age  at  which  a  child  learns  to  walk  is  of  some  significance.  As 
a  rule,  a  normal  child  should  stand  alone  before  the  first  year  and  should 
walk  before  eighteen  months  old.  This,  however,  is  subject  to  many 
exceptions  in  both  directions.  Many  children,  especially  European 
children,  who  appear  to  develop  somewhat  earlier  than  American  chil- 
dren, walk  before  they  are  a  year  old,  and  a  child  may  be  perfectly 


950  THE    EYE    AND    NERVOUS    SYSTEM 

normal  in  all  other  respects  who  does  not  walk  until  two  years  old,  or 
even  later.  It  is  always  advisable,  however,  to  make  a  careful  exami- 
nation of  any  child  who  does  not  walk  at  eighteen  months.  Interruption 
in  the  ability  to  walk  is  of  great  clinical  significance.  It  is  not  un- 
common for  children  to  walk  a  few  steps  at  an  early  age,  and  then,  as 
a  result  of  fright  from  a  fall,  or  of  timidity,  or  of  other  unimportant 
causes,  to  refuse  to  attempt  it  again  for  several  weeks.  If,  however,  a 
child  who  has  walked  for  any  considerable  time  gradually  loses  the 
power,  it  usually  means  that  some  disease  of  the  central  nervous  sys- 
tem is  in  progress.  It  is  very  common,  for  example,  for  children  suffer- 
ing from  the  various  forms  of  degenerative  atrophy,  particularly  of  the 
hereditary  types,  to  lose  the  power  of  walking  at  about  the  time  these 
diseases  manifest  themselves.  Thus,  in  cases  of  the  amaurotic  form  of 
family  idiocy  the  children  cease  to  Avalk  about  the  age  of  two  years. 
This  is  also  true  of  many  other  forms  of  idiocy. 

REFERENCES. 

ViERORDT.     Das  Gehen  des  Menschen,  Tubingen,  1881. 

DE  LA  TouRETTE.     Etude  clinique  sur  la  Marche,  Paris,  1885. 

Marey.     La  photographic  du  mouvement,  Paris,  1892. 

Blocq.    Les  Troubles  de  la  Marche,  Paris.  1892 

Braune  and  Fischer.     Slichs.  Abhandl.,  1895-1900. 

MoNKEMoLLER  and  Kaplan.     Neurolog.  Centralbl.,  1900,  p.  798. 

Jendrassik.     Deutsch.  Archiv.  flir  klin.  Med.,  vol.  Ixx,  p.  81. 


CHAPTER    XXIII. 

DEGENERACY   (DEGENERATION,    CONSTITUTIONAL 
DEFECTS,    DEVIATION.) 

By  G.  L.  WALTON,  M.D. 

The  terms  degeneration  and  degeneracy  in  their  academic  sense 
denote  a  lowering  in  the  scale  of  being,  particularly  with  regard  to 
moral  qualities.  The  custom  has  crept  in  to  apply  them  to  all  evi- 
dences, physical  or  mental,  of  deviation  from  the  average  normal,  and 
to  call  the  possessor  of  such  evidences  degenerate.  The  term  superior 
degenerate,  originally  introduced  to  designate  the  individual  with  men- 
tal deviation,  has  been  extended  to  include  the  possessor  of  minor  signs 
of  deviation,  whether  physical  or  mental.  This  use  of  language  is 
unfortunate,  since  it  implies  a  conclusion  by  no  means  established,  and 
■conveys  an  opprobrium,  lessened,  it  is  true,  by  increasing  familiarity, 
but  in  the  majority  of  instances  quite  inappropriate.  The  desirability 
of  introducing  the  purely  descriptive  terms,  deviation  and  deviate,  will 
be  considered  later. 

This  subject,  aside  from  its  general  importance,  is  of  special  interest 
to  the  neurologist,  since  the  so-called  stigmata  of  degeneration  have  to 
be  constantly  taken  into  consideration  as  bearing  on  the  diagnosis  and 
prognosis  of  nervous  disorders. 

To  the  oculist  the  subject  is  of  equal  interest  for  two  reasons.  In 
the  first  place,  the  so-called  stigmata  of  degeneration,  a  long  list  of 
which  will  be  found  at  the  end  of  the  chapter,  include  a  large  number 
of  ocular  defects,  such  as  astigmatism  and  other  errors  of  refraction, 
difference  in  color  of  irides,  flecks  on  the  iris,  coloboma,  and  albinism, 
besides  such  functional  disturbances  of  vision  as  night  blindness  and 
achromatopsia.  In  the  second  place,  it  is  of  practical  importance  for 
the  oculist,  as  well  as  for  the  neurologist,  to  recognize  these  signs,  since 
they  often  indicate  the  constitution  and  character  of  the  individual, 
whatever  relation  they  may  bear  to  degeneration.  For  in  estimating 
the  relation  of  eye-strain  to  headache  or  other  nervous  disturbances,  it 
must  be  remembered  that  such  symptoms  may  be  constitutional  as  well 
as  reflex. 

Among  the  most  obvious  and  most  uniformly  listed  "  stigmata  of 
degeneration,"  facial  asymmetry  comes  first.     A  common  form,  but 

951 


952  THE    EYE    A:N^D    T^ERVOUS    SYSTEM 

unusual  degree,  of  facial  asymmetry  is  illustrated  by  Figs.  1,  2,  and 
3.  One  eye  appears  higher  than  the  other  without  corresponding  ele- 
vation, or  even  with  depression,  of  the  mouth  and  chin.  A  tendency 
of  this  sort,  apparent  or  real,  will  be  noted  in  a  large  percentage  of 
normal  individuals  without  reference  to  righthandedness ;  in  a  less 
number  the  eye,  mouth,  and  chin  will  all  appear  higher  on  one  side.  A 
difference  of  one  to  three  millimetres  in  the  length  of  the  two  cars 
frequently  also  appears;  the  writer  has  found  this  difference  in  about 
two-thirds  of  ears  examined,  no  relation  appearing  between  length  of 
ears  and  symmetry  of  face. 

Xo  reason  appears  for  deeming  this  asymmetry  of  the  face,  even 
when  verified  by  measurement,  a  sign  of  degeneration.' 

The  variety  of  facial  asA,Tiimetry  shown  in  Fig.  4  is  not  easily  ex- 
plained as  a  mere  twist  in  the  skull  or  as  a  preponderance  of  material 
on  one  side.  It  will  also  be  noticed  that  the  ears,  though  fairly  well 
formed,  are  badly  set  (too  low).  Perhaps  complete  knowledge  of  the 
subject  would  place  this  case  in  quite  another  category  from  the  others. 
Until  such  knowledge  shall  have  been  acquired,  are  we  justified  in 
classing  all  as  degenerates  ?  It  is  true  that  Dante  has  been  reckoned 
among  the  degenerates,^  but  it  would  be  reasoning  in  a  circle  to  deem 
on  this  account  his  cranial  asymmetry,  or,  in  fact,  any  of  the  peculi- 
arities on  which  the  diagnosis  may  have  been  based,  signs  of  degen- 
eracy. 

Unusual  size  of  lips  represents  a  less  common,  but  equally  insig- 
nificant, deviation  from  the  average  normal.  This  peculiarity  has 
recurred  in  one  royal  family  with  sufficient  regularity  to  be  named  the 
"  Ilapsburg  lip."     (Figs.  5  and  6.) 

Another  facial  peculiarity  is  undue  prominence  of  the  lower  part 
of  the  face.  If  a  horizontal  line  be  drawn  from  the  external  auditory 
meatus  to  the  junction  of  the  inferior  nasal  spine  with  the  alveolar 
process  (Broca's  horizontal),  and  is  cut  by  a  line  from  the  root  of  the 
nose,  the  facial  prominence  is  shown  by  the  varying  angle  between  these 


'  Dwight  (Scribner's  Monthly,  April,  1891)  states  that  the  right  eye  is  generally 
the  higher.  He  shows  that  the  left  eye  of  the  Venus  de  Milo,  photographed  behind 
a  framework,  appears  higher,  and  questions  if  this  is  not  characteristic  of  left- 
handedness.  In  a  personal  communication  he  has  assured  the  writer  that  he 
regards  asymmetry  of  the  skull  and  face,  not  as  degenerative,  but  due  to  mechanical 
causes.  It  is  interesting  to  note  in  this  connection  that  among  the  many  hundred 
inmates  of  the  Massachuseets  School  for  Feeble  Minded,  observed  with  reference 
to  this  point  by  the  Superintendent,  Dr.  Fernald,  as  well  as  by  the  writer,  facial 
asymmetry  was  found  no  more  marked  or  prevalent  than  among  average  individuals. 

'Lombroso,  The  Man  of  Genius.     1901. 


Fig.  1.— Common  form  of  facial  asymmetry,  but  unusual  in  degree. 


Fig.  2.— Bust  of  Filippo  Strozzi  by  Baudetto  da  Maiano.     (Berlin  Museum.)      Right  side  of  face 

appears  longer. 


Fig.  3.— Bust  of  Dante.    ( From  bronze  in  Naples  Museum.)    Left  face  appears  longer. 


Fig.  4. — Less  common  form  of  facial  asymmetry. 


Fig.  5.— The  "Hapsburg  lip,"  as  shown  by  Albert,  Archduke  of  Austria,    An  example  of  deviation, 

not  degenerate. 


Fig.  6. — The  same  family  peculiarity  as  shown  by  Leopold  I. 


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DEGENERACY  953 

lines,  or  by  the  angle  between  the  latter  line  and  a  vertical  dropped 
from  the  root  of  the  nose  to  the  horizontal. 

The  accompanying  old  print  (Fig.  7)  shows  the  deviation  of  the 
facial  angle  of  Ferdinand  VII.  from  the  ideal,  as  represented  by  the 
profile  of  Bentham.  It  seems  probable  that  this  is  classed  more  appro- 
priately under  the  stigmata  of  degeneration  than  simple  facial  asym- 
metry, though  even  this  point  is  not  really  established.  It  was  stated 
in  the  original  legend  that  this  represented  the  prognathism  generally 
found  in  kings ! 

The  illustration  of  Charles  V.  of  Spain  (Fig.  8)  presents  a  further 
example  of  the  Bourbon  prognathism. 

In  the  ease  of  Charles  V.  there  was  also  projection  of  the  lower 
teeth,  and  it  is  said  this  malformation  of  the  jaw  seriously  impeded 
mastication. 

Ears  badly  set,  asymmetrical,  of  unusual  shape,  lacking  normal 
markings  and  normal  parts,  fall  into  the  category  of  deviations  which 
have  been  grouped  as  degenerative,  but  like  most  of  the  so-called  stig- 
mata, each  peculiarity  deserves  separate  study  before  accepting  this 
sweeping  dictum.  For  example,  ears  set  too  low,  or  lacking  lobes  and 
markings,  may,  perhaps,  be  degenerative,  while  a  prominent  antihelix 
or  a  moderate  difference  in  length  may  have  no  such  significance. 

It  is  not,  however,  the  purpose  of  this  paper,  to  group  this  long  list 
of  deviations  with  regard  to  their  bearing,  or  lack  of  bearing,  on  the 
question  of  degeneration.  The  illustrations  cited  will  suffice  to  show 
that  the  estimation  of  their  varying  significance  requires  detailed  and 
exhaustive  study.  This  study  is  of  practical  importance  in  that  it 
imparts  knowledge  regarding  the  constitution  of  the  individual,  a 
knowledge  which  helps  the  physician  to  weigh  symptoms.  Whichever 
of  these  deviations,  alone  or  in  combination,  represent  degeneration, 
it  is  true  that  they  are  generally  more  marked  and  more  abundant  in 
the  insane,  the  criminal,  and  the  idiotic,  than  in  the  well-balanced  and 
the  moral.  This  is,  in  fact,  the  important  argument  for  attributing  to 
all  deviations  the  stigma  which  belongs  only  to  a  part,  and  to  that  part 
in  an  uncertain  degree.  It  is  also  true  that  signs  of  deviation  are  com- 
mon among  those  possessing  signs  of  inherited  nervous  and  mental 
instability  as  well  as  hysteria,  epilepsy,  hypochondria,  and  other  psychi- 
cal and  physiological  evidences  of  constitutional  peculiarity  or  defect. 
In  fact,  these  tendencies  have  been  included  in  the  list  as  psychical 
and  physiological  stigmata  of  degeneration. 

Given,  therefore,  a  case  in  which  any  of  these  symptoms  are  sus- 
pected of  resulting  from  eye-strain,  it  behooves  the  practitioner  to  be 


954  THE    EYE    AND    NERVOUS    SYSTEM 

on  the  lookout  for  other  signs,  both  physical  and  mental,  of  deviation, 
for  the  more  he  finds,  the  greater  is  the  probability  that  the  symptom 
in  question  is  due,  in  part  or  in  whole,  to  the  constitutional  makeup  of 
the  individual,  rather  than  simply  to  reflex  irritation. 

THE    BEARING    OF    CONSTITUTIONAL    DEVIATION    UPON    THE 
ESTIMATION     OF     EYE-STRAIN     SYMPTOMS. 

The  study  of  the  relation  between  eye-strain  and  symptoms  refer- 
able to  the  nervous  system  has  been  handicapped  by  the  apathy,  not  to 
say  incredulity,  on  the  part  of  the  patient,  general  practitioner,  neurolo- 
gist, and  sometimes  even  oculist,  regarding  the  variety  and  extent  of 
symptoms  which  may  be  produced  by  the  continuous,  though  apparently 
insignificant,  irritation  resulting  from  moderate  refractive  error.  (See 
Chapter  XVIII.) 

On  the  other  hand,  before  predicting  relief  of  nervous  symptoms 
by  correction  of  refraction,  one  definite  factor  in  diagnosis  must  be 
carefully  estimated,  namely,  the  constitution  of  the  individual.  In  a 
considerable  number  of  cases  the  error  of  refraction  is  merely  one  evi- 
dence of  a  faulty  organism,  among  other  signs  of  which  appear  head- 
ache, the  invalid  habit,  vertigo,  indigestion,  irritability,  and  allied 
nervous  states,  to  say  nothing  of  choreic  movements,  epilepsy,  and  other 
motor  phenomena  whose  relation  to  eye-strain  is  not  yet  definitely  deter- 
mined. While  many  of  these  symj^toms  are  sometimes  solely  dependent 
upon  eye-strain,  hence  susceptible  of  amelioration  by  glasses,  and  while 
the  adjustment  of  glasses  is  often  followed  by  gratifying  results  even 
in  the  constitutionally  defective,  a  certain  proportion  of  cases  will  baffle 
the  most  skilful  and  painstaking  ocular  treatment. 

With  a  view  of  emphasizing  the  practical  bearing  of  this  subject, 
it  seems  appropriate  at  this  point  to  refer  to  a  few  of  the  most  impor- 
tant and  constant  symptoms  requiring  analysis  by  the  neurologist  and 
the  oculist,  with  reference  to  eye-strain  as  an  etiological  factor. 

The  most  constant  and  important  of  these  conditions  is  cephalalgia, 
a  symptom  frequently  relieved,  and  even  cured,  by  simple  correction  of 
refractive  error.  To  Dr.  S.  Weir  Mitchell  is  due  the  credit  of  first  call- 
ing general  attention  to  this  relation,  a  relation  apparently  already  rec- 
ognized in  practice  by  various  oculists. 

It  is,  however,  not  unusual  that  a  sufferer  from  persistent  headache 
is  forced  to  apply,  in  turn,  to  the  oculist,  the  rhinologist,  and  the  gynae- 
cologist. Examination  discloses,  successively,  an  error  of  refraction,  a 
deviated  septum,  or  projecting  turbinate,  and  a  misplaced  or  otherwise 
abnormal  uterus ;   but  each  successive  special  treatment,  though  encour- 


DEGENERACY  955 

aged  by  definite  pathological  findings,  results  in  only  temporary  and 
insignificant  relief  of  the  headache.  In  such  a  case  the  underlying 
constitutional  defect  has  been  overlooked.  This  headache  is  often 
vaguely  localized,  often  vertical,  is  less  apt  to  be  limited  to  the  fore- 
head and  the  occiput,  and  is  less  often  distinctly  migrainous  (see 
Chapter  XVII.)  in  seat  and  character  than  that  of  the  purely  reflex 
ocular  variety. 

The  exact  nature  of  the  constitutional  headache  cannot  be  deter- 
mined. It  seems  improbable  that  there  is  a  definite  physical  basis.  In 
such  event  we  are  forced  to  resort  to  the  unsatisfactory  expression 
"  functional."  In  some  cases  the  description  of  suffering  is  so  much 
at  variance  with  the  appearance  and  demeanor  of  the  patient  that  the 
examiner  is  forced  to  the  conclusion  that  the  so-called  suffering  is  an 
interpretation  put  by  the  patient  upon  what,  in  another,  if  noticed  at 
all,  would  be  merely  a  discomfort ;  in  other  words,  that  the  normal 
pressure  of  jjart  upon  part  of  the  cranial  contents,  or  the  normal  changes 
in  circulation  of  the  various  fluids,  or  the  sensation  accompanying  nor- 
mal mental  effort,  phenomena  unnoted  by  the  average  individual,  may 
be  acutely  and  uncomfortably  felt  by  the  highly  sensitive  possessor  of 
the  psychopathic  temperament. 

A  well-recognized  analogy  is  found  in  tinnitus  aurium,  a  symptom 
generally  due  to  aural  disease,  and  often  susceptible  of  relief  by  aural 
treatment,  but  resulting  sometimes  (in  the  morbidly  sensitive  or  hys- 
terical individual),  from  the  mere  irritability  of  the  sensorium,  unable 
to  accustom  itself  to  the  physiological  impressions  received  by  the 
auditory  nerve. 

This  variety  of  headache  has  been  described  and  classified  by  writers 
on  degeneracy  as  constitutional  headache,  and  must  be  recognized  as 
a  sign  of  defective  nervous  makeup,  of  which  the  refractive  error  and 
the  nasal  and  pelvic  anomalies  furnish  merely  contributory  evidence. 

Another  symptom  not  infrequently  relieved  by  glasses  is  nervous 
irritability.  Here  again  the  practitioner  should  strive  to  distinguish 
between  simple  nervousness  produced  by  continuous  peripheral  irrita- 
tion, and  the  various  mental  peculiarities  resulting  from  inherent,  and 
inherited,  tendencies.  The  practitioner  is  often  made  aware  of  the 
mental  attitude  of  patients  of  the  latter  class  by  a  peculiar  fussiness, 
by  complaints  of  the  temperature  of  the  room,  by  a  call  for  a  fan,  a 
glass  of  water,  the  shading  of  a  light,  the  closing  of  a  window.  They 
often  adopt  an  antagonistic  attitude,  and  question  the  need  of  the 
various  steps  in  the  examination.  Such  patients,  while  complaining 
of  most  excruciating  torture,  are  often  unwilling  to  make  the  sacrifice 


956  THE    EYE    AND    NERVOUS    SYSTEM 

of  wearing  glasses  for  its  relief,  or  if  tliey  make  the  trial  are  unable 
to  accustom  themselves  to  the  bows  over  the  ears,  the  sight  of  the  rims, 
or  the  reflection  from  the  glasses.  The  efforts  of  the  oculist  are  still 
further  handicapped  by  the  inability  of  such  patients  to  relax  the  mus- 
cles of  accommodation  after  the  adjustment  of  glasses.  They  are  also 
peculiarly  prone  to  attempt  extension  of  vision  beyond  its  legitimate 
limits,  and  to  look  from  side  to  side,  though  the  latter  tendency  may 
be,  in  a  measure,  somewhat  counteracted  by  terescopic  or  toric  lenses. 

While  the  psychopath  describes  his  headache  in  most  extravagant 
terms,  no  corresponding  signs  of  physical  suffering  appear.  Though 
complaining  of  utter  collapse,  such  a  patient  will  often  complete  a 
long  examination  with  smiling  countenance  and  showing  less  sign  of 
exhaustion  than  the  examiner.  The  history  in  such  cases  will  often 
disclose  also  hypochondriacal  tendencies,  morbid  fears,  antipathies, 
hyperconscientiousness,  or  mysophobia  (fear  of  contamination).  A 
combination  of  mental  traits  pointing  in  the  same  direction  has  been 
well  summarized  by  Soukhanoff  ^  under  the  "  Ideo-Obsessive  Consti- 
tution," 

Individuals  with  this  peculiarity  are  easily  recognized.  They  are 
"  over-scrupulous,  disquieted  over  trifles,  indecisive  in  action,  and  anx- 
ious about  their  affairs.  They  are  given  early  to  morbid  introspection, 
and  are  easily  worried  about  their  own  indispositions  or  the  illnesses 
of  their  friends.  They  are  impressionable,  possess  considerable  amour 
propre,  and  are  easily  offended ;  but  as  they  are  usually  reticent  they 
hide  their  resentment  and  are  often  secretly  ashamed  of  those  bizarreries 
peculiar  to  their  disposition.  They  are  often  timorous  and  apprehen- 
sive, and  prone  to  pedantism.  The  moral  sentiments  are  pronounced 
in  most  cases,  and  if  they  are,  as  a  rule,  somewhat  exigent  and  egotistic, 
they  have  a  lively  sense  of  their  own  defects." 

H3rpochondria  has  been  made  a  special  subject  in  another  chapter 
(XVI.),  but  deserves  further  brief  mention  in  this  connection.  Hypo- 
chondria implies  undue  attention  to  symptoms,  and  undue  alarm  con- 
cerning them ;  it  may  exist  alone  or  in  combination  with  other  diseases, 
organic  or  functional.  This  tendency  shows  itself,  as  a  rule,  early  in 
the  investigation.  The  visit  of  the  hypochondriac  is  apt  to  be  preceded 
by  a  complete  history  of  his  case  in  writing.  He  is  likely  to  consult 
memoranda  in  the  elaboration  of  this  history,  and  to  describe  his  symp- 
toms with  great  particularity.     He  is  prone  to  describe  his  sufferings 


*Rev.  Neurolog.,  Juin  30,  1903,  p.  613.     Abstract  in  Review  of  Neurology  and 
Psychiatry,  October,  1903,  p,  703. 


DEGENERACY  957 

in  anatomical  terms ;  for  example,  instead  of  stating  he  has  a  pain  in 
the  side,  he  locates  the  pain  in  the  lung,  the  heart,  or  the  intercostal 
nerve.  He  comes  to  the  examiner  with  a  preconceived  and  unshakable 
diagnosis  of  his  own  case,  and  in  its  defence  exhibits  great  argumenta- 
tive ingenuity.  He  is  generally  anxious  and  willing  to  try  a  new 
remedy,  usually  with  the  result  of  temporary  improvement.  After  the 
patient's  departure  the  physician  may  expect  to  receive  letters  contain- 
ing further  elaboration  of  symptoms. 

These  psychoneuroses,  still  often  loosely  included  under  the  title 
of  neurasthenia,^  are  not  to  be  mistaken  for  results  of  eye-strain,  though 
marked  refractive  error  is  extremely  likely  to  appear;  and  while  no 
effort  should  be  spared  to  contribute  to  the  comfort  of  the  patient  by 
removing  every  possible  source  of  reflex  irritation,  the  chance  is  remote 
of  overcoming  inherent  tendencies. 

Another  condition  for  the  relief  of  which  glasses  are  not  infre- 
quently recommended  is  epilepsy.  The  repeated  failure  of  this  pro- 
cedure is  undoubtedly  due  to  the  fact  that  in  the  vast  majority  of  cases 
this  disease  represents  a  constitutional  defect  in  the  cerebral  mechanism. 
(Compare  Chapter  XVIII.) 

If  experience  had  shown  that  overuse  of  other  muscles  than  those 
of  the  eye  produced  generalized  convulsions  with  impairment  of  con- 
sciousness, the  burden  of  proof  would  be  upon  those  denying  that 
epilepsy  is  a  common  result  of  eye-strain,  but  in  the  absence  of  such 
evidence  overwhelming  proof  is  required  to  fortify  the  position  of  those 
who  support  this  proposition. 

In  the  overuse  of  other  muscles  (for  example,  those  of  the  forearm 
and  hand  concerned  in  writing),  if  spasm  appears  it  is  practically 
limited  to  the  muscles  involved ;  there  is  certainly  no  generalized 
spasm ;  nor  is  there  a  suggestion  of  impaired  consciousness,  the  one 
essential  symptom  of  epilepsy.  In  eye-strain  the  analogue  of  the  local 
cramp  is  found  in  the  spasm  of  accommodation,  a  well-recognized 
symptom.  It  is  true  that  the  analogy  between  eye-strain  and  writers' 
cramp  •  is  not  complete  in  every  particular,  inasmuch  as  the  pain  of 
writers'  cramp,  in  contradistinction  to  that  of  eye-strain,  is  usually 
limited  to  the  overused  member,  and  generally  appears  during,  or  im- 
mediately after,  the  overuse  of  the  part.  The  divergence  from  this 
rule  in  the  case  of  pain  resulting  from  eye-strain  is,  however,  insig- 
nificant compared  with  the  introduction  of  such  new  elements  as  gen- 
eralized convulsions  and  impairment  of  consciousness. 


*  See  Dana,  The  Partial  Passing  of  Neurasthenia. 


958  THE    EYE    AND    NERVOUS    SYSTEM 

In  one  case  of  epilepsy  under  the  care  of  the  writer  an  interval, 
of  sixteen  months  followed  the  correction  of  a  very  obvious  refractive 
error.  At  the  end  of  this  period,  however,  the  attacks  returned  in  spite 
of  the  continued  efforts  of  a  particularly  skilful  refractionist.  In  prac- 
tically all  the  epileptics  coming  under  the  writers  observation  in  the 
Neurological  Department  of  the  Massachusetts  General  Hospital  and 
elsewhere,  the  question  of  refraction  has  been  carefully  considered,  but 
its  correction  has  yielded  negative  results  beyond  occasional  variations 
in  the  duration  of  the  intervals,  variations  too  inconsiderable  and  too 
inconstant  to  offer  proof  of  material  benefit,  and  often  to  be  regarded 
as  a  mere  coincidence. 

Such  experiences  have  led  to  scepticism  regarding  the  cure  of  epi- 
lepsy by  adjustment  of  glasses,  and  while  search  through  the  literature 
reveals  a  sufficient  number  of  apparently  ameliorated  cases  to  preclude, 
perhaps,  the  final  closing  of  this  branch  of  investigation,  it  may  at  least 
be  stated  that  the  dependence,  in  any  considerable  proportion,  of  epi- 
lepsy on  eye-strain,  and  its  cure  by  refractive  correction,  is  yet  to  be 
established. 

The  prevalence  of  convulsive  attacks  in  the  epileptic  patient  after 
overloading  the  stomach  has  not  infrequently  led  to  the  conclusion 
that  the  attacks  were  of  reflex  nature  with  digestive  disorder  as  under- 
lying cause,  but,  while  regulation  of  the  diet  often  lessens  the  attacks,  it 
signally  fails,  in  the  majority  of  cases,  to  reach  the  real  seat  of  the 
trouble.  Epilepsy  is,  then,  like  the  refractive  error  and  the  indigestion, 
generally  only  another  sign  of  constitutional  defect.  In  point  of  fact, 
careful  search  through  the  family  history  of  the  epileptic  will  generally 
reveal,  if  not  epilepsy,  hysteria,  or  insanity,  at  least  a  prevalence  of 
some  of  the  morbid  mental  tendencies  to  which  reference  has  been 
made  in  the  consideration  of  constitutional  headache  and  nervous  irri- 
tability. 

In  the  attempt  to  relieve  chorea,  habit  chorea,  hysteria,  and  insanity, 
to  say  nothing  of  indlgestlQu,  vomiting,  and  insomnia  by  refractive 
correction,  the  effort  must  be  made  in  each  case,  before  making  a  prog- 
nosis, to  determine  how  great  a  part  is  played  in  the  production  of  the 
clinical  picture,  not  only  by  other  causes,  but  also  by  the  inherent 
makeup  of  the  individual. 

If  there  are  signs  by  which  constitutional  instability  can  be  detected 
at  the  outset  of  the  examination,  the  knowledge  of  such  signs  will  be 
of  material  aid  in  diagnosis  and  prognosis.  Reference  has  already 
been  made  to  the  mental  attitude  sometimes  characterizing  these  pa- 
tients.    Compulsive  acts  will  sometimes  be  noted ;    frequent  grunting, 


Fig.  8.— a  further  illustration  of  the  prognathism  of  the  Bourbons,  as  shown  by  Charles  V.  of  Spain, 


Fig.  9.— Ears  asymmetrical,  badly  shaped,  badly  set.    (From  Massachusetts  School  for  Feeble  Minded, 
through  kindness  of  Dr.  Femald. ) 


Fig.  10.— Absent  IoIjc,  crumpled  helix,  antihelix  prominent  and  of  unusual  shape.   Tndsoura  intertragica 
broadened,  concha  large.    (Same  source  as  Fig.  9. ) 


Fig,  11.— Helix  narrow,  thin,  and  irregularly  shajwd,  with  suggestion  of  Darwinian  tubercle;  antihelix 
prominent ;  abnormal  fold  in  lobe.    ( Same  source  as  Fig.  9. ) 


DEGENERACY  959 

blinking,  or  sniffing,  mannerisms  in  speech  and  deportment  are  sugges- 
tive. Among  the  physical  peculiarities  may  be  noted  deviations  in  form 
of  the  skull  and  face,^  unusual  shape,  size,  or  position,  of  the  orbits, 
narrow  palpebral  fissure,  excessive  prominence  of  the  forehead,  prog- 
nathism and  projection  of  the  lower  teeth  (Figs.  7  and  8),  peculiarity 
in  shape  of  the  palate,  peg-shaped  incisors,  hair  upon  the  face  (in  the 
female),  ears  badly  shaped,  unlike,  or  badly  set  (Figs.  9,  10,  and  11), 
and  curvature  of  the  spine.  It  must  not  be  understood  that  the  physical 
and  mental  stigmata  always  go  hand  in  hand. 

DEVIATION. 

The  various  constitutional  defects  and  peculiarities  have  been 
summed  up,  as  has  been  said,  rather  loosely,  under  the  term  degeneracy 
or  degeneration.  These  stigmata  include  every  conceivable  variation 
from  the  average  normal  (Dana,  Church  and  Peterson,  and  others), 
ranging  all  the  way  from  such  innocuous  peculiarity  as  a  difference  in 
color  of  the  two  eyes,  to  perversion  and  imbecility.  It  has  not,  how- 
ever, been  yet  established  that  every  sign  of  deviation  from  the  average 
normal  is  degenerative,  and  it  seems  to  the  writer  ^  desirable  to  name 
the  phenomena  signs  of  deviation  and  to  call  their  possessor  deviate, 
or  a  deviate,  as  the  case  may  be,  limiting  the  term  degeneration  only 
to  such  deviations  as  obviously  imply  deterioration,  either  of  the  indi- 
vidual or  of  the  race.  The  nomenclature  which  has  hitherto  obtained 
in  anthropological  study,  largely  through  the  writings  of  Nordau  "^  and 
of  Lombroso,*  if  applied  to  the  vegetable  kingdom,  would  without 
investigation  stigmatize  the  four-leaved  clover  as  degenerate. 

The  illogical  nature  of  the  present  method  is  illustrated  by  the 
statement  of  Lombroso  ^  that  four  per  cent,  of  normal  individuals  have 
five  or  moriB  signs  of  degeneration.  Would  it  not  be  equally  descriptive 
and  more  accurate  to  say  that  this  percentage-  of  normal  individuals 
have  signs  of  deviation  ? 

BIOGRAPHICAL    STUDY    OF    CONSTITUTIONAL 
DEVIATIONS. 

A  careful  study  of  the  lives  of  various  men  prominent  in  literary 
and  scientific  work  would  indicate  that  they  were  hampered  by  eye- 


^  The  usual  form  of  asymmetry,  previously  discussed,  has  probably  no  bearing. 

'  Walton,  The  Prevailing  Conception  of  Degeneracy  and  Degenerate,  with  a 
Plea  for  Introducing  the  Supplementary  Terms  Deviation  and  Deviate.  Boston 
Medical  and  Surgical  Journal,  January  21,  1904. 

'  Degeneration.  Max  Simon  Nordau.     New  York:    Appleton  &  Co.,  1895. 

°  L'uomo  delinquente  ecc,  1889. 

*  Lombroso.  in  Twentieth  Century  Practice  of  Medicine.     New  York,  1897,  p.  381. 


you  THE    EYE    AND    NERVOUS    SYSTEM 

strain.  ( Gould.  ^*')  But  the  same  study  made  with  due  consideration 
for  possible  etiological  factors  other  than  eye-strain,  for  the  mental  and 
physical  discomforts  under  which  they  labored,  shows  that  the  problem 
is  not  so  simple.  It  is  a  question  whether  enthusiasts  in  other  branches 
of  medicine  might  not  find  ample  evidence  of  the  injurious  effects  of 
pathological  states  involving  other  organs.  Might  not,  for  example, 
the  dentist  attribute  De  Quincey's  indigestion  to  his  defective  teeth? 
Or,  again,  might  not  the  diathetic  enthusiast  find  sufficient  evidence 
in  Carlyle's  case  to  claim  an  excess  of  uric  acid  as  the  cause  of  his 
unfortunate  disposition  ?  However  this  may  be,  the  history  of  such 
cases  is  apt  to  reveal  signs,  physical,  mental,  or  both,  of  constitutional 
peculiarity.  Carlyle  in  his  boyhood  was  shy,  proud,  pugnacious,  with 
strong  affections  and  with  equally  violent  antipathies.  In  later  life  he 
declared  war  upon  the  neighbor's  poultry,  and  had  constructed  a  room 
for  his  labors,  proof  against  sound.  De  Quincey  was  a  man  of  diminu- 
tive stature,  given  to  day-dreaming  before  he  became  addicted  to  his 
unfortunate  habit,  the  inability  to  resist  which  may  quite  as  plausibly 
be  attributed  to  constitutional  defect  as  to  the  discomfort  caused  by 
eye-strain. 

The  exalted  character  of  the  many  celebrities  cited  by  Lombroso,^' 
in  establishing  his  conclusion  that  genius  is  a  degenerative  psychosis, 
accentuates  the  necessity  of  supplementing  the  word  degeneracy  by 
some  less  opprobrious,  while  equally  descriptive  and  inclusive  term. 
Even  the  fact  that  many  of  these  individuals  showed  decided  deteriora- 
tion in  the  physical  or  moral  scale  does  not  militate  against  adopting 
a  term  for  the  classification  of  their  variations  from  the  normal  before 
drawing  conclusions  regarding  the  significance  of  those  variations.  In 
dealing  with  genius  it  is  well  to  remember  also  that  the  impression  pre- 
vailing in  popular,  not  to  say  scientific,  circles,  that  moral  qualities 
stand  in  an  inverse  ratio  to  intellectual  endowment,  has  been  contro- 
verted by  the  statistical  investigations  of  Woods.^^ 

Etiology. — The  varied  etiology  of  the  so-called  stigmata  of  degener- 
ation would  already  indicate  great  diversity  in  their  downward,  nega- 
tive, and  even  occasionally  upward  values.  The  wide  and  indiscrim- 
inate use  of  the  word  degeneration  has  led  to  conflicting  views  of  the 
origin  of  the  stigmata.  Critical  review  of  this  etiology  is  as  far  beyond 
the  ability  of  the  writer  as  beyond  the  scope  of  this  treatise,  but  brief 


'"Biographic  Clinics.     Philadelphia:    P.  Blakiston's  Sons  &  Co.,  1903. 
"  The  Man  of  Genius.    London:    Walter  Scott,  1901. 

"  F.  A.  Woods.  The  Correlation  between  Mental  and  Moral  Qualities.     Popular 
Science  Monthly.  October,  1903. 


DEGENERACY  961 

mention  of  some  of  the  suggested  causes  will  be  in  place.  From  Nor- 
dau's  point  of  view  it  is  natural  that  disease,  privation  and  excesses  in 
the  ancestry  should  be  regarded  the  main  factors  in  the  production  of 
degeneracy,  and  it  is  doubtless  true  that  such  causes  result  in  enfeeble- 
ment,  undersize,  and  defective  resistance  in  the  progeny ;  but  according 
to  modern  scientific  views  the  presence  or  absence  of  inherent  charac- 
teristics transmitted  by  the  germ  are  independent  of  such  influences. 
In  other  words,  acquired  characteristics  are  not  transmitted. 

The  present  anomalous  use  of  the  term  degeneration  includes  among 
the  deviations  from  the  average  normal,  even  such  evolutional  spurts 
as  unusual  development  of  Broca's  convolution  in  the  individual  pos- 
sessed of  unusual  linguistic  aptitude  (Lombroso).  This  use  of  the  term 
renders  inadequate  the  etiology  of  Nordau.^^  Paul  Jacoby  ^*  has 
endeavored  to  show  that  selective  tendency  in  aristocratic  circles  is 
answerable  for  sterility  and  family  decadence.  Similarly,  Ireland  ^^ 
has  laid  stress  on  indolence  and  the  "  insanity  of  power"  as  causing 
degeneracy  in  royal  families.  The  studies  of  Woods  ^^  clash  with  these 
conclusions,  for  he  has  shown  by  the  analysis  of  many  royal  families, 
in  which  the  surroundings  and  manner  of  life  remain  practically  con- 
stant factors,  that  while  some  families  have  deteriorated  others  have 
advanced,  the  variations  in  the  individuals  and  families  depending  pre- 
eminently on  the  combinations  of  characteristics  inherited. 

Classification  of  Causes  of  Deviation. — I.  Modern  embryological  in- 
vestigations have  shown  that  the  inherent  and  therefore  hereditary  char- 
acteristics transmitted  through  the  germs  entirely  dominate  the  struc- 
ture of  the  progeny .^"^  Just  what  characteristics  the  child  will  inherit 
from  each  parent  cannot  be  foretold  ;  still  less  can  it  be  predicated  what 
combinations  of  more  remote  ancestral  traits  will  appear  in  any  given 
progeny.     For  the  study  of  this  fundamental  branch  of  the  subject  the 


"Prior  to  Nordau's  more  popular  treatise,  Morel  (Traits,  etc.,  1857,  p.  17) 
had  dwelt  upon  the  constitutional  basis  for  insanity. 

"  Etudes  sur  la  Selection  dans  ses  Rapports  avec  I'HereditS  chez  THomme, 
Paris,  1884. 

"The  Blot  upon  the  Brain.     G.  P.  Putnam's  Sons,  New  York,   1896. 

"Mental  and  Moral  Heredity  in  Royalty.  Popular  Science  Monthly,  August, 
1902,  to  April,  1903. 

"  Wilson  summarizes  the  present  view  of  maturation  of  the  sexual  elements 
thus :  "  In  both  sexes,  the  final  reduction  in  the  number  of  chromosomes  is  effected 
in  the  course  of  the  last  two  cell  divisions  by  which  the  definite  germ  cells  arise, 
each  of  the  germ  cells  thus  formed  having  but  half  the  usual  number  of  chromo- 
somes. In  the  female  but  one  of  the  germ  cells  forms  the  ovum  proper,  while 
the  other  three,  known  as  the  polar  bddies,  are  minute,  rudimentary  and  incapable 
of  development."  "  In  the  male,  on  the  other  hand,  all  four  of  the  cells  become 
61 


962  THE    EYE    AND    NERVOUS    SYSTEM 

reader  is  referred  to  Weissmann,^^  Minot,^®  Wilson,^®  and  other  author- 
ities, and  for  experimental  studies  in  transmission,  to  the  researches  in 
heredity  inspired  by  the  discovery  of  Mendel's  law  (de  Vries,  Correns, 
Tschennak,  Bateson,  Castle.) 

Weissmann's  view,  that  acquired  characteristics  cannot  be  transmit- 
ted, is  accepted  by  the  majority  of  biologists  ^^  and  tends  still  further  to 
accentuate  the  importance  of  the  original  nuclear  substance  in  the  trans- 
mission of  stigmata  of  deviation,  though  constitutional  enfeeblement  of 
varied  forms  in  the  progeny  may  well  result  from  deleterious  influences, 
such  as  defective  nutrition  of  the  germ  in  utero,  due  to  the  progenitors. 

Inherited  tendencies  certainly  constitute  an  important  factor  in 
the  production  of  the  psycho-neuroses,  hysteria  and  insanity. 

(a)  The  tendency  of  family  characteristics  to  recur  after  a  period 
of  latency  is  known  as  atavism,  a  phenomenon  which  would  seem  espe- 
cially prone  to  occur  in  the  offspring  of  extremely  dissimilar  parents. 
Darwin  found,  for  example,  that  by  crossing  a  black  Spanish  and  white- 
silk  fowl  offspring  could  be  produced  with  red  plumage  resembling 
that  of  the  wild  ancestor  common  to  both  races.  (Mercier.^^)  Ri- 
band ^^  denies  degenerative  tendency  in  this  class  of  cases,  and  suggests 
that  in  man  Polydactyly  (supernumerary  fingers)  and  polymastia 
(many  breasts)  come  under  the  same  category,  the  former  serving  as  a 
reminder  of  the  numerous  swimming  rays  of  the  fishes,  the  latter  of 
the  multiple  breasts  of  mammals  inferior  to  man.     Whether  Bibaud'a 

functional  spermatozoa," — "  the  polar  bodies  are  therefore  to  be  regarded  as  abor- 
tive eggs."  Again  "if  it"  (the  process  of  reduction  of  chromosomes)  "did  not 
occur,  the  number"  (of  chromosomes)  "would  be  doubled  in  each  succeeding 
generation  through  union  of  the  germ  cells."  He  quotes  Weissmann's  explanation 
of  the  reason  for  the  reduction  as  preventing  the  excessive  accumulation  of  differ- 
ent kinds  of  hereditary  tendencies  or  germ  plasms. 

"  Ueber  Vererbung,  Jena,  1883,  1885.  Die  Continuitat  dea  Keimplasmas  u.  s.  v. 
Jena,  1883,  1885.  Ueber  die  Zahl.  der  Richtungs  Korper.  u.  s.  v.  Jena,  1887.  Essays 
on  Heredity,  Oxford.  1891,  1892. 

"Human  Embryology.     Wm.  Wood  &  Co.,  N.  Y.,  1892. 

"The  Cell  in  Development  and  Inheritance.     The  MacMillan  Company.  1896. 

"Minot  states  authoritatively  that  the  "hypothesis  of  pangenesis"  (Darwin's 
theory  that  the  egg  consists  of  a  multitude  of  germs  or  gemmules  thrown  off  from 
each  part  or  unit  of  the  parent  body,  each  gemmule  possessing  the  properties  which 
the  unit  had  when  they  were  thrown  oflF)  "both  in  its  original  form  and  in  all  its 
subsequent  modifications,  has  been  definitely  set  aside";  again,  "the  child  is  like 
the  parents  because  its  organism  is  regulated  by  not  merely  similar,  but  by  some 
of  the  same  chromatin  as  that  of  the  parents,"  adding  that  possibly  tlie  more 
general  term  nuclear  substance  would  be,  perhaps,  preferable  in  place  of  chromatin. 
(Loc.  cit.  p.  90.) 

="Mercier,  Sanity  and  Insanity.     Ix>ndon:    Walter  Scott. 

="  Abstracted  from  Revue  Soientifique  in  the  Literary  Digest,  December  5.   1903. 


DEGENERACY  903 

views  receive  general  acceptance  or  not,  this  line  of  thought  will  serve 
to  remind  us  that  the  absence  of  neurotic  history  in  the  immediate 
family  does  not  preclude  such  inheritance  from  a  distant  ancestor.  It 
is  possible  that  Mendel's  laws  of  dominance  and  recession  (latency) 
may  prove  of  aid  in  determining  the    reappearance  of  deviative  signs. 

(&)  The  view  long  accepted  without  question  that  the  effect  of 
consanguineous  marriage  in  itself  is  harmful  is  voiced  by  Mercier,  who 
states  that  while  a  certain  degree  of  dissimilarity  between  parents  pro- 
duces the  best  offspring,  too  great  similarity  in  parents  tends  to  enfee- 
blement  and  idiocy,  while  too  great  dissimilarity,  if  productive  at  all, 
tends  towards  wildness  in  the  offspring.  It  is,  he  says,  on  account  of 
the  deleterious  effect  of  parental  similarity  that  marriage  between  near 
relatives  is  undesirable,  but  if  it  should  chance  that  cousins  obtain  their 
prepotent  qualities  from  different  ancestors  rather  than  from  the  same 
ancestor,  the  liability  to  deterioration  in  the  offspring  is  lessened. 

If  the  prepotent  qualities  are  represented  by  heavy  lines  in  Fig.  12, 
the  cousins  C,  C,  children  of  brothers  B,  B,  inherited,  in   (a),  pre- 


c  c  o  o 


(a) 

Fig.  12.— After  Mercier. 


(6) 


potently  the  characteristics  of  this  common  (paternal)  grandfather,  O, 
hence  their  marriage,  according  to  Mercier,  would  be  unwise.  One  of 
the  cousins  in  (&),  however,  derives  prepotent  qualities  from  the  mater- 
nal grandparent  (G^),  so  that  the  risk  to  posterity  is  reduced  to  the 
minimum. 

This  view  of  the  deleterious  effects  of  close  intermarriage  is  not 
borne  out  by  the  extended  investigations  of  A.  H.  Iluth,^^  excepting 
in  so  far  as  the  cousins  inherit  in  common  vicious  tendencies. 

Whatever  evil  effects  may  follow  continued  inbreeding,  they  may 
be  materially  reduced  by  the  introduction  of  new  blood  after  several 
generations,  as  in  the  case  of  the  most  notable  instance  on  record  of 
continued  consanguineous  parentage,  namely,  that  of  the  Ptolemies. 
(Mercier.)      The  repeated  recurrence  of  the  Hapsburg  lip   (Figs.   5 


"Castle,  Proceedings  Amer.  Acad,  of  Arts  and  Sciences,  vol.  xxxviii.    No.   18. 
With  bibliography.     Cambridge,  Mass.,  January,  1903. 
■■'^The  Marriage  of  Near  Kin,  etc.     London,   1887. 


964  THE    EYE    AND    NERVOUS    SYSTEM 

and  6)  furnishes  another  illustration  of  the  effect  of  (c)  pure  selective 
tendency  in  the  parents,  an  illustration  which  it  would  not  be  just  to 
stigmatize  without  further  analysis  as  degenerative.  (Woods.)  It  is 
also  of  interest  in  this  connection  to  study  the  collection  of  Meige,^® 
illustrating  the  degenerate  types  in  art.  This  collection  includes  cases 
of  myxcpdema,  achrondroplasia,  and  rhachitic  diathesis,  as  well  as 
individuals  showing  marks  of  moderate  deviation  from  the  average 
normal  among  the  subjects  treated  by  various  prominent  painters,  nota- 
bly Velasquez. 

In  the  production  of  insanity,  heredity  and  stress  are  essential  and 
variable  forces;  the  greater  the  instability  of  the  nervous  system  the 
less  the  stress  required.  (Mercier).  Similarly,  the  character  of  an 
individual  depends  on  both  heredity  and  environment.  Environment 
and  education  can  do  much  to  modify  inherited  characteristics ;  but 
education  and  surroundings  can  no  more  entirely  remove  the  evidences 
of  psychopathic  endowment  than  they  can  efface  the  anatomical  signs 
of  deviation. 

II.  In  the  causation  of  such  degenerative  conditions  as  hydro- 
cephalus (Fig.  13),  Vaschide  and  Vurpas  ^"  have  called  attention  to 
the  infective  processes  operating  upon  the  developing  embryo.  Upon 
this  etiological  factor  the  writer  is  not  qualified  to  comment,  further 
than  to  suggest  that  in  comparison  with  the  long  list  of  stigmata  cited, 
it  seems  of  comparatively  limited  application. 

III.  Still  another  cause  of  deviation  may  be  found  in  mechanical 
influences  affecting  the  embryo.  Dr.  Whitney  (Curator  of  the  Warren 
Museum)  has  suggested  amniotic  adhesions  and  amputation  by  the 
foetal  cord  (Figs.  14  and  15). 

Possibly  the  common  asymmetries  of  the  face  should  fall  under  this 
head. 

IV.  Certain  anomalies,  such  as  congenital  absence  or  peculiar 
growth,  of  certain  parts,  whether  due  to  absence  of  certain  elements 
in  the  germ,  or  to  their  later  disappearance  or  faulty  development,  have 
been  termed  freaks  of  nature.  The  fact  that  they  are  not  yet  explained 
does  not  preclude  a  definite  etiology.  The  mandible  hand  (Fig.  16) 
must  be  i)laced  in  this  category. 

This  class  of  deviations  sometimes  recurs  in  families.  This  was 
the  case  in  the  defective  lobe  in  Fig.  IT. 


^  La  Presse  MMicale,  Juillet  15,  l«o:}. 

"Ann.  di  Nevro.  February   1,   1903.     Abstr.  in  Review  of  Neurology  and  Pay 
chiatry,  June  1,  1903. 


Fig.  13.— a  remarkable  degree  of  hydrocephalus.  Fig.  11.— Amputation  by  strangulation  of 

(Copyright,  1S88,  by  Langill,  Hanover,  N.  H. )  foetal  cord.    ( From  cast  in  Warren  Museum. ) 


T''  7 


Fig.  15.- Results  of  amniotic  adhesions.    (From  plates  in  AMfeld,  Misbildang  des  Menschen  1880. ) 


u    3 

1  § 


2  i 


li 


«5 


5  5| 


d  t-  S 
w  c>  o 


Fig.  19.— Group  of  mlcrocephaUc  inmates  of  Massachusetts  School  for  Feeble  Minded.    (By  kindness 

of  Dr.  Fernald.) 


Fig.  20.— Group  of  macrocephalic  inmates  of  the  same  institution. 


Fic.  21.— Casts  of  palates  from  the  collection  of  Dr.  Channinjr.  They  illustrate  varieties  in  shape, 
width,  height,  and  symmotry ;  one  shows  "torus  palatimus;"  none  are  absolutely  norinal.  All  are 
taken  from  the  feeble  minded. 


Fig.  22. — Inmate  of  the  same  institution,  aged  23.    Absence  of  hair  on  face  and  pubes,  hypospadias, 
infantile  penis,  cryptorchismus,  muscular  insufficiency. 


DEGENERACY  965 

V.  The  investigations  of  Thompson  tend  to  show  that  mechanical 
influences  affecting  the  development  of  the  infant  should  be  given  a 
place.  He  maintains  that  the  shape  of  the  skull  depends  largely  on 
the  cranial  capacity,  and  in  some  degree  also  upon  the  strength  of  the 
temporal  muscle.  This  investigator  replaced  the  calvarium  by  a  rubber 
bag  to  represent  the  membranous  condition  of  this  part  of  the  skull 
during  its  developmental  period ;  moderate  inflation  of  the  bag  pro- 
duced a  shape  resembling  that  of  the  ape's  skull  with  slight  frontal 
development,  and,  viewed  from  in  front,  a  shape  suggested  by  that  of 
the  idiotic  child  shown  in  Fig.  18  (for  which  I  am  indebted  to  Dr. 
Waterman). 

Great  distention  produced  the  normal  adult  shape.  This  differ- 
ence was  accentuated  by  weight-bearing  cords  attached  to  the  bag  in 
the  region  of  the  temporal  muscle.  The  question  is  complicated  by 
the  difference  in  leverage  under  Avhich  the  temporal  muscle  w^orks  with 
different  shaped  jaws,  the  long  arm  of  the  lever  being  the  condylo- 
symphyseal  measurement,  the  short,  the  condylocoronoid  measurement. 
The  similarity  in  shape  of  microcephalic  and  macrocephalic  skulls  re- 
spectively in  the  groups  of  feeble  minded  (Figs.  19  and  20)  seems  to 
lend  weight  to  Thompson's  hypothesis. 

VI.  It  has  already  been  stated  that  constitutional  disease,  depriva- 
tion, and  excesses  in  the  ancestry  may  so  far  impair  the  nutrition  of 
the  germ  as  to  cause  undersize,  enfeeblement,  and  lessened  resistance 
in  the  offspring  though  not  influencing  its  inherent  characteristics. 

YII.  Certain  glands  have  a  distinct,  though  not  thoroughly  under- 
stood, influence  on  the  nutrition  of  the  body;  hypertrophy  or  defect 
of  these  glands  produce  marked  variation  in  its  growth.  Giantism 
is  found  associated  with  enlarged  pitituary  gland,  and  results  perhaps 
from  its  oversecretion ;  possibly  dwarfism  implies  diminution  of  this 
secretion,  though  this  point  has  not  been  investigated  as  far  as  the 
writer  is  aware.  Acromegaly,  a  condition  in  which  the  face,  jaws, 
hands,  and  feet  become  enormous  in  later  life,  after  the  bones  have 
acquired  their  length,  is  also  found  associated  with  disease  of  this 
gland  and  deserves  a  place  in  the  same  category  with  giantism.  De- 
fective glandular  tissue  in  the  thyroid  produces  cretinism  in  infancy, 
myxoedema  in  adult  life. 

YIII.  Dr.  Minot  (to  whom  I  am  indebted  for  critical  revision  of 
the  portions  of  this  chapter  which  bear  on  embryology)  suggests  that  a 
fundamental  etiological  factor  should  be  added,  namely,  such  lack  of 
development  as  is  evidenced  by  harelip.  He  attributes  the  erroneous 
use  of  the  word  degeneration  mainly  to  failure  to  recognize  the  essential 


966  THE    EYE    ANI>    NERVOUS    SYSTEM 

steps  in  degejierative  processes,  embraced  under  cytomorpliosis.  This 
word  he  uses  "  to  designate  comprehensively  all  the  structural  modifi- 
cations which  cells,  or  successive  generations  of  cells,  may  undergo, 
from  the  earliest  undifferentiated  stage  to  their  final  destruction."  *® 
In  other  words,  existence  of  protoplasm  and  its  gro\\i;h  are  prerequisites 
for  degeneration.  A  deposit  of  horny  material  in  the  skin  may  then 
be  correctly  classed  as  degenerative,  in  contrast  to  such  conditions  as 
harelip.  Xor  can  degeneration  of  the  race  be  established  upon  signs 
which  do  not  indicate  degeneration  in  the  individual. 

It  would  seem,  then,  that  we  must  include  among  the  possible  causes 
of  the  so-called  stigmata  of  degeneration  (which  include  signs  of  devia- 
tion only),  at  least 


Fig.  23.— Unusual  developmeut  of  the  breasts  in  a  negro  boy.    (Case  of  Dr.  Spiller.) 

I.  The  potential  variations  from  the  average  normal  contained  in 
the  parent  germ,  including  the  results  (a)  of  atavism,  {b)  oi  parental 
similarity,  and  (c)  of  selective  tendency  on  the  part  of  the  ancestry. 

II.  Intra-uterine  infection. 

TIL  Mechanical  injury  during  intra-uterine  life. 

IV.  The  absence  or  peculiarity  in  the  germ  of  certain  elements, 
or  their  disappearance  or  anomalous  development,  without  traceable 
inherited  tendency  or  other  known  explanation.  To  this  class  of  stig- 
mata the  unsatisfactory  and  doubtless  inadequate  term  "  freak  of 
nature"  (lusus  natura^)  has  been  applied. 


"  A  Laboratory  Text-Book  of  Embryology.     Philadelphia :    P.  Blakiston's  Son 
&  Co..  1903. 


DEGENERACY  967 

V.  Mechanical  influence  exerted  during  infancy. 

VI.  Deleterious  influences  and  habits  in  the  ancestry  (productive 
of  enfeeblement,  undersize,  and  lessened  resistance  in  the  progeny  but 
not  altering  the  essential  potential  characteristics  transmitted  by  the 
parent  germ). 

VII.  xVbsence  or  hypertrophy  of  certain  glands  (pituitary,  thyroid) 
which  have  a  nutritional  influence. 

VIII.  Arrest  of  development  such  as  is  seen  in  harelip  and  similar 

defects. 

List  of  Stigmata. — The  following  list  comprises  a  large  number, 
though  by  no  means  all,  of  the  pathological  conditions  and  anomalies 
which  have  been  cited  by  various  authorities  as  stigmata  of  degenera- 
tion. It  is  obviously  more  appropriate  to  classify  a  considerable  num- 
ber of  them  as  signs  merely  of  deviation.  Some  of  those  whose  pres- 
ence, either  alone  or  in  combination  with  others,  have  little  if  any 
bearing  on  the  question  of  true  degeneracy  are  starred. 

ANATOMICAL    STIGMATA. 

Cranial  Anomalies. 

Asymmetry  of  skull,  especially  frontal  bone. 

Macrocephalus. 

Microcephalus. 

Platycephalus  or  chemocephalus   ( flat ) . 

Leptocephalus  ( narrow ) . 

Oxycephalus  or  acrocephalus   (steeple  shaped). 

Plagiocephalus  (oblique). 

Scaphocephalus  ( keel  shaped ) . 

Trigonocephalus  or  trigonal   (pointed  anteriorly). 

Short  parietal  arc. 

Short  frontal  arc. 

High  prominent  forehead  in  women. 

Heavy  jaws. 

Great  or  unequal  prominence  of  malar  bones. 

Lemurian  hypophysis. 

*  *  )    Unusual   projection,   straightness  or  re- 

Orthognathism.  y  •         *  au    i  * 

"  (        cession  of  the  lower  face. 

Opisthognathism  or  retrognathism.  J 

Large  frontal  sinuses,  small  orbit. 

Crania  progenaea  (lower  teeth  projecting  beyond  upper,  and  inferior  maxillary 

angle  obtuse). 

•Facial  asymmetry. 

Anomalies  of  the  eye. 

Narrow  palpebral  fissure. 

Micropthalmus. 

•Flecks  on  the  iris. 

•Chromatic  asymmetry  of  the  iris. 

Albinism. 

Congenital  cataracts. 


968  THE    EYE    AND    NERVOUS    SYSTEM 

Cbakial  Anomalies   (Continued). 
Anomalies  of  the  eye. 

Pigmentary  retinitis. 

Muscular  insufficiency,  strabismus. 
•Hypernietropia. 

Myopia. 

Astigmatism. 
Deformities  of  the  palate. 

High  and  narrow   (sometimes  associated  with  adenoid  hypertrophy). 

Horseshoe. 

Torus  palantinus  (longitudinal  ridge  along  palatine  suture), 

Gothic  arch. 

Dome-shaped. 

Flat-ioofed. 

Hip-roofed. 

Asymmetrical. 
Dental  Anomalies. 

Badly  set  and  badly  nourished. 

Small  or  peg-shaped  lateral  incisors. 
•Double  rows. 
•Adventitious  teeth. 
•Double  crown. 
•Macrodentism. 

Mierodentism. 

Projecting  teeth. 

Badly  placed  or  misplaced  teeth. 

Striated  transversely. 

Hutchinson's  teeth. 
Anomalies  of  the  nose. 
•Deviation  of  nose. 

Defective  development  of  cartilage  and  tissue  of  alse  (Gudden). 

Absent. 

Atresia  of  nasal  fossa. 

Defective  osseous  development  (nasus  aduncus). 
Anomalies  of  the  tongue  and  lips. 
•Macroglossus. 

Microglossus. 
"Bifidity  of  point. 
•Harelip. 
•Cleft  palate. 
Anomalies  of  the  ear. 
•Excessively  long. 
•Excessively  prominent. 
*Set  too  close  to  the  head. 

Set  too  far  back. 

Set  too  low. 

Absence  of  helix,  antihelix,  or  lobule. 

Obliteration  of  markings. 

Absence  of  fissura  intertragica. 

Too  oonchoidal    (antihelix,  crura,  etc.,  too  little  marked  and  helix  like 

rim  of  funnel). 
•Excessively  large  (absolutely  or  relatively). 

Too  small. 

Lack  of  uniformity  in  width. 


DEGENERACY  969 

Anomalies  op  the  Ear  (Continued), 

Asymmetry  of  the  two  ears,  general  anomaly  of  left  (Blainville  ear). 
•Prominence  of  antihelix. 
"Adherent  lobules  (found  in  a  large  percentage  of  normal  individuals). 

[Definite  types  of  misshapen  ears  are  described  and  named,  for  example,  the 
Stahl,  Nos.  1,  2,  and  3;  the  Wildermuth;  the  Morel;  the  Cagot.  Descriptions  and 
illustrations  of  these  varieties  may  be  found  in  the  text-book  of  Church  & 
Peterson,  p.  628  et  seq.^ 

Anomalies  of  the  Limbs. 

*Left  arm  and  leg  longer  than  right. 
'Excessive  length  of  arms. 
*Long  fingers. 
•Polydactyly. 

Symphysodactyly  or  achistodactylus  (joining  of  fingers). 

Syndactyly  (web  fingers). 

Ectrodactyly  ( fingers  wanting ) . 

Amelus  or  ecromelus   (limb  wanting). 

Phocomelus   (segment  of  limb  wanting). 
*Megalomelus. 
*Megalodactyly. 

Oligomelus  (excessive  gracility). 

Oligodactyly. 

Anomalies  of  the  Trunk  and  General  Structure. 
Hernias. 

Malformation  of  the  breasts  and  thorax. 
Dwarfism. 
Giantism. 
Infantilism. 
Femininism  in  men. 
Maseulinism  in  women. 
*Spina  bifida. 
Feebleness  of  construction. 
Lordosis. 
Scoliosis. 
Kyphosis  (Fere). 
Malformed  coccyx. 
Thoracic  asymmetry. 
Mammary  development  in  males. 
Mammary  absence  or  reduplication  in  females   (polymastia). 

Anomalies  of  the  Genital  Organs. 

Small  or  deformed  genitals. 

Hermaphrodism. 

Cryptorchisnnis. 

Hypospadias. 

Epispadias. 

Defect,  torsion  or  great  volume  of  prepuce. 

Imperforate  meatus. 

Labia  too  large  or  too  small. 

Clitoris  large. 


970  THE    EYE    AND    NERVOUS    SYSTEM 

Akomaliks  of  tue  Genital  Oboans  (Continueo). 
Labia  minora  hypertrophied. 
Folds  between  labia  majora  and  minora. 
Labia  minora  pigmented,  particularly  in  brunettes. 
Imperforate  vulva. 
Artresion  of  vagina. 
Double  vagina. 
Uterus  bicornis. 
Atrophic  uterus. 

Muscular  Anomalies. 
Dystrophies. 

Unequal  inervation  of  facial  muscles  on  the  two  sides. 
Depression  above  glabella  due  to  over-action  of  corrugators. 

Anomalies  of  the  Skin. 

Polysarcia. 

Hypertrichosis  (superfluous  hair). 
*Premature  grayness. 

•Precocious  and  abnormal  hairy  development. 
•Rudimentary  tail. 

Glabrous  chin   (no  beard). 

Vitiligo. 

Absence  of  nails  or  foetal  state  of  nails. 

Melanism  of  skin. 

Pigmented  or  vascular  nevi. 

MoUuscum. 

Ichthyosis. 

Pigmented  spots. 

PHYSIOLOGICAL    STIGMATA. 

Anomalies  of  Motor  Function. 

•Left-handedness. 
Retardation  of  learning  to  walk  and  talk. 
Tremors. 
Epilepsy. 
Nystagmus  (congenital). 

Tics. 

'facial  spasm, 
habit  chorea, 
tic  convulsif. 

Anomalies  of  Sensory  Function. 
Deaf-mutism. 
Neuralgia. 
Migraine. 

Constitutional  headaches. 
Hyperaesthesia. 
Anaesthesia. 
Blindness. 

Daltonism  (color  blindness,  achromatopsia). 
Hemeralopia    (night  blindness). 


DEGENERACY  971 

Anomalies  of   Sensory   Function    (Continxjed). 
Nyctalopia  (day  blindness) . 
Concentric  limitation  of  the  visual  field. 

Anomalies  of  Speech. 

Mutism. 

Defective  speech. 
•Stammering. 
•Stuttering. 

Anomalies  of  Genito-Urinary  Function. 

Sexual  irritability. 

Impotence. 

Sterility. 

Urinary  incontinence.  , 

Amenorrhoea. 

Anomalies  of  Instinct  or  Appetite. 

Mercyism   ( gluttony ) . 

Rumination. 

Uncontrollable  appetites   (food,  liquor,  drugs). 

Diminished  Resistance  against  External  Influences  and  Diseases. 

Retardation  of  Puberty. 

Deficient  Vital  Activity  of  Organic  Functions. 

Weak  heart. 

Low  arterial  tension. 

Coldness  of  extremities. 

Flushing  of  extremities. 

General  chills  and  flushes. 

Weak  digestion. 

Constipation. 

PSYCHIC   AND    PSYCHO-NEUROTIC   STIGMATA. 

Dementia  prsecox. 
Compulsive  insanity. 
Manic  depressive. 
Melancholia. 
Hysteria. 
Hypochondria. 
Phobias. 

Psychopathic  endowment. 
Invalid  habit. 
Idiocy. 

Feeble-mindedness. 
•Eccentricity. 
Moral  deliquency. 
Sexual  perversion. 
Precocity. 

Overdevelopment  of  certain  aptitudes. 
Paranoia. 
Ideo-obsessive  constitution. 


INDEX. 


¥¥ 


Abducen?  nerve, 

intracerebral  anatoraj-  of,  16 

paralysis  of,  from  meningitis,  16 

palsy  of, 

symptoms  of,  235 
with  migraine,  717 
Aberration,  36 

varieties  of,  36 
Abiotrophy,  645 
Accessorius  nerve, 

cerebral  portion  of, 

intracerebral  anatomy  of,  18 

spinal  portion  of, 

intracerebral  anatomy  of,  19 
Accommodation, 

anomalies  of, 

hysterical,  641,  640,  644 
pathogenesis  of,  645 

determination  of  power  of,  56 

failure  of,  with  age, 
symptoms  of.  778 

impairment  of,  with  paresis,  484 

loss  of,  with  multiple  neuritis,  605,  608 
with  tabes,  504 

measurement  of,  38 

mechanism  of,  37 

power  of  at  different  ages,  39 

painful,  with  hysteria,  640 

palsy  of,  with  brain  tumor,  404 

range  of,  38 

spasm  of, 

epileptic,  957 
Achromatopsia, 

(see  Color  blindness),  88 
Acoustic  nerve, 

intracerebral  anatomy  of,  17 
Acromegaly, 

cause  of,  559 

diagnosis  of,  561 

differential,  564 

ocular  signs  of,  561 

Packard's  case  of,  560 

pathology  of,  562 

symptoms  of,  559  to  562 

treatment  of,  564 

with  pituitary  disease,  426 
Agrammatism,  144 

cause  of  (theoretical).  147 

mechanism  of,  144 

training  in  treatment  of,  145 
Agraphia  and  allied  conditions, 

cause  and  mechanism  of.  1.39 

with  migraine,  725 
Akataphasia,  146 
Alcoholism. 

ocular  signs  of.  525-528 
Aleucopia,  87 
Alexia,  1.38 

causes  of.  138 

treatment  of,  142 

with  brain  tumor.  390 
A  tnaurosis, 

(see  Blindness) 
Amaurotic,  family  idiocy, 

cause  of,  534 


Amaurotic,  family  idiocy,  characteristics  of,  532 

history  of,  532 

ophthalmoscopic  signs  with,  532 

pathology  of,  535 

symptoms  and  course  of,  535,  533 

treatment  of,  536 
Amblyopia,  central  or  muscular,  108 

cortical,  102 

crossed,  102 

exanopsia,  177 

feigned, 

tests  to  detect,  692 

hysterical,  621 

explanation  of,  113 

simulation  of,  to  sympathetic  disease,  669 

monocular,  108 

pretended,  692 

uraemic,  177 

with  alcoholism,  525 

with  lead  intoxication,  528 

with  liver  disease,  742 

with  migraine,  700,  703 

with  multiple  neuritis,  603 

with  paresis,  479,  486 
Amblyoscope  test,  204 
Ammetropia,  see  Refraction  anomalies 
Amnesia, 

color,  88,  101 

lesions  causing,  122 
Amorphopia,  87 

Amyotrophic  lateral  sclerosis,  580 
Anasmia, 

pernicious, 

ocular  and  spinal  signs  of,  582,  583 

visual  disturbances  with,  741 
Anaesthesia, 

corneal,  322 

conjunctival.  171 
Anaphoria,  782 
Anisocoria,  314 
Anisometropia,  49 
Anomia, 

color,  101 

visual,  148 
Anophthalmus,  552 
Anopia, 

cortical, 

differential  diagnosis  of,  85 
Antimetropia.  49 
Aphasia, 

color,  88 

causes  of,  101 

crossed,  135 

graphic, 

epilepsy  with.  1.39 

optic,  148 

lesions",  causing,  149 

teaching  in  treatment  of,  143 

visual,  115 

treatment  of,  142 
varieties  of,  118 

with  brain  tumor.  390.  .398,  399 

with  migraine,  708,  725 
Apraxia, 

sensory,  120 

visual,  118 

973 


974 


INDEX 


Aqueous  homor  of  eye,  30 

Astasia  abasia  with  exophthalmic  goitre,  830 

Astereognosis, 

causes  of,  122 

visual  disorders  with,  114 

with  exophthalmic  goitre,  830 

with  brain  tumor,  396 
Asthenic  paralysis,  457 
Asthenopia, 

accommodation,  811 

causes  of,  781,  814,  779 

constitutional,  815 

detinition  of,  750 

effect  of,  on  neurasthenia,  779 
on  refraction,  781 

facial  expression  in,  814 

from  strenuous  life,  779 

general,  811 

hysterical,  659,  640 

muscular,  781,  813 

neurasthenic,  672 
in  children,  673 

reflex,  815 

symptoms  of,  818 
Astigmia, 

of  refraction,  46 

measurement  of,  49 

varieties  of,  47 
Ataxia,  cerebellar,  393 

hereditary,  diagnosis  of,  475 

resemblance     of,  to     Friedreich's 

ataxia,  550 
with  small  cerebellum,  550 

Friedreich's,  474 

diagnosis  of,  475 
ocular  signs  of,  474 

locomotor  (see  Tabes) 

with   brain   tumor,  393,  400,  403,  404,  421, 
429,  431 

with  multiple  neuritis,  602 
Athetosis, 

atrophy  of  cerebrum  with,  549 
Atrophy, 

primary  neurotic,  852 
Attention  reflex,  304 
Auditory  nerve, 

palsy  of, 

with  ocular  palsy,  240 
Aurae  (visual), 

with  epilepsy,  732 

with  migraine,  700 


B 

Bar  reading  test,  203 
Basedow  s  (lisease 

(see  Exophthalmic  goitre),  828 
Baudry's  test,  693 
Blepharospasm,  336 

diagnosis,  prognosis  and  treatment,  337 

epileptic,  733 

hysterical,  651 

tabetic,  502 
Blindness,  alcoholic,  526 

uua.>mic,  741 

color  (see  Color  blindness,  88) 

diagnosis,175 

epileptic,  734 

green,  88 

hysterical.  614 
bilateral,  614 
intermittent,  616 
nature  of,  619 
unilateral,  616 

letter,  137 


Blindness,  mind,  118 

causes,  122,  127 
numeral,  142 
object,  118 
paroxysmal,  466 
peripheral,  173 
psychic,  118 
red,  88 

reflex  (supposed),  329 
soul,  118 

with  acromegaly,  562 
with  brain  tumor,  391 
with  epilepsy,  734 

with  lead  intoxication,  528  « 

with  migraine,  700 
with  multiple  neuritis,  602,  604 
with  myelitis,  567,  568 
Blind  spot,  158 

detection  of,  159 
enlargement  of,  166 
Brachial  plexus, 
diseases  of,  592 

ocular  signs  of,  593 
Brain,  anomalies  in  development  of,  545 
Brain  tumors,  387 

Benedict  symptom  complex  of,  422 
course  and  signs  of,  387 
dangers  of  lumbar  puncture  in,  395 
difl'erential  diagnosis  of,  432 
lesions  simulating,  432 
of  cerebellum,  430 

surgical  treatment  of,  877 
of  cerebral  peduncle,  421 
of  chiasm  surgical  treatment  of,  876 
of  corpora  quadrigemina  of,  403 
of  frontal  lobe,  395 
of  medulla  and  pons,  429 
of  motor  area,  399 
of  occipital  lobe,  387 

surgical  treatment,  873 
of  optic  nerve,  428 
of  optic  thalamus,  400 
of  parietal  lobe,  395 
of  pituitary  body  and  pineal  gliind,  426 
of  temporal  lobe,  399 
symptoms  of,  387 

resemblance    of,     to     multiple 
sclerosis,  419 
Bulb  (medulla  oblongata), 

acute  apoplectiform  paralysis  of,  445 
aneurism  of,  451 
disease  of,  451 

symptoms  of,  435 

with  typhoid  fever  and  leukaemia, 
451 
embolism,  hemorrhage,  and   thrombosis  of, 

445 
meningeal  thickening  of,  451 
myelitis  of,  451 
neuritis  of,  451 
progressive  palsy  of,  436 
softening  of,  448 
traumatic  affections  of,  451 
Bulbar  (and  pseudobulbar)  diseases,  435 
general  symptomatology  of,  435 
paralysis  (palsy),  436 

acute  apoplectiform,  445 


Campbell's  conclusions,  72 
Car  sickness 

due  to  asthenopia,  819 
Catalepsy, 

from  eye-strain,  758 


INDEX 


975 


Cavernous  sinus, 

thrombosis  of,  868 

diagnosis  of,  871 
surgical  aspects  of,  871 
symptoms  of,  870 
Centres  (cerebral), 
color,  76 

convergence,  195 
grammatical,  148 
language  co-ordinating,  148 
macula,  77 
naming,  150 
of  sight,  76 
speech  and  allied,  135 
visual  (primary),  76 

relation  of  eyes  to  (diagram),  129 
relation  to  optic  tracts 
(scheme  of),  104 
Cerebellum, 

diagnosis  of   lesions  of,  (see  also  Brain  tu- 
mors), 424  et  seq. 
operations  on, 

anatomic  considerations,  in  878 
indications  for,  877 
palliative,  883 
results  of,  884 
technique  of,  879 
small,  resemblance  to  Friedreich's  disease, 

550 
tumors  of 

(see  Brain  tumors) 
Cerebral 

absence,  549 
atrophy,  549 
defects,  549 
fissuration, 

abnormal,  551 
heterotopia,  551 
palsies 

(see  Paralysis) 
conjugate,  266 
of  children,  538 
sclerosis,  diffuse,  474 
surface, 

function  of,  72 
Choked  disc, 

operations  for  relief  of,  877 
significance  of,  in  brain  tumor,  426 
Chorea, 

atrophy  of  cerebrum  with,  549 
facial,  801 
habit  spasm,  799 
relation  of  eye-strain  to,  799 
with  brain  tumor,  400,  421 
with  exophthalmic  goitre,  830 
Chorioid, 

anatomy  of,  24 
Chromatic  aberration,  36 
Chromatopsia, 

hysterical,  669 
Chromidrosis, 

hysterical,  669 
Ciliary 
body, 

anatomy  of,  23 
muscle, 

hypersesthesia  of  (hysterical),  640 
nerves, 

origin  of,  25 
Clock-face  chart,  53 
Cochlear  nerve, 

intracerebral  anatomy  of,  17 
amnesia,  anomia,  aphasia,  101 
Colorblindness  (achromatopsia),  172 
causes  and  detection  of.  172 
central,  88 


Color  blindness  (achromatopsia),  hysterical,  101, 
633 

partial,  88 
uniocular,  102 
with  myelitis,  667 
disorders, 

hysterical,  101,  633 
field, 

contraction  of, 

with  acromegaly,  562 
hysterical,  629,  630 
fundamental 

centres  for  recognition  of,  92  et  seq. 

clinics  pathologic  position  of,  94  et 
seq. 
cortical  per5eption  of,  90 
Wildbrand's  hypothesis  of,  92 
impairment, 

focal  causes  of,  91 
hysterical,  101 
phenomena, 

cerebral,  88 
hysterical,  101,  633 
terms  used  in  study  of,  89 
theory  of,  90 
recognition, 

cortical  centres  for,  88 
sensations, 

centre  for,  76 

cerebral  activity  regarding,  89 
hysterical  disturbance  of,  633,  101 
paths  of  transmission  of,  91 
with  ocular  arteriosclerosis,  466 
scotomata, 

central,  163 
hysterical,  631 
Conjugate, 

paralysis  and  spasm, 
causes  of,  272 
cerebellar,  270 
cerebral,  269 
course  of,  266 
diagnosis  of,  267 

diflferential  ,  267,  272 
nature  of,  266 
pontine,  270 

site  of  lesion  causing,  269 
diagnosis  of,  271 
symptoms  of,  266 
treatment  of,  273 
restricted  movements, 
non-paralytic,  267 
spasmodic  deviation, 
hysterical,  661 
Conjunctiva  and  cornea, 
anaesthesia  of,  322, 

hysterical,  667 
anatomy,  21 
ecchymosis  of, 

with  epilepsy,  736 
erosions  of,  329 
hypemesthesia  of, 

hysterical,  668 
reflex  of,  351 
ulcer  of, 

dendritic,  329 

with  exophthalmic  goitre,  839 
Conjunctivitis. 

neurasthenic,  686 
with  exophthalmic  goitre,  839 
Contraction, 

amplitude  of,  295 
hysterial  anomalies  of,  657 
Convergence, 

loss  of,  in  tabes,  504 
movements  of,  195 


976 


INDEX 


Convergence,  near  point  of.  206 
nerve  centre  for,  195 
neurasthenic  insufficiency  of,  675 
prism,  206 

reaction  iu  brain  tumor,  404 
reflex,  196 
test-s  for,  206 
unilateral, 

with  exophthalmic  goitre,  836 
Convulsions  due  to  brain  tumor,  395,  399 

to  cerebral  atrophy,  549 
Corpora  quadrigeraina,  diagnosis 
of  lesions  of,  424 
(see  also  Brain  tumors) 
Corpus  callosum, 
absence  of,  550 
tumors  of  (see  Brain  tumors) 
Cough  and  phonation, 

difficulties  of,  in  bulbar  palsy,  438 
Cranial  hernia,  554 
Cranial  nerves 

in  multiple  neuritis,  601 
intracranial  portion  of,  I 
abducens,  16 
accessorius.  19 
acoustic,  17 
cochlear,  17 
facial,  17 

glossopharyngeal,  18 
hypoglossal.  19 
oculomotor.  1 1 
olfactory,  1 
optic,  1 

pneumogastric,  18 
spinal  accessory,  19 
trigeminal  15 
trochlear,  15 
vagus,  18 
vestibular,  18 
nuclei  of, 

defective  development  of,  552 
paralysis  of,  with  acromegaly.  562 

with  brain  tumor.  393,  421,  429 
with  bulbar  palsy,  439 
with  exophthalmic  goitre,  830 
with  migraine.  714.  717,  718 
with  multiple  neuritis,  601,  603, 

604,  605 
with  myelitis,  568 
with  paresis,  488 
with  tabes,  501,  504 
Cranial  sinuses. 

lesions  of,  867 
Craniectomy,  882 
Cutjineous  signs, 

with  exophthalmic  goitre,  841 
Cycloplegia  (see  Palsy  of  accommodation) 
Cycloplegics 

for  testing  vision.  51 


Dalrymple's  sign,  350,  385 
Deafness, 

with  brain  tumor,  404.  423.  429,  431 

with  ocular  palsy,  240 

with  Ubes,  502 
Deafness  (mind),  122 
Deafness  (word), 

with  migraine,  726 
Degeneracy,  stigmata  of.  951 
etiologv  of,  960 
list  of.  967 
Deglutition  and  mastication, 

difficulties  of.  in  bulbar  palsy,  4.38 


Dementia, 

with  multiple  neuritis,  604 
Deorsum  version,  268,  269 
Deviation  (constitutional), 

biographical  study  of,  959 

causes  of,  961 

in  relation  to  eye-strain,  959,  954 

signs  of,  959 
Diabetes, 

visual  disorders  of,  741 
Digestive  disorders, 

due  to  exophthalmic  goitre,  843 

due  to  eye-strain,  807,  808 

due  to  migraine,  701 
Dihitor  pupillaj,  286 

sympathetic  tnvct  of,  297 
Diplopia,  • 

artificial  or  prism,  184 

character  of  images  in,  187 

crossed,  183 

heteronymous,  183 

homonymous,  183 

incongruity  of  images  in,  187 

overcoming,  185 

pathological,  184 

mechanism  of,  184 

physiological,  183 

prism  or  artificial,  184 

varieties  of  (table),  186 

with  ocular  palsy,  209,  213 

with  epilepsy,  735 

with  migraine,  717 

with  tabes,  502 
Dissociated  eye  movements, 

hysterical,  666 
Divergence, 

anomalies  of,  249 

in  vertical  plane,  196 

movements  of,  196 
centres  for,  196 
tests  for,  207 

paralysis,  hysterical,  658  , 

squint  due  to,  255 
Double  consciousness, 

with  migraine,  725 
Dumbness  (word),  148 
Dyschromatopsia,  88 

epileptic,  733 

hysterical.  631 
Dyskinesia,  245 
Dyslexia,  139 


Emmetropia,  40,  749 
Encephalitis,  superior 

(Wernicke's  disease), 

analogy  of,  to  poliomyelitis,  453 
Encephalocele,  554 
Enophthalmus, 

with  myelitis,  567 
Entropion, 

spasmodic,  337 
Epilepsy, 

definition  of,  782 

etiology  of,  784 

from  ophthalmologic  examination,  737 

ocular  signs  of,  731,  789 

resemblance  of,  to  migraine,  710 

retinal,  734 

sequelai  (ocular)  of,  737 

simulation  of,  by  hysteria,  637 

treatment  of.  795 

with  eye-strain,  757,  957 

with  multiple  neuritis,  604 
Epiphora, 

hysterical,  668 


INDEX 


977 


Epiphora,  with  exophthalmic  goitre,  840 
Erb-Hoppe-(ioldHiuii, 

syiiiptoin  complex  of,  457 
Erb's  disease,  457 

etiology  and  course  of,  461 
symptoms  of,  459 
treatment  of,  462 
Erythropsia  (red  vision),  176 
Esophoria, 

asthenopia  from,  781 
muscular  anomalies,  with,  781 
refractive  errors,  with,  781 
Exophthalmic  goitre, 
bibliography  of,  849 
corneal  changes,  with,  839 
course  and  prognosis  of,  843 
cutaneous  signs  of,  841 
digestive  signs  of,  843 
exophthalmus,  with,  831 
frequency  of,  844 
generative  signs  of,  843 
lachrymation  anomalies  with,  840 
lid  signs  of,  833 
nervous  signs  of,  829 
ocular  signs  of,  831 

unusual,  840 
pathology  of,  844 
respiratory  signs  of,  841 
treatment  of,  845 

surgical,  847 
urinary  signs  of,  843 
vascular  signs  of,  828 
Exophthalmus, 

neurasthenic,  687 
surgical  treatment  of,  847 
with  acromegaly,  561 
with  goitre,  831 
with  myelitis,  568 
Expression  (staring), 

with  exophthalmic  goitre,  835 
External  rectus, 

paralysis  of,  from  meningitis,  16 
Eye,  aqueous  humor  of,  30 
arteriosclerosis  of,  465 

affections  caused  by,  468 
associated  movements  of,  406-408 
ataxia  of,  244,  505 
binocular  movements  of,  193 
check  ligaments  of,  189 

action  of,  190 
conjugate  deviations  of, 

causes  of,  406 
deviations  of,  179, 

anomalous,  283 
conjugate,  406 
lateral  epileptic,  734 
disjunctive  movements  of,  283 
dryness  of,  * 

with  exophthalmic  goitre,  840 
equilibrium  of,  143 
examination  of,  152 
fundus  of  (normal),  362 
hysterogenetic  zones  about,  668 
illumination  (oblique)  of,  156 
imperfections  of,  as  refractory  apparatus,  35 
lateral  deviation  of 

epileptic,  734 
monocular  movements  of.  194 

tests  for,  204 
movements  of, 

anomalies  of  (tests  for),  207 
associated, 

hysterical.  408 

influence  of  cerebral  cortex  on,  408 
palsy  of,  410 
ataxia  of,  244.  505 

62 


Eye,  movements  of,  binocular,  193 
centre  for,  406 
lateral  (palsy  of),  410 
centre  for,  406 
transitory  palsy  of,  407-408 
normal  condition  of,  207 
physiology  of,  178 
searching,  273 
muscles  of,  188 
action  of,  190 

field  of,  193 
anatomy  of,  188 
ataxia  of,  244 
internal,  285 

and  external, 

interdependence  of  function  of, 
778 
operations  on, 

for  insanity,  853 

mental  disturbances  after,  854 

bibliography  of,  865 
psychologic  effect  of,  852 
paralyzed, 

rule  to  determine,  215 
position  of, 

determination  of,  154 
equilibrium,  193 
protrusion  of, 

see  also  Exophthalmus,  156 
refractory  condition  of, 
determination  of,  50 
properties  of,  31 
surfaces  and  media  of,  34 
relation  of  (physiologic), 
to  refractory  errors,  777 
to  visual  centre,  129 
searching  movements  of,  273 
sensitive  spots  about, 

hysterical,  668 
stomach  and 

nerve  connections  between,  808 
strain, 

effects  of, 

accommodation  spasm,  957 
asthenopia,  814 
catalepsy,  758 
chorea, 

relation  of  to,  799 
constitutional  deviation,  959 
epilepsy,  relation  of  to,  957 
errors   of  refraction,   748,  749,  760, 

762 
facial  twitching,  763 
gastric  disturbance,  807,  808 
headache,  743  et  seq. 
and  insomnia,  764 
character  of,  955 
location  of,  752 
mechanism  of,  773 
treatment  of,  767  et  seq. 
head-bowing  of  infants,  825 
hypochondriasis,  956 
insanity.  780 
malaise  and  fatigue,  762 
mental  attitude  in,  958 
migraine.  711 

nerve  exhaustion    and  irritability, 
776.  955 

char.acter  of,  956 
visual  apparatus  in,  776 
ocular  neurosis,  758 
ocular  palsies,  822 
physical  characteristics,  959 
poor  progress  at  school,  763 
pseudotorticollis.  823 
psychopathies,  956 


978 


DfDEX 


Eye  strain,  effects  of,  restlessness,  763 

spasm  of  acfoiiiiuodation,  957 

of  head,  821 
spinal  curvature,  823 
torticollis,  823 
vertigo,  803 

diagnosis  of,  804 
treatment  of,  805 
structure  of,  20 

sympathetic  nerves  of,  338 
diseases  of,  340 
tremor  of,  273 

lesions  causing,  280 
vascular  lesions  (epileptic)  of,  736 
vasomotor  disorders  of,  339 
visual  power  of, 

determination  of,  50 


Facial 

anaesthesia,  321 
hemiatrophy  (progressivf).  556 
causes  and  pathology,  556 
diagnosis  and  symptoms,  557 
hemihypertrophy,  558 
nerve, 

clinical  anatomy  of,  332 
intracerebral  anatomy  of,  17 
paralysis  of,  333 
etiology  of,  335 
symptoms  of,  333 
treatment  of,  335 
surgical,  885 

results  of,  891 
with  abducens  paralysis,  16 
with  multiple  neuritis,  602 
with  myelitis,  668 
with  ocular  palsy,  240 
peripheral  lesions  of,  332 
spasm,  335 
twitching, 

due  to  eye-strain,  763 
Fixation, 

binocular, 

derangements  of,  179 
field  of  vision  in,  168 
test  for,  205 
squint,  caused  by,  187 
central, 

mechanism  of,  178 
eccentric, 

mechanism  of,  178 
field  of, 

mechanism  of,  192 
tests  for,  204 
monocular, 

mechanism  of,  178 
tests  for,  197 
Foot-drop, 

with  multiple  neuritis,  594 
Friedreich's  disease,  475 
diagnosis  of,  475 
gait  in,  946 
ocular  signs  of,  474 
Functional  insanity  (see  Insanity),  508 
Fundus  changes 

with  exophthalmic  goitre,  841 
Fusion  faculty, 
centre  of,  182 


Gait. 


Q 


ataxic,  944 
chicken,  943 


Gait,  classification  of,  940 

flaccid  paralytic,  941 

in  anterior  poliomyelitis,  941 

in  astasia  abasia,  948 

in  ataxia  (locomotor),  944 
cerebellar,  946 
cerebral,  947 

in  athetosis,  947 

in  brain  tumor,  400 

in  cerebellar  disease,  946 

in  cerebral  disease,  946 

in  chorea.  947 

in  Friedreich's  disease,  946 

in  hemiplegia,  943 

in  hysteria,  947 

in  lateral  sclerosis,  943 

in  Little's  disease,  943 

in  locomotor  ataxia,  944 

in  multiple  neuritis,  947 

in  muscular  dystrophy,  942 

in  myotonia  congenita,  948 

in  paralysis 

agitans,  948 
flaccid,  941 
spastic,  943 

in  peripheral  neuritis,  941 

in  pseudomuscular  hypertrophy,  942 

in  sciatica,  949 

in  spastic  paralysis,  943 

in  tabes  dorsalis,  944 

in  Thomsen's  disease,  948 

method  of  study  of,  938 

symptomatic,  937 

varieties  of.  940 
Gasserian  ganglion, 

division  of  sensory  root  of,  900 

advantages  of,  901 
technique  of,  901 

excision  of,  902 

technique  of,  903 

physiologic  extirpation  of,  900 
Gastric  disturbances 

due  to  eye-strain,  807 

mechanism  of,  808 

due  to  migraine,  701 
Generative  disorders, 

with  exophthalmic  goitre,  843 
Gerlier's  disease,  465 
Glaucoma, 

sympathectomy  for,  341 
Glossopharyngeal  nerve, 

intracerebral  anatomy  of,  18 
Graefe's  sign,  350,  833 


H 

Harlan's  test,  693 
Head 

bowing  of  infants,  825 
epileptic  deviation  of,  734 
movements  (spasmodic), 
causes  of.  821 
classification  of.  824 
Headache 

and  insomnia  from  eye-strain,  764 
blind. 

in  migraine,  706 
with  asthenopia,  816 
with  exophthalmic  goitre,  830 
with  e.ve-strain,  743  et  seq. 
location  of,  752 
mechanism  of,  773 
treatment  of,  767 
with  migraine,  706 
with  ocular  polsy,  211 
with  refractive  errors,  748 


IN^DEX 


979 


Hemeralopsia 

(day  blindness),  175 
Hemiachromatopsia,  88 
Uemialencopsia,  87 
Hemianiesthesia, 

visual  disturbances  in,  114 
Hemianopsia  (hemianopia). 

cortical,  79 

determination  of,  86 
definition  of  terms  used,  80,  428 
differentiation  of,  85 
relative,  84 

forms  of,  87 

heteronymous,  167 

homonymous, 

causes  of,  167 

hysterical,  634 

incomplete, 

causes  of,  168 

location  of  lesions  in,  81  et  seq. 

subcortical,  86 

temporal  (causes  of),  170 

with  acromegaly  562 

with  brain  disease,  426 

with  lead  intoxication,  529 

with  migraine,  700 
Hemianopic  hallucinations  with  migraine,  708 

pupilreflex,  318 
Hemiasynergy,  431 
Hemichromatopsia,  168 

amblyopia  with,  169 

functional  or  hysterical,  170 

visual  field  defects  in.  169 

lesions  causing,  169 
Hemicrania  (migraine),  701 
Hemiplegia, 

due  to  atrophy  of  cerebrum,  549 

position  of  head  after,  822 
Hereditary  cerebellar  ataxia,  475 
Heterophoria 

(see  Squint),  180 
Hippus,  674,  316 
Hydrencephalocele, 

diagnosis  of.  554 
Hydrocephalus, 

congenital,  551 
signs  of.  552 
Hyperopia 

(hypermetropia),  40 

absolute.  42 

axial,  41 

causes  of  40 

curvature,  40 

facultative,  42 

in  childhood,  42 

index,  41 

latent.  42 

manifest,  42 

total,  42 

treatment  of,  43 
Hypertrophy  of 

half  of  body,  558 

of  internal  organs.  558 

of  limbs,  558 
Hypnotism  and  sleep, 

ocular  manifestations  of,  689 
Hypochondriasis 

from  eye-strain,  956 

ocular  manifestations  of  688 
Hypoglossal  nerve,  19 

palsy  of,  with  myelitis,  568 

with  ocular  palsy,  240 

Hysteria, 

color  fields  in.  629 
ocular  signs  of,  614 
simulation  of,  to  epilepsy,  637 


Hysteria,  simulation  of,  to  exophthalmic  goitre, 
637 

to  insular  sclerosis,  637 

to  migraine,  722 

to  retrobulbar  neuritis,  637 

to  syndrome  of  Webster,  653 

to  syringomyelia,  637 
treatment  of  ocular  disorders  of,  687 
with  exophthalmic  goitre.  830 
with  migraine,  709 
Hysterical 

accommodation  anomalies,  641,  645 
achromatopsia,  631 
alopecia  of  lids,  670 
amblyopia,  669 
anaesthesia  of  cornea,  667 
associated  movement  palsy,  665 
asthenopia,  640,  659 
attacks, 

pupils  in,  649 
blepharospasm,  651 
chromidrosis,  669 
conjugate  deviation,  661 
convergence,  657 
color,  fields,  629 

scotomata  631 

sense,  632 
cycloplegia,  644 
cyclospasm,  641 
divergence  paralysis,  658 
dissociated  eye  movements,  666 
dyschromatopsia,  631 
epiphora,  668 
hemianopia,  634 
byperaesthesia  of  ciliary  muscle,  640 

of  cornea  and  conjunctiva.  668 

of  spots  about  eyes,  668 
insular  sclerosis,  637 
iridochoroiditis,  669 
kopiopia,  640 
light  and  color  sense, 

importance  of,  632 
migraine,  641 
mydriasis,  647,  649 
myosis,  646,  649 
neurasthenia,  672 
neuroses  (traumatic),  672 
nystagmus,  666 
ocular  palsies.  663 

stigmata.  670 
opacity  of  lens,  669 
ophthalmoplegia  interna.  648 
painful  visual  sensations.  640 
paradox  pupil  reaction,  650 
paralysis  of  accommodation,  644  ■ 

of  levator.  655 

of  orbicularis,  654 
ptosis,  652 

morning  and  night.  656 
pupillary  phenomena.  646 

during  attacks.  649 
retinal  changes.  669 
sensitive  spots  about  eye,  668 
spasm  (ocular),  654 
strabismus,  659 
traumatic  neuroses.  672 
triplopia.  643 
visual  field.  633 

sensations,  640 
zones  about  eye.  668 

I 

Imbecility 

with  cerebral  atrophy,  549 
Inferior  dental  nerve. 

exposure  of.  899 


980 


IKDEX 


Infraorbital  nerve, 

exposure  of,  898 
Insanity  caused  by  eye-strain,  780 
functional, 

ocular  signs  of,  508 
summary  of,  524 
relief  of, 

operations  on  eye  for,  853 
with  exophthalmic  goitre,  830 
Iriilo  choroiditis 

hysterical,  669 
Iridolegia, 
reflex, 

causes  of,  589 


Ir 


anatomy  of,  22,  285 
examination  of,  288 
function  of,  287 
method  of  measuring,  289 
rigidity  of, 

with  acromegaly,  562 


Kataphoria,  782 
Keratitis 

exposure,  353,  326 
lagophthalmic,  353,  326 
neuropathic,  325 

course  and  treatment  of,  326 
xerotica 

with  exophthalmic  goitre,  840 
Keratometry,  55 
Knee-jerk,  925 

with  brain  tumor,  419,  432 
Kopiupia, 

hysterical,  640 
Korsakoff's  psychosis,  595 

with  alcoholic  neuritis,  595 


Lachrymation,  anomalies  of:  diminished,  344 
increased,  334 
mechanism  of,  344 
with  exophthalmic  goitre,  840 
with  tabes,  506 
Lagophthalmos 

from  exposure,  352 
from  facial  paralysis,  352 
from  old  age,  352 
keratitis  with.  326.  353 
Lamina  vitrea,  286 
Landry's  paralysis 

(see  Paralysis,  acute  ascending),  590 
Lateriversion,  267,  268 
Lead  intoxication, 

ocular  signs  of,  528-531 
Lens,  crystalline. 

hysteric  opacity  of,  669 
structure  of,  30 
Letter  blindness,  137 
Levator  spusm  and  paralysis. 

hysterical  (see  Hysterical  ptosis),  654,  655 
differential  diagnosis  of.  656 
Lids,  alopecia  (hysteric)  of.  670 
exnininntion  of.  153 
movements  of,  192.  .345 

harmony   of,  with  those  of  eyeball, 
348 
oedema  of, 

with  exophthalmic  goitre,  H40 
reactions  of,  .306 
reflexes  referable  to,  351 
signs  referable  to.  350,  351 

with  exophthalmic  goitre,  833 


Lids,  tremor  of, 

with  exophthalmio  goitre,  840 
Lid  signs  :  Dalrymple  s  sign,  350 
Graefe's  sign.  350,  351 
of  goitre,  833 
Stellwag's  sign,  351 
Light  reflexes,  290 

arc  of,  course  of  fibres  in.  290  et  seq. 
lesions  of,  291,  292,  294 
examination  of,  299 
Light  test, 

Wernicke's  or  Wilbrand's,  170 

mechanism,  significance  and  value 

of,  170 
methods  to  apply,  171 
Locomotor  ataxia 

(see  Tabes  dorsalis) 
Lumbar  puncture, 

danger  of,  in  brain  tumors,  395 


M 

Macropsia, 

with  epilepsy,  733 
Macula  lutea, 

anatomy  of.  29 
functions  of,  108 
Macular  bundle, 

distribution  of  fibres  of,  109 
centre,  connecting  tracts  of,  111 
fibres, 

decussation  of,  4 
representation  centre,  103 
location  of,  110 
Maddox  rod  test,  201 
'Mastication  and  deglutition, 
difficulties  of, 

in  brain  tumor,  429 
in  bulbar  palsy,  438 
Medulla  and  pons, 
tumors  of,  429 
Membrane  of  Baruch,  286 
Meningocele.  554 
Mental  alterations, 
(see  Psychoses) 
attitude. 

anomalies  of,  due  to  eye-strain,  958 
Microcephalus,  547 
diagnosis  of,  548 
treatment  of,  549 
Microphthalmus,  552 
Micropsia, 

with  epilepsy.  733 
Migraine, 

auras  with,  700 

causes  of,  698 

eye-strain  in  relation  to,  711 

gastric  disorders  of,  701 

hemicrania  with,  701 

hysterical  ophthalmic,  641 

in  hysteria,  paresis,  and  tabes,  709 

malaria  as  a  cause  of,  721 

motor  type  of,  702 

nasal  catarrh  in  relation  to,  721 

nuclear    ophthalmoplegia     differentiated 

from.  720 
ophthaliiiic.  703 

aphasia  with,  708 
headache  in,  706 
hysterical,  641 
visual  signs  of,  703  et  seq. 
ophthalinique  accompagn^e,  708 
ophthalmoplegic.  24.3,  712 
course  of,  713 

cranial  nerve  palsies  with.  714,  717,  718 
diagnosis  of,  719 


INDEX 


981 


Migraine,  ophthalmoplegic,  pain  and  paralysis 
in,  713,  714 

optic  illusions  with,  700 

pain  of,  701 

pathology  of,  710 

psychical,  702,  723 
symptoms  of,  725 

relation  to  epilepsy,  711 

resemblance  to  epilepsy,  710 

scotoma  with,  175 

spectra  of,  114 

symptoms  of,  699 

tabes  in  relation  to,  721 

treatment  of,  726 

varieties  of,  697 

with  exophthalmic-  goitre,  830 
Mind-blindness, 

causes  of,  122,  127 

synonyms  for,  118 
with  brain  tumor,  398 
Mfibius  sign,  836,  932 
Multiple  sclerosis, 

ocular  signs  of,  469 

resemblance  of,  to  brain  tumor,  419 
Muscular  atrophy, 

with  exophthalmic  goitre,  830 

contraction,  932 

difference  of,  from  reflexes,  910 
idiopathic,  932 
Myasthenia  gravis,  457 
Myasthenic  reaction, 

with  Erb's  disease,  462 
Mydriasis, 

hysterical  (see  Dilatation  of  pupils),  647 

interchange  of  myosis  and  (hysterical),  649 

spastic,  314 
Myelitis,  565 

cranial  nerve  palsies  with,  668 

etiology  of,  565 

histology  of,  569 

pathology  of,  565 

prognosis  and  treatment  of,  572 

relation  of,  to  optic  neuritis,  571 

symptoms  of,  565 

treatment  of,  572 
Myoidema,  932 
Myopia, 

apparent,  62 

axial,  43 

benign  or  school,  45 

curvature,  43 

index,  43 

malignant,  45 

school,  45 

treatment,  45 

with  hysterical  cyclospasm,  642 
Myosis,  hysterical,  646 

interchange  of.  with  mydriasis,  649 


N 

Nasal  smile  with  Erb's  disease,  460 
Near-sightedness 

(see  Myopia),  43 
Nervous  irritability  and  exhaustion 

due  to  eye-strain,  776 

character  of,  956 
visual  apparatus  in,  776 

with  exophthalmic  goitre,  829 
Neuralgia, 

facial 

(see  Trigerminal  neuralgia) 
Neurasthenia, 

effect  of  asthenopia  on,  779 

hysteric  manifestations  of,  672 

Rosenbach's  sign  of,  674 


Neuritis, 

multiple, 

alcoholic,  649 

arsenical,  603 

beriberi,  608 

carbon  monoxide  and  disulphide,   60S, 
604 

carcinomatous,  611 

diabetic,  613 

diagnosis  of,  594,  600 

diphtheritic,  605 

etiology  of,  594 

influenzal,  608 

lead  (plumbic),  600 

leprous,  609 

malarial,  608 

pathology  of,  598,  601,  607 

senile,  610 

sulphonal  and  trional,  604 

symptoms  of,  594 

syphilitic,  609 

tuberculous,  609 
retrobulbar, 

simulation  of,  by  hysteria,  637 
Neuroretinitis  due  to  lead  intoxication,  629 
Neuroses,   ocular, 

due  to  eye-strain,  758 
traumatic, 

hysteric  manifestations  of,  672 
Neurotabes,  594 
Numeral-blindness,  142, 
Nyctalopia 

(night-blindness),  176 
Nystagmus,  273 
atactic,  474 
epileptic,  735 
etiology  of,  276 
hysterical,  666 
occupational,  277 
pseudo,  273,  474 

etiology  of,  277 
symptoms  of,  281 
treatment  of,  282 
unilateral,  275 
variations  in,  275 
varieties  of,  274 
voluntary,  279 
with  acromegaly  662 
with  ataxia,  474 
with  brain  tumor,  432,  417 
with  epilepsy,  735 
with  exophthalmic  goitre,  840,  841 
with  Friedreich's  disease,  474 
with  multiple  neuritis,  600,  603 
with  myelitis,  668 


Occipital  lobe, 
tumors  of 

(see  Brain  tumors),  873 
Ocular 

defects  (hysterical),  614 
deviation,  250 

measurement  of,  198 
equilibrium, 

position  of,  193 
innervation, 

impairment  of,  due  to  lead,  528 
movements, 
binocular, 
^         parallel,  193 
test  for,  205 
centres  for,  195 
etiologie  classification  of,  208 
incoordination  of 

(diplopia  with).  H»5 


982 


INDEX 


Ocular  moveiuents,  tests  for,  196 

muveles,  anomalies,  neurasthenic,  of,  674 
associated  action  of.  193 
ventral  nerve,  supply  of,  12  et  seq. 
epilepsy  of,  134 
neurasthenic  anomalies  of,  674 
paralysis  of,  209 

associated  palsies  with,  239 

basal,  228 

causes  of,  234 

conditions  allied  to,  245 

cortical,  221 

course  and  prognosis  of,  241 

diagnosis  of,  211 

differential,  from  spasm,  247 
of  cause  of,  234 
(if  site  of  lesion  in,  237 
value  of   associated     paralysis 
in,  239 

due  to  acromegaly,  233,  562 
to  epilepsy,  737 

to  alcohol  and  other  poisons,  234 
to  autointoxication,  234 
to  basal  brain  lesions,  220,  238, 

227 
to  beriberi,  233 
to  brain  tumors  400,  403,  405, 

421,  429 
to  cerebro  spinal  fever,  233 
to  congenital  conditions,  222 
to  diabetes,  233 
to  diphtheria,  231 
to  erysipelas,  233 
to  exophthalmic    goitre,    233, 

836 
to  extracranial  lesions,  219 
to  general  paresis,  229 
to  trerlier's  disease,  233 
to  hemorrhage,  234 
to  heredity,  235 
to  herpes  zoster,  232 
to  hysteria  and  allied  neuroses, 

230 
to  influenza,  231 
to  intracranial  lesions,  220 
to  kidney  disease,  233 
to  malaria,  233 
to  meningitis,  227 
to  multiple  neuritis,  603  etseq. 
to  mumps,  233 
to  nasal  affections,  234 
to  nuclear  disease,  239 
to  orbital  disease,  237 
to  pneumonia,  233 
to  pontine  disease,  239 
to  pyaemia,  233 
to  renal  diseases,  233 
to  rheumatism,  232 
to  sclerosis,  229 
to  spinal  disease,  228 
to  syphilis,  230 
to  tabes.  228,  501 

frequency  of,  504 
to  tetanus,  233 
to  traumatism,  225 
to  tuberculosis.  232 
to  typhoid.  232 
to  Weil's  disease,  233 
to  whooping  cough,  233 

etiology  of,  218 

exciting  causes  of,  222 

fascicular,  220 

hysterical,  230 

inncrvational,  219 

insertional,  218 

intracranial,  220 


Ocular  muscles,  paralysis  of,  nuclear,  220,  239 
oculomotor,  235,  237 

with  brain  tumor,  404,  421 
orbital,  219,  237 
peripheral,  219 
pontine,  239 
prognosis  of,  241 
significance  of  associated,  406,  416, 

417 
structural,  218 
supranuclear,  221 
symptoms  of,  209 
(see  Ophthalmoplegia) 
treatment  of,  244 
total,  218 
varieties  of,  217 
spasm  of,  246 

etiology  of,  248 
evidence  of,  246 
primary, 

affections  characterized  by,  248 
resemblance  to  paralysis,  247 
secondary, 

affections  characterized  by,  249 
true,  248 
nerves, 

tumors  of,  387 
neuralgia, 

with  acromegaly,  562 
with  myelitis,  667 
neuroses, 

with  eye-strain,  758 
oedema,  324 

pain,  significance  of,  323 
palsies, 

following  epilepsy,  737 
reflex  (supposed),  329 
with  acromegaly,  662 
with  basal  meningitis,  12 
with  Friederich's  disease,  476 
with  hysteria,  663 
with  lead  intoxication,  531 
with  migraine,  714,  717 
with  multiple  neuritis,  595,  603,  608 
stigmata,  hysterical,  614,  670 

etiology  of,  670 
symptoms, 

relation  to  spinal  disease,  579 
with  exophthalmic  goitre,  831,  840 
with  hysteria,  614 
Oculomotor  nerve, 

intracerebral  course  of,  1 1 
nucleus  of,  11 

basal  co-ordinating  centre  of,  412 
function  of,  411 
subdivisions  of,  13 
paralysis  of,  214,  218 
Oculopupillary, 

fibres,  course  of,  688 
reflex,  304 
Olfactory  palsy  with  ocular  palsy,  239 
Ophthalmic  examination, 

epileptic  seizure  caused  by,  737 
herpes,  326 
signs  of  disease,  364 
Ophthalmoplegia, 
chronic,  464 

progressive,  242 
hysterical,  648.  665 
interna,  312 
nuclear,  720 
total,  218 

with  brain  tumor,  404,  419 
with  migraine,  719  et  seq. 
diagnosis  of,  719 
with  myelitis,  668 


INDEX 


983 


Ophthalmoplegia,  with  ocular  palsy,  237 

with  tabes,  498,  503 
Ophthalmoscopy,  54,  360 
instruments  for,  360 
technique  of,  361 
Optic, 

aphasia, 

centre  affected  in.  150 
lesions  causing,  149,  150 
mechanism  of,  149 
atrophy, 

ascending,  consecutive  or  neuritic,  384 
ophthalmic  appearances  in,  382,  384 
prognosis  and  treatment  of,  384 
symptoms  of,  383,  385 
with  acromegaly,  562 
with  myelitis,  567,  569 
with  paresis,  480 
with  pituitary  diseases,  426 
with  tabes  dorsalis,  499 
centres, 

primary,  5 
chiasm, 

decussation  of  fibres  in,  1 
structure  of,  3 
surgical  treatment  of.  876 
tumors  of,  876 
illusions, 

with  migraine,  700,  707 
nerve, 

anatomy  of,  1 
atrophy  of, 

due  to  acromegaly,  562 
to  arteriosclerosis,  467 
to  lead  intoxication,  529 
to  sclerosis,  469 
hereditary,  385 
primary,  382 
secondary,  384 
hemorrhage  into,  368 
inflammation  of 

(see  Optic  neuritis).  375 
involvement  of,   in  spinal  diseases,  585 
multiple  sclerosis  of.  469 
palsy  of, 

with  ocular  palsy,  239 
tumors  of,  428 

early  failure  of  vision,  429 
hallucinations  of  sight  in.  429 
reflex  pupillary  response  in,  429 
neuritis,  375 

acute  retrobulbar,  380 
causes  of,  375 
chronic  retrobulbar 

(toxic  amblyopia),  3S1 

ophthalmic  appearances  in,  382 
pathology    and    sj'mptoms   of, 

381 
prognosis  of,  .382 
color  field  in,  378 
mechanism  of  intracranial,  376 
ophthalmic  signs  of,  377 
prognosis  of,  379 
relation  of,  to  myelitis,  571 
symptoms  of,  377 
treatment  of,  380 
visual  acuity  in.  377 
visual  field  "in,  378 
with  brain  tumor,  430,  419 
with  R-iedreieh's  disease,  476 
with  lead  intoxication,  529 
with  multiple  neuritis,  602,  576,  598 
with  myelitis,  566 
with  pituitary  disease,  426 
tracts, 

intracerebral  course  of,  5 


Optic  tracts,  visual  centres,  scheme  of,  104 
Orbicularis, 

paralysis,  hysterical,  654 
reaction,  306 
Orbit, 

relation  of  structures  of,  347 
Orbital  pain, 

with  acromegaly,  562 
with  myelitis,  567 
with  tabes,  505 
Orbito  palpebral  sulcus, 
obliteration  of. 

with  exophthalmic  goitre,  840 
Orientation, 

lesions  causing,  loss  of,  84 
Orthophoria,  180 


Palpebral  opening, 

changes  in  form  of,  349 

variations  in  size  of,  348 
widening  of, 

due  to  contraction  of  elevator,  352 
Palsies, 

acute  bulbar,  apoplectiform,  445 
bulbar,  progressive,  436 
course  of,  445 
electrical  reactions  in,  441 
etiology  of,  444 
historical  data  of,  436 
pathology  of,  442 
physical  signs  of,  439 
pseudo,  454 
symptoms  of,  436 
accessory,  439 
general,  435 
synonyms  for,  436 
treatment  of,  445 
cerebral,  of  children,  539 

differential  diagnosis  of,  543 
etiology  of,  539 
eye  symptoms  of,  542 
pathology  of  542 
symptoms  of,  539 

ocular,  542 
treatment  of,  544 
types  of.  538 
Parachromatopsia,  88 
Parallax  test,  200 
Parallel  movements 
(associated), 
hysteric  palsy  of,  665 
Paralysis, 

acute  ascending.  Landry's,  590 
etiology  of,  591 
sj'mptoms  of,  590 
ocular,  591 
agitans.  476-477 
asthenic,  457 
cerebral  of  children 

(see  Palsies),  538 
conjugate,  266 
extra-ocular, 

with  exophthalmic  goitre,  836 
of  associated  parallel  movements,  hysteric, 

665 
unilateral  progressive 

ascending  spinal,  581 
with  exophthalmic  goitre,  830 
Paresis, 

juvenile.  491 

ocular  signs  of,  478  et  seq. 
physical  signs  of,  488 
sequence  of  phenomena  in,  489 
summary  of  ocular  signs  of.  488 
with  migraine.  709 


984 


INDEX 


I'criiiu'ter 

for  tesiting  fixation,  204 

porUible.  159 
I'ernifiou.s  una'iiiiii, 

spiniii  Higns  of,  582 

ocular  tsiKDi*  of,  583 
IMioiiatioii  and  cough,  difficult 

with  bulbar  palsy,  438 
I'horometer,  202 
Photophobia, 

significance  of,  330 

treatment  of,  331 

with  epilepsy,  733 
Phy.sical  peculiarities 

due  to  eye-strain,  959 
Pineal  glan<l. 

diagnosis  of  lesions  of,  426 
Pituitary  body, 

diagnosis  of  lesions  of,  426 
Pneuuiogastric  nerve, 

intracerebral  anatomy  of,  18 
Poliu-ncephaiitis.  acute, 

diagnosis  of, 

from  multiple  neuritis,  600 
Poliomyelitis, 

anology  of,  to  encephalitis  superior,  452 
Polyneuritis 

(see  Multiple  neuritis) 
Pons, 

embolism,  hemorrhage   and   thrombosis   of, 
445 

anatomic  facts  in,  445 
I)athology  of,  446 
symptoms  of,  447 

softening  of,  448 

course,  prognosis,  and  treatment  of,  450 

tumors  of,  429 
Porencephaly, 

causes  and  typies  of.  546 

diagram  of,  547 
Position  sense. 

cortical  centre  of,  ,397 
Posterior  lateral  sclerosis.  581 
Presbyoi>ia,  39 
Projection,  binocular,  182 

monocular,  178 
law  of.  179 
Proptosis, 

determination  of,  155 

with  exophthalmic  goitre,  8.30 
Pseudobulbar  paralysis,  4.54 
pathology  of,  455 
prognosis  and  treatment  of,  457 
symptoms  of,  456 
Pseudokinesis,  210 
Pseudosclerosis  of  Westphal,  479 
Pseudoscopic  apparatus,  694 
Pseudotabes,  594 

Pseudotorticollis  due  to  eye-strain,  823 
Psychic  blindness,  118 
Psychopathies 

due  to  eye-strain,  956 
Psychoses 

after  eye  operations,  854 

bibliography  of,  865 
etiology  of,  S.58 
groups  of,  857 
treatment  of,  864 

KorsohoflF's,  595 

with  brain  tumors,  398  et  seq. 

with  exophthalmic  goitre  829,  8.30 

with  migraine,  725 

with  multiple  neuritis.  601.  604 
Ptosis,  atonic,  .355 

general  characteristics  of,  353 

hysteric.  656 


Ptosis,  neurasthenic,  675 
pseudoparalytic,  652 
spastic,  653 
traumatic,  356 
with  brain  tumor,  416 
with  multiple  neuritis,  602,  604,  608 
with  myelitis,  568 
with  ocular  palsy,  2.36 
with  paresis,  488 
with  tabes,  502 
Puerperal  eclampsia, 

eye  symptoms  of,  740 
Pupil, 

Argyll  Robertson,  308 

frequency  and  significance  of,  493 
lesions  causing.  309 
unilateral,  495 
dilated, 

with  multiple  neuritis,  603 

with  paresis,  482 

with  tabes,  497,  498 
during  gastric  crises,  498 
fixation  of, 

with  acromegaly,  562 

with  multiple  neuritis,  603 
inequality  of,  674 

with  brain  tumor,  404 

with  multiple  neuritis,  604 

with  paresis,  479,  481 

with  tabes,  496,  498 
in  hysteric  attacks,  649 
light  reaction  of,  315,  604 
narrowed, 

with  multiple  neuritis,  603 

with  paresis,  480 

with  tabes,  496 
neurasthenic  signs  in,  674 
normal,  288 
paradox, 
in  tabes,  505 

pathologic  variations  of,  307 
phenomena  of, 

with  epilepsy,  735,  736 

with  myelitis,  567 
rigidity  of, 

with  multiple  neuritis,  603 
Pupillary 

centre  (reflex),  589 

dilator,  286 

lid  reaction,  306 

nerve-fibres, 

anatomy  and  physiology  of,  5 
orbicularis  reaction,  306 
phenomena, 

epileptic,  735 

medicolegal  value  of,  736 

hysterical,  646 
reflexes.  290 

convergence,  295 

hemianopic,  318 

light,  290 

Marian's  experiments,  with  296 

myotonic,  314 

oculo,  304 

on  movement  of  eye.*,  307 

pain,  303 

paradoxical.  317 
hysterial,  650 
sphincter,  285 

R 

Red-glas.s  test,  200 
Reflexes, 

abdominal,  930 

Achilles-tendon,  925 

adductor.  925 

ankle-clonus.  926 


IN^DEX 


985 


Reflexes,  uttention,  304 
Babinsky's,  927 

modifications  of,  928 
bicipital,  918 
chin-jerk,  917 
conditions  increasing,  91;') 
conjunctival  (corneal),  351 
cortex,  305 
Chvostck's,  916 
cremasteric,  931 
definition  of,  910 
difterences   of,    from    muscular   contraction, 

910 
femoral,  929 
frontal,  930 
fundus,  362 
gluteal,  932 
humeroscapular,  932 
hypogastric,  931 
hypothenar,  919 
iliac,  932 
Kernig's,  929 

knee-jerk,  patellar.  919,  924 
method  to  elicit.  920 
significance  of.  911,  922 
lid,  351 
light.  290 

arc  of,  290 

fibres  of,  291,  294 

decussation  of,  292 
lesions  of,  291,  292,  294 

examination  of  299  et  seq. 
literature  on,  933 
malar,  917 
mechanical,  910 
meahanism  of,  911 
method  of  eliciting,  915 
nature  of,  914 
patellar,  924 

clonus,  922 

with  brain  tumor,  419,  432 
plantar,  927 
pupillary  cortical,  304 
psychical,  910 
pyramidalis,  931 
scapulohumeral,  932 
Sinkler's  toe-jerk,  930 
study  of,  915 
supinator,  919 
supra-orbital,  351,  916 

significance  of,  916 
tibialis  phenomenon,  930 
tricipital,  919 
Refraction, 

anomalies  of,  ammetropia,  40 
effect  of  asthenopia  on,  781 
errors  of, 

headache  in  relation  to,  748 

ocular  defects  and 

position  of  head  in.  822 

physiologic  relation  of  eye  to,  777 

with  exophthalmic  goitre,  841 
Respiratory  signs, 

with  exophthalmic  goitre,  842 
Retina, 

anaemia  of,  365 
anajsthesia  of,  365 
anatomy  of,  26 
angeoid  streaks  of,  367 
apoplexy  of,  367 
arteriosclerosis  of.  466 
artery,  central,  of. 
embolism  and  thrombosis  of,  368 
blindness  due  to.  742 
causes  of,  368 
treatment  of,  369 


Retina,  blood  supply  of.  29 

corresponding  points  of,  182 

diseases  of,  ;>ti(i 

dyssesthesia  of. 

with  epilepsy,  733 

epilepsy  of.  734 

fatigue  of,  160 

function  of,  108 

hemorrhage  of,  367 

hypersBsthesia  of,  365 
hysterical.  640 

hysteric  changes  in,  669 

inflammation  of, 

(see  Retinitis).  370 

macula  lutea,  364 

oedema  of,  366 

papillae  of.  363 

sensory  affections  of,  365 

vascularity  of,  367 

vascular  lesions  of,  365 

vein,  central,  of 

thrombosis  of,  369 

vessels  of,  363.  29 

anomalies  of,  366 
inflammation  of,  367 
Retinitis,  370 

albuminuric,  373 

anaemic,  374 

diabetic,  374 

pigmentation,  371 

proliferating.  367 

syphilitic,  372 

varieties  of,  371 

with  brain  tumor.  431 

with  paresis.  486 


Sclera, 

anatomy  of,  22 
Sclerosis, 

diffuse  cerebral,  474 
insular, 

simulation  of  hysteria  to,  637 
lateral. 

amyotrophic,  580 
postero,  581 
primary,  580 
Scotoma,  definition  of,  158 
fatigue, 

neurasthenic,  680 
lead,  529 
ring,  166 

with  arteriosclerosis  of  eye,  466 
with  epilepsy,  732 
with  migraine,  700,  704.  706 
with  multiple  neuritis,  603 
Scotoma,  annular 
central,  165 
hysteric,  627 
Sight, 

hallucination  of, 

following  epilepsy,  737 
Skiascopy,  54 

anomalies  in  development  of,  545  554 
with  opticatrophj-,  554,  555 
Sleep  and  hypnotism, 

ocular  manifestations  of,  689 
Soul  blindness,  118 
Speech, 

cerebral  zone  of,  115 

anatomy  .and  physiology  of,  115 
factors  determining  location  of,  135 
disorders  of  (see  also  Aphasia), 
with  migraine,  925 
with  brain  tumor,  399 
with  bulbar  palsy,  437 


986 


INDEX 


Sphincter  iris  muscle,  293 
Spinal  cord, 

disea^e^i  und  injury  of,  683 
gunshot,  588 
ocular  signs  of,  584 
optic  nerve  involvement  in,  585 
sympathetic  disease  with,  586,  588 
curvature 

due  to  eye-!»train,  823 
Squint  (strabismus), 
alternating,   199 
bilateral  nature  of,  261 
binocular  vision  in,  187 
classification  of,  180 
convergent,  255 
determination  of,  198 
development  of, 

conditions  favoring,  260 

due  to  convergence  excess,  252 

to  convergence  insuflSciency,  256 
to  convergence  paralysis,  257 
to  divergence  paralysis,  255 
to  epilepsy,  735 
to  esophosia,  252 
to  exophoria,  256 
rationale  of,  259 
divergent,  258 
epileptic,  735 
hysterical,  659 
nature  of,  249 
position  of  head  in,  823 
symptoms  of,  263 
testa  for,  198 
treatment  of,  264 
vertical, 

hyperphoria  with,  262 
Stanley  Barnes,  diagram  by,  141 
Stell wag's  sign,  351,  835 
Stereognosis, 

cortical  centre  of,  397 
Stereoscopic  test,  204 
Stomach  disturbances 

due  to  eye-strain,  807 
Strabismus 

(see  Squint) 
Supraorbital  nerve, 

exposure  of,  898 
Sursumversion,  268,  269 
Sympathetic  nerve, 

involvement  of,  in  spinal  disease,  586 

symptoms  of,  588 
removal  of,  for  glaucoma,  341 
SynUx,  146 
Syringomyelia,  572 

cranial  nerve  paralysis  with,  578 
ocular  palsies  with,  576 
pathology  of,  573 
symptoms  of,  572  et  seq. 

ocular,  573 
simulation  of,  by  hysteria,  637 
visual  field  restriction,  575 


Tabes  dorsalis,  deafness  with,  502 

diagnosis  of,  from  multiple  neuritis,  594 
from  pseudotabes,  594 
facial  anissth&sia  with,  505 
gait  in,  944 
lachrymation  in,  506 

neuralgia  (periorbital)  in,  505 
ocular  crises  with.  505 
signs  of,  493 

summary  of,  506 
pseudotabes,     diagnosis    of,     from    true 
tabes,  594 


'  Tabes  dorsalis,   lachrymation   in,  exophthalmic 
goitre,  with,  830 

with  migraine,  709 
Taste  impairment  with  brain  lesions,  430 
Tears, 

secretion  of, 

in  disease  of  fifth  nerve,  342 
in  disease  of  seventh  nerve,  343 
mechanism  of,  342 

part  played   by  seventh    nerve  in, 
343 
variations  in  amount  of,  344 
Tenon's  capsule, 

anatomv  of,  188 
Test-letters,' 50,  51 
Tetanus, 

cephalic,  728 

pathology  of,  730 
hydrophobic,  728 
Tetany, 

ocular  signs  of,  730 
with  exophthalmic  goitre,  830 
Thyroid  enlargement 

in  exophthalmic  goitre,  830 
Tic  douleureux 

(see  Trigeminal  Neuralgia) 
Tongue,  impaired  mobility  of, 
with  brain  tumor,  429 
with  bulbar  palsy,  437,  439 
Torticollis 

due  to  eye-strain.  823 
pseudo,  823 
with  ocular  palsy,  211 
Tremors, 

causes  of,  906 
classification  of,  907 
congenital,  907 
definition,  906 
diagnosis  of,  907 
fibrillary,  429 
imitation,  909 
in  brain  tumor,  421 
in  exophthalmic  goitre,  908,  829 
in  hysteria.  908 
in  multiple  sclerosis,  908 
in  neura.sthenia.  909 
in  paralysis  agitans,  908 
in  paresis,  909 
pseudo,  906 
significance  of,  906 
Trigeminal  nerve,  antcsth&sia  of,  321 

with  ocular  palsy,  240 
with  tabes.  505 
anatomy  of,  320 
distribution  of,  321 
functions  of,  321 
herpes  zoster  of,  326 
causes  of,  328 

diagnosis  from  erysipelas,  327 
symptoms  and  ocular  signs  of,  317 
treatment  of,  328 
implication  of,  in  spinal  lesions,  16 
lesions  of,  320 
nuclei  of,  15 

neuralgia  of,  (tic  douleureux),  323,  894 
medical  treatment  of.  896, 
surgical  treatment  of.  897 
neuritis  of,  323 
palsy  of, 

with  ocular  palsy,  240 
parnesthesia  of,  322 
physiology  of,  321 
sensory  fibers  of,  15 
trophic  disorders  of,  324 
Triplopia, 

hysterical,  643 


INDEX 


987 


Trochlear  nerve, 

intracerebral  anatomy  of,  13 
Tropouieter. 

for  testing  fixation,  204 

u 

Unconsciousness 

with  multiple  neuritis,  604 
Uraemia, 

blindness  due  to,  738 
Urinary  disorders, 

with  exophthalmic  goitre,  843 
Uvea, 

anatomy  of,  22 

blood  and  nerve  supply  of,  25 


Vagus  (pneumogastric)  nerve, 
disease  of, 

with  multiple  neuritis,  595 
intracerebral  anatomy  of,  18 
Vascular  disorders, 

with  exophthalmic  goitre,  828 
Vertigo, 

ocular,  803 

diagnosis  and  treatment  of,  804 
paralyzing 

(Gerlier's  disease),  462 
reflex,  803 

with  brain  tumor,  431 
with  migraine,  726 
with  ocular  palsy,  210 
Vertigo  and  false  projection, 

with  conjugate  paralysis  and  spasm,  266 
Vestibular  nerve, 

intracerebral  anatomy  of,  18 
Vibration  sensation,  133 
Vision, 

acuteness  of,  50 
apparatus  of, 

congenital  absence  of,  552 
retino-intracranial,  65 
tumors  in  relation  to,  367 
binocular,  single,  181 

comparison  of,  to  binocular  fixation,  187 
in  squint,  187 

test  for  field  of.  187,  205,  206,  195,  199 
centre  of,  and  optic  tracts, 

scheme  of,  104 
cortical  area  of.  8,  59,  60,  75 

comparison   of,  in    man  and   lower  ani- 
mals, 63 
focal  disturbances  of,  79 
psychic  and  sensory  areas  of.  73 
retinal  representation  in,  79 
disturbances  of,  in  brain  tumor,  874 
treatment  of,  892 

lumbar  puncture,  892 
ventricular  puncture. 

Keen's  method  of,  893 
hallucinations  of,  174 
causes  of.  174 
varieties  of.  174 
with  brain  tumor.  388 
with  epilepsy.  737 
with  migraine,  707 
with  multiple  neuritis.  602,  604 
hyperaesthesia  of,  with  paresis,  479 
impaired  near, 

with  multiple  neuritis.  605 
impairment  of,  with  brain  tumor.  874 

operation  to  prevent,  875 
with  epilepsy,  732 
with  multiple  neuritis,  566 
with  myelitis,  566 


Vision,  line  of,  36 
normal,  746 

peripheral  limitation  of,  169 
lesions  causing,  169 
zone  of,  166 
physiology  of,  20 
single,  antipathy  to,  186 
stereoscopic.  182 
telescopic,  166 
Visual  act, 

acuteness,  50 

reduction  of,  hysteria,  621 
apparatus,  20 
apraxia,  118 
aurae,  114 
cortex,  8,  59,  60,  75 

concrete  conception,  area  of,  61 
connections  and  associations  of,  63 
differences   of,  in   man    and  lower   ani- 
mals,  63 
evolution  of,  69 
focal  diseases  of,  79 
histology  of,  66 

layers  of, 
Cajal's,  67 

Campbell's,  72,  73 
von  Monakow's,  67 
image  (binocular),  182 

in  diplopia.  187 
limits  and  subdivisions  of,  59 

Flechsig's,  60 
localization, 

histology  in  relation  to,  66 
defects  due  to  atrophy  of  cerebrum,  550 

in  neurasthenia,  due  to  eye-strain,  776 
disturbances, 

in  astereognosis,  114 
in  hemiantesthesia.  114 
with  acromegaly,  560 
with  multiple  neuritis,  602 
fibres,  decussation  of,  1 
field,  boundaries  of,  161 
changes,  epileptic,  733 

with  exophthalmic  goitre,  841 
concentric  contraction  of, 
examination  for,  624 
hysteric,  622 
types  of,  626 
contraction,  hysterical, 

nature  of,  633 
defects  of,  159 
disturbances  of, 
hysteric,' 621 

types  of,  626 
with  myelitis.  568 
extent  for  color,  162 
fatigue, 

diagnostic   significance  of,    684    ct 

seq. 
types  of,  681  et  seq. 
hysterical,  634 
measurement  of,  157 
neurasthenic  anomalies,  675  et  seq. 
oscillating  (neurasthenia),  680 
peripheral  zone  of,  166 
sector-like  defects  in,  169 
shape  of,  164 
fusion,  181 
impairment, 

with  acromegaly,  662 
pragraatamnesia,  118 
psychology  of,  57 
sensation  (subjective).  174 
sense.  614 

anaesthesia  of  (complete), 
hysterical,  614 


988 


INDEX 


Visual   act,   sense,   anaesthesia  of  (incomplete), 
hysterical,  621 
signs 

of  epilepsy,  731 
system, 

congenital  absence  of,  652 

Spiller's  case  of,  563 
Vitreous  body, 

structure  of,  31 

w 

Walking  (see  (iait), 

delayed,  949 

description  of,  937 
Webster's  syndrome, 

simulation  of,  by  hysteria,  8&3 
Wernicke's  disease,  452 

light  test.  170 

symptom, 

in  differentiation  of  anopsia,  85 
Wilbrand's  light  test.  170 
Winking, 

mechanism  of,  349 
Word-blindness.  127 


Word-blindness,  cortical,  128 
signs  of,  128 

definition  of,  128 

etiology  of,  132 

lesions  causing,  132 

mechanism  of,  128 

pure,  128 

varieties  of,  128 
Word-deafness, 

with  brain  tumor,  399 

with  migraine,  725,  72(J 
Word-dumbness,  148 
Word-seeing,  centre  for,  128 
Wrist-drop, 

with  multiple  neuritis,  594,  599,  600 


Xanthopsia 

(yellow  vision),  176 


Yellow  vision,  176 


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